Fitzgerald LF, Simpson RK and Trask T
Locked-in syndrome resulting from cervical spine gunshot wound
Journal of Trauma 42:147-9, 1997

Abstr.: A patient sustained a cervical spine gunshot wound resulting in immediate quadriplegia and obstruction of both vertebral arteries. After an 8-hour delay, basilar artery thrombosis occurred and resulted in locked-in syndrome. Bilateral occlusion of the vertebral arteries after penetrating cervical trauma has not been reported previously. The incidence and treatment of traumatic basilar thrombosis and locked-in syndrome is reviewed. Rapid diagnosis of ascending thrombosis and prompt treatment with hyperdynamics and anticoagulation may improve outcome.

Onofrj M, Thomas A, Paci C, Scesi M and Tombari R
Event related potentials recorded in patients with locked-in syndrome
Journal of Neurology, Neurosurgery & Psychiatry 63:759-64, 1997

Abstr.: OBJECTIVE: To determine the possibility of recording "cognitive" event related potentials (ERPs) in locked-in patients and therefore to determine whether ERPs can have a role in differential diagnosis of coma. METHODS: ERPs to classic auditory or visual "odd ball paradigms" were recorded three to four days, seven to eight days, and 30 to 60 days after admission to the intensive care unit, in four patients affected by basilar artery thromboembolism resulting in locked-in syndrome. Two patients (one 32 year old man, one 31 year old woman) could move the eyes laterally and vertically spontaneously and on command. One patient (a 39 year old man) had a "one and half syndrome", one patient (a 40 year old woman) could only elevate the left eyelid and eye. Results were compared with data from 30 age matched controls. In the last recording session a letter recognition paradigm was applied, in which ERPs were produced by the identification of letters forming a word. Results were compared with five age matched controls. Brainstem lesions extending to the pontomesencephalic junction were found on MRI and CT. RESULTS: ERPs to the oddball paradigms were recorded in three patients in the first recording session, in all patients in the second recording session. Latency, amplitude, and topographic distribution of ERP components were inside normal limits. With the letter recognition paradigm the patients could emit a P3 component to correspond with target letters, with the same margin of error as controls. CONCLUSION: It is possible to record ERPs in patients with locked-in syndrome shortly after the acute ischaemic lesion, and therefore to assess objectively cognitive activities. Furthermore the letter recognition paradigm could be implemented to facilitate linguistic communication with patients with locked-in syndrome.

Giacino JT
Disorders of consciousness: differential diagnosis and neuropathologic features. [Review] [41 refs]
Seminars in Neurology 17:105-11, 1997

Abstr.: Disorders of consciousness present intriguing challenges to the neurologist and neurorehabilitation specialist. Assessment is constrained by the lack of reliable methods of assessing consciousness, and there are no treatment interventions known to influence the course of recovery from these conditions. In addition, the relationship between the clinical features associated with these disorders and their corresponding pathophysiologic substrate is also unclear. Our understanding of disorders of consciousness has not kept pace with the advances in neurosurgical management that have decreased mortality following severe injury. There is still considerable confusion regarding differential diagnosis and prognostication concerning states of severely altered consciousness. The purpose of this article is to discuss the content and neural basis of consciousness and to review the terminology most often used to describe altered states of consciousness. The neurobehavioral criteria for differentiating among specific syndromes associated with severe alterations in consciousness are presented. Representative case studies are utilized to illustrate the characteristic clinical profiles of coma, vegetative state, persistent and permanent vegetative state, minimally conscious state, akinetic mutism, and locked-in syndrome. Areas of ambiguity and controversy are emphasized and future directions for research are suggested. [References: 41]

Park SA, Sohn YH and Kim WC
Locked-in syndrome with bilateral peduncular infarct
Journal of Neuroimaging 7:126-8, 1997

Abstr.: Locked-in syndrome due to midbrain infarct is very rare. This report describes a patient with locked-in syndrome whose magnetic resonance images showed bilateral infarcts in the cerebral peduncle. Cerebral angiography disclosed bilateral irregular thrombotic narrowing of the vertebral and proximal basilar arteries with rostral basilar artery occlusion, a previously unreported findings.

Wijdicks EF, Nichols DA, Thielen KR, Fulgham JR, Brown R Jr., Meissner I, Meyer FB and Piepgras DG
Intra-arterial thrombolysis in acute basilar artery thromboembolism: the initial Mayo Clinic experience [see comments]. [Review] [13 refs]
Mayo Clinic Proceedings 72:1005-13, 1997

Abstr.: OBJECTIVE: To investigate the feasibility of intra-arterial thrombolysis in acute basilar artery thrombosis. DESIGN: We reviewed a consecutive series of patients in whom intra-arterial thrombolysis was performed during the period from 1994 to 1996. MATERIAL AND METHODS: Intra-arterial thrombolysis with urokinase was done in an attempt to recanalize the basilar artery in a series of nine patients with basilar artery thrombosis admitted to the neurologic intensive care unit. At the time of initial assessment, all nine patients had major neurologic deficits attributable to brain-stem ischemia, including two patients with locked-in syndrome. RESULTS: Recanalization of the basilar artery system was successful in seven of the nine patients (a range of 2 to 13 hours after the ictus). Failure to recanalize the basilar artery occurred in two patients, who died after progressing to coma. Complete recovery or only minimal neurologic deficits were demonstrated in five of the nine patients. Despite recanalization of the basilar artery, two patients had no major change in their neurologic function, and both ultimately had severe ataxia and were fully dependent on others. A cerebellar hemorrhage occurred in one patient but without clinical worsening. Two patients had a retroperitoneal hematoma. CONCLUSION: Intra-arterial thrombolysis with urokinase in acute basilar artery occlusion resulted in recanalization in seven of the nine patients (78%). Five of the nine patients recovered fully, including two patients who had had locked-in syndrome. In light of the devastating natural course of acute basilar artery occlusion, these initial results are encouraging and indicate that intra-arterial thrombolysis may be a useful emergency treatment, even in patients with prolonged symptoms of ischemia (up to 12 hours). [References: 13]

Bassetti C and Hess CW
Electrophysiology in locked-in syndrome [letter; comment]
Neurology 49:309, 1997


Loyau P
[Locked-in syndrome--wrote a book with his left eye]. [Danish]
Sygeplejersken 97:15-7, 1997


Scott JS, Ockey RR, Holmes GE and Varghese G
Autonomic dysfunction associated with locked-in syndrome in a child
American Journal of Physical Medicine & Rehabilitation 76:200-3, 1997

Abstr.: This is a report of a young boy with the unusual combination of autonomic dysfunction with locked-in syndrome following multiple shunt revisions for hydrocephalus. A review of the literature on autonomic dysfunction syndrome and the complex clinical picture of the combined syndromes in a pediatric patient are discussed. The marked effectiveness of treatment with carbidopa/levodopa over bromocriptine for both syndromes is noted.

Thiel A, Schmidt H, Prange H and Nau R
[Treatment of patients with thromboses of the basilar artery and locked-in syndrome. An ethical dilemma]. [German]
Nervenarzt 68:653-8, 1997

Abstr.: Decisions regarding the extent of treatment of severely ill patients can be an ethical dilemma when life-prolonging intensive care contradicts the goal of avoiding unnecessary suffering on the part of the patient. Here we present the results of a written survey of physicians on neurological intensive care units in Germany regarding the treatment of patients with basilar artery thrombosis and locked-in syndrome. 52% of the 93 physicians who replied advocated not treating severe infections with antibiotics, 38% were in favor of stopping intensive care. In contrast, 55% recommended intubating the patient in the presence of swallowing disturbances and imminent aspiration. 58% were in favor of discussing these problems in detail with the patient, and 87% advocated discussing them with relatives. Nearly all physicians (97%) recommended using adequate amounts of opiates and benzodiazepines. In very rare cases, 99% would agree to the use of passive euthanasia and 19% to active euthanasia. These findings illustrate the current disagreement on some of the important treatment decisions among physicians on neurological intensive care units. An open exchange of views on these questions could facilitate the appropriate consideration of ethical matters in the treatment of these patients.

Tabarki B, el Madani A, Alvarez H, Husson B, Lasjaunias P, Landrieu P, Tardieu M and Sebire G
[Ischemic cerebral vascular accident caused by vertebral artery dissection]. [French]
Archives de Pediatrie 4:763-6, 1997

Abstr.: BACKGROUND: Strokes due to vertebral artery lesions are not yet well known in children. CASE REPORT: We report on a case of post-traumatic vertebral artery dissection responsible for ischemic stroke in a 8-year old boy. Headache, vomiting and a brief loss of consciousness were the main initial signs. Neurological examination showed a locked-in syndrome. Cerebral imaging revealed lesions in cortical cerebellar hemisphere, cerebral pedoncular and protuberance. An arteriogram performed on day 10 showed left vertebral artery occlusion at C2 levels consistent with vertebral dissection. Antiagregants treatment was given. Neurological recovery was good. Pertinent clinical data of 24 children who had strokes due to a vertebral artery dissection are analysed. CONCLUSION: Vertebral artery dissection is presently a well-known cause of childhood strokes. Benefits from anticoagulants are now established.

List WF
[Ethics in intensive medicine]. [Review] [14 refs] [German]
Anaesthesist 46:261-6, 1997

Abstr.: In more than 30 years of development of intensive care medicine (ICM), our specialty has acquired moral and ethical standpoints, although not without public pressure and discussions. Special commissions dealing, e.g., with brain death, terminal care, ethics of foregoing life-sustaining treatment in the critically ill, withholding or withdrawing mechanical ventilation, and other issues have been formed in a number of medical societies. International consensus conferences have helped to clarify some of the issues. With increasing experience, a multitude of ethical problems have arisen in ICM that have to be dealt with, such as the issue of quality of life. What is an unworthy life? Are we allowed to make judgments for our patients? What is cost-effectiveness in ICM? Other restrictions include bed and equipment shortages in the intensive care unit (ICU), the necessity for triage--undisputed in catastrophe medicine--and how one should proceed in managing elective patients? In situations of limited ICU bed availability, sicker patients will be admitted, sparing out patients who are less ill for observation and those with poor quality of life and poor prognosis. For the future, it will likely be necessary to define the patients who should be admitted to an ICU more than those who should not be admitted. An ICU treatment entitlement index would be directly proportional to the probability of successful outcome and the quality of the remaining life, and would be inversely related to costs for achieving success. The ICU outcome with survival, hospital mortality, and follow-up of ICU patients is considered. DNR (do not resuscitate), the dying patient, terminal care, terminal weaning--DNT (do not treat)--active and passive euthanasia, living wills, quality of life, and cost-effectiveness for ICU patients are defined. Their application in the ICU will be discussed and problems pointed out. Outcome predictions using scores (APACHE III, SAPS II, MPM) have been developed based on previous experience, but should only be applied to patient groups and for quality assurance in ICUs. The most frequent and difficult problem in the ICUs is the vegetative state, which requires an exact diagnosis. The differential diagnosis from other comatose states such as coma, brain death, and locked-in-syndrome is depicted. The ethics of interrupting life-sustaining treatment in critically ill patients have been worked out by a Task Force on Ethics of the Society of Critical Care Medicine (1990). A consensus was found that the patient may judge to forego therapy; ethically it is then appropriate to withhold or withdraw therapy. According to the consensus, withdrawing an already initiated treatment should not necessarily be regarded as more problematic than a decision not to initiate treatment. In my mind, however, there is a great difference between withdrawing or withholding, e.g., ventilation. A dissentive opinion by some members of the Task Force stated that hydration and nutrition other than high-technology or parenteral nutrition are key components of patient care, and should not be equated with medical intervention. The ethical problems associated with active euthanasia (physician-assisted suicide or death) as practised in the Netherlands are also discussed. In most countries this practice seems unacceptable. From 30 years experience in ICM, there are many more ethical questions and case reports without clear solutions. Care decisions for single patients in unacceptable situations should be made after medical evaluation by the intensivist with the medical team and, if possible, by the patient and/or his or her surrogate. Legislation and solutions cannot be expected for single patients, but ethics committees could be helpful in decision-making. [References: 14]

Ara JR, Marzo ME, Brieva L, Uson M and Capablo JL
["Locked-in" syndrome due to hyperglycemia]. [Spanish]
Revista de Neurologia 25:1091-2, 1997

Abstr.: INTRODUCTION: Hypoglycemia can cause a diffuse brain malfunction and sometimes a focal neurological deficiency, that could lead to a mistaken diagnosis of cerebrovascular disease. CLINICAL CASE: We describe the case of a 67 year old man, with diabetes mellitus type II treated with glibenclamide, that was referred to our hospital due to worsening of his chronic obstructive pulmonary disease. On the fifth day following admission he developed acute weakness in the right extremities and experienced difficulty in talking: six hours later he was conscious, with normal eye movements, but there was an absence of spontaneous facial motility and to pain; he showed complete cuadraplegia and bilateral Babinski. A determination of glycemia was made with the result of 24 mg/dl; after immediate treatment with glucose solution intravenously the patient recovered in a few minutes. CONCLUSION: This clinical observation reminds us of the importance of determining blood glucose in the assessment of any acute neurological dysfunction.

Arunodaya GR, Vani S, Shankar SK, Taly AB, Swamy HS and Sarala D
Fibromuscular dysplasia with dissection of basilar artery presenting as "locked-in-syndrome"
Neurology 48:1605-8, 1997

Abstr.: We report on a young man with fibromuscular dysplasia involving the basilar artery detected at autopsy. He presented with sudden onset of stroke, and the lesion was complicated by thrombosis and dissection of the vessel wall. The organizing thrombotic lesion of the basilar artery was responsible for the ventral pontine infarct that resulted in "locked-in syndrome."

Gutling E, Isenmann S and Wichmann W
Electrophysiology in the locked-in-syndrome [see comments]
Neurology 46:1092-101, 1996

Abstr.: We investigated five patients with a locked-in-syndrome (LIS) and reported the clinical, electrophysiologic, neuroradiologic, and neuropathologic findings. EEG reactivity was present in two and absent in three cases. Somatosensory evoked potentials (SEP) varied from unilaterally normal to bilaterally absent. We conclude that there is no specific pattern of SEP abnormality characteristic of LIS and that EEG reactivity cannot be taken as a sole measure of consciousness.

Orimo S, Goto K, Ozawa E and Murota T
[Early recovery from locked-in syndrome due to brain infarction in a young patient with hypoplasia of bilateral vertebral arteries and a persistence of primitive trigeminal artery]. [Japanese]
Rinsho Shinkeigaku Clinical Neurology 36:993-5, 1996

Abstr.: A 36-year-old man was admitted to Kanto Chuo Hospital because of hearing loss and dysphagia. On admission physical and neurological findings revealed obesity, hypertension, nystagmus, right hearing loss, dysarthria, and dysphagia. Routine laboratory findings disclosed leukocytosis, liver dysfunction, hypercholesterolemia, proteinuria, and glucosuria. Immunological, coagulopathic, and endocrinological findings, electrocardiogram, echocardiogram, and brain CT scan were unremarkable. He was diagnosed as brainstem infarction, and then conservative therapies were begun. Seven hours after admission, he suddenly fell into coma and apneutic state, requiring artificial ventilation. The next day he was fully conscious, but could'nt make any voluntary movements except for vertical eye movements, suggesting locked-in syndrome (LIS). Brain MRI showed infarction of pons, medulla oblongata, and right cerebellum. Cerebral angiography revealed hypoplasia of bilateral vertebral arteries, a persistence of right primitive trigeminal artery (PTA), and retrograde blood flow of basilar artery from the PTA. Then he made a rapid recovery, and on 80th day he was discharged only with right hearing disturbance and mild left cerebellar sign. We speculated that hypoplasia of the bilateral vertebral arteries caused the brain infarction, and that back flow of the basilar artery from the PTA, in part, contributed to the early recovery from the LIS.

Childs NL and Mercer WN
Sinemet in locked-in syndrome [letter; comment]
Archives of Physical Medicine & Rehabilitation 77:523-4, 1996


Onofrj M, Melchionda D, Thomas A and Fulgente T
Reappearance of event-related P3 potential in locked-in syndrome
Brain Research. Cognitive Brain Research 4:95-7, 1996

Abstr.: In a patient affected by locked-in syndrome, because of a lesion of the caudal-ventral pontine tegment, we recorded event-related potentials (ERPs) to an acoustic odd-ball paradigm. We did not record N2 and P3 components in the 50 days following the acute lesion although the patient was able to understand the task and communicate with blinking. 55 days after the acute lesion, N2 and P3 reappeared on scalp derivations. This findings suggest that brainstem structures are involved in P3 or N2-P3 generation.

Canova CR, Kuhn M, Hartmann K and Reinhart WH
[Thromboembolic events in women treated with hormones. Acute cerebrovascular thrombosis in 2 young women receiving ovulation inhibitors, and experiences of SANZ (Swiss Drug Monitoring Center) 1991-1995]. [Review] [43 refs] [German]
Schweizerische Medizinische Wochenschrift. Journal Suisse de Medecine 126:2119-26, 1996

Abstr.: We describe 2 young patients with severe cerebrovascular thrombotic events whose only risk factor was intake of oral contraceptives. A 41-year-old woman suffered thrombosis of the basilar artery and remained disabled with a locked-in syndrome; a 23-year-old woman had thrombosis of the sinus sagittalis. These 2 cases and the current discussion regarding the use of hormones either as oral contraceptives or hormonal replacement therapy with estrogens and/or progestins prompted a review of the literature and a review of all spontaneous reports from 1991 to 1995 to SANZ (Schweizerische Arzneimittel-Nebenwirkungs-Zentrale), the Swiss Drug Side Effects Monitoring Center. The spontaneous reporting under this system does not allow conclusions on prevalence and incidence of adverse events. In these 5 years 33 vascular events associated with different hormones were reported. There were 28 reports of venous thrombosis of the lower and upper limb or pulmonary embolisms, and 5 reports of cerebrovascular complications. In 9 of 33 cases the hormones were used as hormonal replacement therapy and in 24 of 33 cases as oral contraceptives. In half of these women other risk factors for thromboembolic events such as overweight, family history of thrombosis and immobilization were known. Complications occurred with different preparations containing different estrogens and progestins. According to the literature, third generation progestins seem to involve a slightly increased risk of venous thromboembolism but a reduced risk of arterial thromboembolic events, which was confirmed by the SANZ data (17 of all 24 cases). Increasing age and rising estrogen dosage of oral contraceptives are associated with increased risk of vascular events. Although thromboembolic events also occur during hormonal replacement therapy with naturally occurring estrogens, the prophylactic potential of these drugs with regard to osteoporosis and cardiovascular events certainly outweighs this risk. [References: 43]

Wijdicks EF and Scott JP
Outcome in patients with acute basilar artery occlusion requiring mechanical ventilation
Stroke 27:1301-3, 1996

Abstr.: BACKGROUND AND PURPOSE: Many patients with acute basilar artery occlusion may require endotracheal intubation and mechanical ventilation. The circumstances and predictive value for outcome in these patients are not well documented. METHODS: We reviewed the medical records of 25 patients admitted into the intensive care unit with a clinical diagnosis of acute basilar artery occlusion and need for mechanical ventilation. The medical records were reviewed for clinical features, breathing patterns, mode of mechanical ventilation, ability to wean from the ventilator, and neurological outcome. RESULTS: Apneic episodes resulted in endotracheal intubation in 8 patients. In the remaining 17 patients, intubation was needed for airway protection. Seven of 8 patients presenting with apneic episodes lost all brain stem reflexes. All 17 patients intubated for airway protection could be successfully weaned to a T-tube circuit. Outcome was generally poor and 22 patients died, of whom 7 died of early systemic complications. Only 3 of 25 patients, all with locked-in syndrome, survived. CONCLUSIONS: Mortality is high in patients who require mechanical ventilation after acute basilar artery occlusion. No neurological improvement beyond a locked-in syndrome occurred in survivors. Recurrent apnea appears to predict further progression to brain stem death.

Habre W, Caflisch M, Chaves-Vischer V, Delavelle J and Haenggeli CA
Locked-in syndrome in an adolescent patient with pneumococcal meningitis
Neuropediatrics 27:323-5, 1996

Abstr.: A 15-year-old adolescent with pneumococcal meningitis and increased intracranial pressure presented clinical and neurophysiological evidence of the locked-in syndrome. MRI studies showed an area of infarction involving the ventral part of the medulla.

Haig AJ, Ho KC and Ludwig G
Clinical, physiologic, and pathologic evidence for vagus dysfunction in a case of traumatic brain injury
Journal of Trauma 40:441-4, 1996

Abstr.: A trauma victim with locked-in syndrome demonstrated severely decreased bowel sounds, intact response to suppository, and elevated, but unchanging pulse. Absent cardiac response to tracheal suctioning, high gastric residual volumes, and pulmonary edema in response to a urecholine challenge demonstrated dysfunction in the automatic system. Symptoms persisted for 2 1/2 years until death. At autopsy, asymmetric bilateral involvement of the dorsal motor nucleus of the vagus and of the nerve tract in the medulla were demonstrated. In contrast, a control subject with locked-in syndrome caused by a stroke did not demonstrate these phenomena. In trauma patients with delayed gastric emptying, measurement of the heart rate response to deep suctioning may lead to the diagnosis of this vagus dysfunction syndrome.

Popova LM
[Prolonged artificial ventilation of the lungs in nervous system diseases]. [Russian]
Anesteziologiia i Reanimatologiia 2:4-9, 1996

Abstr.: Artificial ventilation of the lungs (AVL) has been carried out for many years in 25 patients with nervous diseases. Spontaneous respiration ceased because of peripheral neuron involvement in malignant myasthenia (5 cases), chronic polyneuropathy (1), paralysis of motor muscles after poliomyelitis (1), amyotrophic lateral sclerosis (11), spinopontocerebellar atrophy (1), and because of the central neuron involvement in 5 patients, 3 of these with the apnea syndrome during sleeping caused by syringobulbomyelia, atrophic process in the cerebellum and stem, and Arnold-Chiari's syndrome, and 2 with an extensive infarction in the pons (deefferentiation or locked-in syndrome). AVL was the longest in patients with paralysis of the motor muscles following acute poliomyelitis (23 years) and with amyotrophic lateral sclerosis (about 14 years). Prolonged AVL is possible in malignant myasthenia, but it may be discontinued during obligatory specific treatment. Specific features of many-year AVL, somatic functions and metabolic disorders associated with it are described.

Ueyama T, Shirataki K and Tamaki N
[Traumatic basilar artery dissection presenting with "locked-in" syndrome: report of a case]. [Japanese]
No Shinkei Geka Neurological Surgery 24:1035-9, 1996

Abstr.: We encountered a case of traumatic basilar artery dissection presenting with "locked-in" syndrome. A 46-year-old-man fell approximately 2 meters and landed in an inverted position. He was admitted to our hospital after 4 days. He was in coma on admission, and neurological examination revealed that his pupils were 2 mm in size and reactive, the corneal reflex of both sides was absent, the pharyngeal reflex was absent, and quadriplegia was present. CT and MRI showed infarction at the ventral portion of the pons. 3D-CT and CT using a bone algorithm showed fractures of the upper clivus, the left petrous bone, and the left occipital condyle. Angiography showed a pearl and string sign at the upper basilar artery. It is suggested that the cause of the infarction of the ventral portion of the pons was the occlusion of the perforating arteries due to the basilar artery dissection, and the mechanism of the basilar artery dissection was the hyperextension or hyperflexion of the basilar artery by the movement of the brain stem caused by the traumatic impact. Repeated angiography revealed gradual improvement. Eventually he freed himself from the "locked-in" syndrome.

Riva M, Brioschi AM, Ferrante E and Marazzi R
[Central pontine myelinolysis as potential complication of cerebellar astrocytoma: report of a case]. [Italian]
Annali Italiani di Medicina Interna 11:144-6, 1996

Abstr.: Central pontine myelinolysis (CPM), a rare condition first observed by Adams et al. in 1959 in a group of malnourished chronic alcoholic subjects, has subsequently been seen in patients treated with thiazide diuretics, patients hyperhydrated postoperatively, and in other clinical situations. it is characterized by quadriplegia and pseudobulbar palsy which sometimes evolves into a locked-in syndrome. The rapid correction of severe hyponatremia (> 12 mmol/L/24 h) seems to be the causal factor, with consequent osmotic edema in the richly vascularized white matter of the pons as the proposed pathogenetic mechanism. We describe the case of a chronic psychotic man with nutritional disorders and inappropriate water intake who came to our attention for a clinical picture of CPM. Neuroradiological findings and postmortem studies revealed a slow-growing cerebellar astrocytoma in addition to the typical features of CPM. We discuss the hypothesis that damage to the nervous pathways and centers involved in water and electrolyte regulation could be the causal factor of CPM pathogenesis in this case.

Heywood P, Murphy K, Corfield DR, Morrell MJ, Howard RS and Guz A
Control of breathing in man; insights from the 'locked-in' syndrome
Respiration Physiology 106:13-20, 1996

Abstr.: Control of breathing was studied in a patient with a lesion in the ventral pons; no volitional behaviour, including voluntary breathing acts, was possible (locked-in syndrome, LIS). Spontaneous breathing via a tracheostomy maintained a normal PETCO2 of 39-40 mmHg. Variability of ventilatory parameters awake was similar to that seen in five tracheostomized control subjects during stage IV sleep but much smaller than during resting wakefulness. Emotion associated with laughter caused disturbances of breathing. The ventilatory response to CO2 was normal and was associated with 'hunger for air' when the PETCO2 was 49-50 mmHg. Mechanical ventilation to reduce PETCO2 by as little as 1 mmHg resulted in apnoea when the ventilator was disconnected; breathing resumed when PETCO2 crossed the threshold of 39-40 mmHg. These results demonstrate the functional dependence of the human medullary respiratory oscillator on a threshold level of PCO2 in the absence of cortico-bulbar input, even during wakefulness. The absence of such input may explain the regularity of breathing.

Richard I, Pereon Y, Guiheneu P, Nogues B, Perrouin-Verbe B and Mathe JF
Persistence of distal motor control in the locked in syndrome. Review of 11 patients
Paraplegia 33:640-6, 1995

Abstr.: We report 11 patients with the locked in syndrome (LIS). The functional outcome was good in four patients with notable motor recovery, but motor deficit remained seriously disturbed in seven patients. All of the patients regained some distal control of finger and toe movements, often allowing functional use of a digital switch. The independence thereby gained is worthwhile, in some patients allowing environment control, communication by means of a computer, and electric wheelchair ambulation. When motor recovery occurs, the progression is disto-proximal with dramatic axial hypotonia. In five patients clinical insomnia was noted and polysomnography showed a reduction of REM sleep. The implications of systems other than the pyramidal tracts in the physiopathology of LIS are discussed.

Ejigu A, Taye M, Zenebe G and Mamo G
Locked-in syndrome (LIS). [Review] [13 refs]
Ethiopian Medical Journal 33:59-62, 1995

Abstr.: Two young female patients, who were admitted to Tikur Anbessa Hospital in September 1993 and January 1994, respectively, with locked-in syndrome are reported and literature is reviewed. [References: 13]

Recommendations for use of uniform nomenclature pertinent to patients with severe alterations in consciousness. American Congress of Rehabilitation Medicine [published erratum appears in Arch Phys Med Rehabil 1995 Apr;76(4):397] [see comments]
Archives of Physical Medicine & Rehabilitation 76:205-9, 1995

Abstr.: There continues to be considerable confusion and controversy on the use of diagnostic and clinical terms assigned to patients with severe alterations in consciousness. This confusion results largely from the lack of a uniform classification system that is based on behaviorally defined criteria. This position paper provides recommendations for defining coma, vegetative state (including persistent and permanent vegetative state), akinetic mutism, the minimally responsive state, and locked-in syndrome based on neurobehavioral and neuropathologic features. Current controversies surrounding use of these terms also are discussed.

Ockey RR, Mowry D and Varghese G
Use of Sinemet in locked-in syndrome: a report of two cases [see comments]
Archives of Physical Medicine & Rehabilitation 76:868-70, 1995

Abstr.: Locked-in syndrome describes a neurological condition usually associated with infarction of the ventral pons. Such patients are diagnosed with quadriplegia, lower cranial nerve paralysis, and mutism. The patient is alert but communication is severely limited and usually is accomplished by blinking or using vertical eye movements. The rehabilitative management of locked-in syndrome has consisted of preventative and supportive measures. In this study we report two cases of locked-in syndrome which were treated successfully with Sinemet (DuPont Pharmaceuticals, Wilmington, DE). Even though the exact neuropharmacological mechanism is unclear, the dramatic improvement in these cases, as well as in one other published case report, dictates that use of Sinemet should be considered in the management of locked-in syndrome.

Martin PJ and Young CA
Central pontine myelinolysis: clinical and MRI correlates
Postgraduate Medical Journal 71:430-2, 1995

Abstr.: Central pontine myelinolysis (CPM) is a rare condition characterised by spastic tetraparesis, pseudobulbar palsy and the 'locked-in syndrome'. It is frequently fatal. We report a patient who developed CPM secondary to profound hyponatraemia and who recovered with no disability. Serial magnetic resonance imaging (MRI) demonstrated characteristic abnormalities within the pons at the onset of the disease, whereas computerised tomography was normal. Clinical improvement was followed six months later by progressive resolution of the MRI changes, with almost complete resolution after 18 months. Clinical and MRI findings correlate early in the course of CPM but clinical recovery predates MRI improvement by several months.

Go M, Amino A, Shindo K, Tsunoda S and Shiozawa Z
[A case of central pontine myelinolysis and extrapontine myelinolysis during rapid correction of hypernatremia]. [Review] [10 refs] [Japanese]
Rinsho Shinkeigaku Clinical Neurology 34:1130-5, 1994

Abstr.: A 69-year-old woman was admitted because of severe dehydration due to anorexia. Consciousness disturbance was found to be due to severe abnormalities of serum electrolyte balance, but recovered quickly by correcting the hyperosmolality. While the initial serum sodium value of 186 mEq/L was corrected to 139 mEq/L in 5 days, locked-in syndrome, bilateral hand tremor and tetraparesis appeared. Brain magnetic resonance imaging (MRI) revealed symmetrically high signal intensity areas on T2-weighted images and low signal intensity areas on T1-weighted images in central part of pons and bilateral middle cerebellar peduncles. One and a half month later, these neurologic symptoms were improved and the MRI abnormalities also disappeared. Auditory brain stem responses which showed prolongations of III to V wave peak to peak latency at the onset returned to normal. It is noted in this case that central pontine myelinolysis (CPM) and extrapontine myelipolysis (EPM) appeared during the period of rapid correction of hypernatremia. Although it is known CPM and EPM are caused by hypernatremia or the rapid correction of hyponatremia, there has been reported only one case of CPM and EPM after rapid correction of hypernatremia. According to the hypothesis of Norenberg, rapid rise in serum sodium may cause CPM and EPM, but if CPM and EPM are caused by the rapid correction of hypernatremia in this case, CPM and EPM may be caused by another pathogenesis of the disorder. [References: 10]

Vingerhoets F and Bogousslavsky J
Respiratory dysfunction in stroke. [Review] [47 refs]
Clinics in Chest Medicine 15:729-37, 1994

Abstr.: Respiratory function depends on numerous neurologic structures, the organization of which extends from the cerebral cortex to the medulla. The study of patients who have had strokes has allowed deductions about this organization, and various neurologic pathways have been increasingly recognized. The voluntary pathway travels with the corticospinal motor tract. It is typically damaged in the "locked-in" syndrome and leads to normal automatic breathing that cannot be voluntarily altered. The automatic pathway takes its origin in the lower brainstem and is damaged mainly in lateral medullary strokes. Even a unilateral lesion here may lead to complete failure of respiration and result in death during sleep (Ondine's curse). The preservation of the limbically induced fluctuations of respiration in automatic breathing suggests a third pathway that may share its distal extent with the automatic pathway. Respiratory dysfunctions may also provide useful information regarding the cause and prognosis of stroke. [References: 47]

Blunt SB, Boulton J, Wise R, Kennard C and Lewis PD
Locked-in syndrome in fulminant demyelinating disease [letter]
Journal of Neurology, Neurosurgery & Psychiatry 57:504-5, 1994


Wang DL and Ye HH
[Care of "locked"-in syndrome due to cerebrovascular disease]. [Chinese]
Chung Hua Hu Li Tsa Chih Chinese Journal of Nursing 29:412-4, 1994


Bassetti C, Mathis J and Hess CW
Multimodal electrophysiological studies including motor evoked potentials in patients with locked-in syndrome: report of six patients
Journal of Neurology, Neurosurgery & Psychiatry 57:1403-6, 1994

Abstr.: Clinical and electrophysiological findings in six patients with locked-in syndrome are reported. Motor evoked potentials (MEPs) after magnetic stimulation of the motor cortex were absent in four patients, none of whom recovered clinically. In two patients, MEPs could be obtained from the severely paretic limbs and almost full motor recovery followed. Somatosensory evoked potentials were altered in four of the patients, and brainstem auditory evoked potentials were altered in two of four patients examined, showing a clinically unsuspected tegmental involvement. The EEG showed a predominance of reactive alpha activity in all patients, documenting a preserved consciousness. It is concluded that a multimodal electrophysiological approach, in addition to clinical assessment, can be helpful in diagnosing locked-in syndrome, estimating the extension of the underlying brainstem dysfunction, and predicting functional outcome.

Benitez JT, Ernstoff RM, Wang AM and Arsenault MD
BAER, EOG, MRI and 3-D MR angiography correlates in a case of Locked-In syndrome
Ear, Nose, & Throat Journal 73:23-4, 1994

Abstr.: A case of Locked-in syndrome is reported with correlation of BAER and EOG with MRI for localization and identification of brain lesions, and 3-D MRA and 2-D gradient echo MR imaging, a non-invasive new diagnostic modality for confirmation and visualization of vascular pathology. There was thrombosis of the basilar artery with extensive infarct of ventral pons and lower mid-brain. The patient had ocular bobbing. This eye movement in its classical form consists of irregular vertical oscillations, the fast component being downward with complete absence of horizontal eye movements. To our knowledge, the change from ocular bobbing to upbeat nystagmus has not been previously reported in the Locked-in syndrome. Bilateral involvement of the lateral lemniscus might have contributed to its appearance in our patient.

Fujiyama K, Motomura M, Shirabe S, Nakamura T, Isomoto I, Shibayama K, Nagasato K, Yoshimura T, Tsujihata M and Nagataki S
Locked-in syndrome and abnormal orientation of the right vertebral artery in a young man
Internal Medicine 33:476-80, 1994

Abstr.: A 35-year-old man developed a cerebral infarction and experienced transient ischemic attacks originating from the vertebrobasilar artery, as well as locked-in syndrome. He recovered with minimal neurological deficit. On cerebral angiography, the orientation of the right vertebral artery was markedly abnormal as it entered the foramina of the transverse process at the level of the third cervical vertebra. We concluded that the abnormal orientation of the vertebral artery caused the thrombosis and that the transient ischemic attacks, locked-in syndrome and cerebral infarction were brought about by thromboemboli originating in this artery.

Ozawa H, Tadaki T and Tagami H
Acral psoriasiform hemispherical papulosis, a new entity?
Dermatology 189:159-61, 1994

Abstr.: A 46-year-old patient with 'locked-in syndrome' due to brainstem infarction developed symmetrically distributed, grouped erythematous cornified hemispherical papules on the fingertips and toes in 2 years. Histologically they showed psoriasiform changes. There was no evidence of internal malignancy nor a history of special drug intake. To our knowledge, such an eruption has not been previously reported.

Landi A, Fornezza U, De Luca G, Marchi M and Colombo F
Brain stem and motor evoked responses in "locked-in" syndrome
Journal of Neurosurgical Sciences 38:123-7, 1994

Abstr.: A patient suffering for an ischemic "locked-in" syndrome following a subarachnoid hemorrhage, was evaluated with brain stem acoustic evoked potentials (BAEPs), short latency somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs). Neuroradiological findings failed to reveal any lesion in the brain stem, while a transcranial Doppler showed increased flow velocity upon the basilar artery, suggesting vascular spasm. BAEPs were normal and SEPs showed to be slightly impaired while MEPs upon magnetic cortical stimulation were heavily deranged. Neurophysiological investigations appeared remarkable for the early diagnosis of the disease, when neuro-radiological findings were still negative.

Ostrum AE
The 'locked-in' syndrome--comments from a survivor
Brain Injury 8:95-8, 1994


Budak F, Ilhan A, Ozmenoglu M and Komsuoglu SS
Locked-in syndrome: a case report
Clinical Electroencephalography 25:40-3, 1994

Abstr.: A 46-year-old man developed a "locked-in" syndrome due to ventral pontine infarction. MRI showed abnormal signal intensity regions in the ventral pons. The EEG, BAEPs and SEPs were all within normal limits.

Anderson C, Dillon C and Burns R
Life-sustaining treatment and locked-in syndrome [letter; comment]
Lancet 342:867-8, 1993


Sawada T
['Locked-in' syndrome]. [Review] [18 refs] [Japanese]
Nippon Rinsho Japanese Journal of Clinical Medicine 51:558-63, 1993


Dukes RR and Alexander LA
Transient locked-in syndrome after vascular injection during stellate ganglion block
Regional Anesthesia 18:378-80, 1993

Abstr.: Intravertebral artery injection of local anesthetics is a rare and alarming complication of neural blocks of the neck. Although seizures, apnea, and loss of consciousness occur, an unusual syndrome with sparing of eye movement might also occur. In transient locked-in syndrome, the patient remains conscious despite inability to breathe or move. The authors report a patient with apparent transient locked-in syndrome that resulted during a stellate ganglion block. This complication is different from frequently reported stroke-like complications. Needle position in the vertebral artery is a possible explanation for the negative aspiration of blood this patient experienced.

Latronico N, Tansini A, Gualandi GF, Gasparotti R, Bochicchio M, Chiesa A and Candiani A
Ischaemic pontomedullary transection with incomplete locked-in syndrome. A case report with MRI
Neuroradiology 35:332-4, 1993

Abstr.: We describe a case of incomplete locked-in syndrome (LIS) due to basilar artery thrombosis, in which MRI showed a complete, sharply demarcated infarct at the pontomedullary junction. This supports experimental data showing that the lower reticular formation is not critical for the maintenance of consciousness. To our knowledge, this is the first reported case of ischaemic pontomedullary transection with LIS.

Masuzawa H, Sato J, Kamitani H, Kamikura T and Aoki N
Pontine gliomas causing locked-in syndrome
Childs Nervous System 9:256-9, 1993

Abstr.: The terminal phase of pontine glioma is reportedly characterized by disturbance of consciousness. The authors retrospectively reviewed 8 children who died of pontine gliomas in their hospitals. The hospital records were analyzed specifically in regard to neurological status and terminal case. All children became mute and quadriplegic with cranial nerve palsies. The oldest child, 17 years in age, unquestionably showed the classical locked-in syndrome for the last 4 months. Six of the remaining 7 (average 5 years of age), while labeled as semicomatose, responded to calling by blinking and/or vertical eyeball movement. The authors consider that they were indeed awake in the locked-in state until very near death. This would raise a serious ethical problem of whether or not they should be intubated and kept ventilator-dependent at the time of respiratory failure, which often occurs.

Rechlin T
A communication system in cases of 'locked-in' syndrome
International Journal of Rehabilitation Research 16:340-2, 1993


Isaka Y, Iiji O, Ashida K, Imaizumi M and Itoi Y
Cerebral blood flow and magnetic resonance imaging in locked-in syndrome
Journal of Nuclear Medicine 34:291-3, 1993

Abstr.: The cerebral blood flow (CBF) of a patient suffering from locked-in syndrome (LiS) was examined before and after the onset using 99mTc-hexamethylpropyleneamine oxime single-photon emission computed tomography (SPECT) and the intravenous 133Xe injection method. The mean CBF during the locked-in state was 32.2 ml/100 g/min, a 42% reduction from the asymptomatic stage. SPECT showed profound reductions of perfusion in the bilateral cerebral cortices, subcortical regions and in the cerebellum, with a less marked reduction in the frontal cortices. On Day 49, the patient showed some minimal voluntary return with a moderate increase in mean CBF of 40.2 ml/100 g/min. The relative CBF values in the cerebral cortices and subcortical regions were restored, but the bilateral cerebellar hypoperfusion remained unchanged. SPECT and CBF are useful for a better characterization of the brain pathophysiology in LiS.

Kamondi A and Szirmai I
Topographic EEG analysis in two patients with basilar thrombosis
Clinical Electroencephalography 24:138-45, 1993

Abstr.: The EEG activity of two patients with occlusion of the vertebral and basilar arteries was analyzed. Power maps, peak power frequency, and field power differences during both photic drive and verbal command were evaluated. Clinical findings of one patient fit the criteria of locked-in syndrome. Photic stimulation and event related desynchronization paradigm was used for testing the reactivity of EEG. Averaged EEG epochs during intended movement after verbal command showed significant alpha and sub-alpha power reduction. Regional differences of EEG reactivity were assumed secondary to the underlying hemispheric infarcts. Awareness of the patient in a locked-in state was documented by EEG analysis. In the second case permanent vegetative state was associated with a nonreactive rhythmic alpha pattern. Time sequence analysis of power ratios showed spontaneous alternating activity of alpha and sub-alpha generators. The authors conclude that pseudo-periodic fluctuation of alpha activity reflects partial preservation of thalamo-cortical connections.

Truog RD
Locked-in syndrome and ethics committee deliberation [comment]
Journal of Clinical Ethics 3:209-10, 1992


Schmid C, Gulba DC, Heublein B, Kemnitz J and Haverich A
Systemic recombinant tissue plasminogen activator lysis for left atrial thrombus formation after single-lung retransplantation
Annals of Thoracic Surgery 53:338-40, 1992

Abstr.: This report describes a recipient of single-lung transplantation surviving extraordinary complications: (1) early graft failure mandating retransplantation; (2) left atrial thrombus formation, which resolved by recombinant tissue plasminogen activator lysis; (3) and development of a "locked-in-syndrome." Possible underlying mechanisms are discussed.

Echenberg RJ
Permanently locked-in syndrome in the neurologically impaired neonate: report of a case of Werdnig-Hoffmann disease [see comments]
Journal of Clinical Ethics 3:206-8, 1992


Katz RT, Haig AJ, Clark BB and DiPaola RJ
Long-term survival, prognosis, and life-care planning for 29 patients with chronic locked-in syndrome
Archives of Physical Medicine & Rehabilitation 73:403-8, 1992

Abstr.: We present a life-table analysis of a cohort of 29 locked-in syndrome (LIS) patients followed for a minimum of five years, and we report on the status of the chronic LIS patient. Twenty-nine LIS patients who remained locked-in for more than one year were identified. Inpatient charts were reviewed for demographic, medical, and functional data. Telephone followup was obtained to examine medical complications after discharge, survival, neurologic recovery, care issues, and permanent disposition. A life-table analysis was performed on survival data. Cerebrovascular disease was the most common cause of LIS. Survival ranged from 2.02 to 18.15 years. Twenty of the 26 patients available for five-year followup survived; hence, five-year survival was 81%. An alternative method of communication and emotional stress for the patient's caregiver was the key issue in patient care. Most patients were cared for in their own homes. Although minimal late neurologic recovery occurs in chronic LIS, survival may, nonetheless, be prolonged with adequate supportive care. Modern computerized technology offers LIS patients the ability to interact with their environment. This information may assist physicians in making ethical and long-term care decisions with the patient rather than for the patient with LIS.

Kponkton A, Hamonet C, Montagne A and Devailly JP
[Complications of cervical spine manipulation. A case of "locked-in syndrome" (see comments)]. [French]
Presse Medicale 21:2050-2, 1992

Abstr.: The current craze for cervical spine manipulation is due to its rapid and even dramatic results and to the ineffectiveness of many treatments prescribed by physicians. Complications of thoracic or lumbar spine manipulation are very rarely reported, but this is not the case with the cervical spine. We present the case of a woman who suffered a severely disabling complication caused by an "osteopathic" manoeuvre. This technique should be rejected as it is dangerous and as harmless methods can be used with good results in common cervicalgia, even if it is very severe.

Markus E and Reber A
[The course of rehabilitation in patients with locked-in syndrome]. [German]
Rehabilitation 31:85-90, 1992

Abstr.: On the basis of observations of the course taken by 5 patients with so-called Locked-in Syndrome (LIS), the level of functional performance that can be achieved by long-term rehabilitation notwithstanding very severe initial symptoms is set out. The priority measures in the early phases of the condition and the particular problems of long-term rehabilitation are summarized in a checklist. Technical aids to ameliorate communication are dealt with in some detail. Special importance is attributed to patient-centered psychotherapeutic support and to family involvement.

Herderschee D
Prediction of outcome in ischaemic locked-in syndrome: importance of the time of angiographic findings. [Review] [30 refs]
European Journal of Medicine 1:367-9, 1992


Kleinschmidt-DeMasters BK and Yeh M
"Locked-in syndrome" after intrathecal cytosine arabinoside therapy for malignant immunoblastic lymphoma
Cancer 70:2504-7, 1992

Abstr.: A 22-year-old man with malignant immunoblastic lymphoma had "locked-in" syndrome within 48 hours of receiving a single (100 mg) dose of intrathecal cytosine arabinoside (ara-C) in conjunction with intravenous ara-C, cisplatin, and doxorubicin. Eight hours after therapy, the patient had central hypoventilation and blurred vision that progressed to blindness within 3 hours. During the next 10 hours, he became completely quadriplegic but remained intermittently alert and was able to respond to commands by eye or head movements. Radiographic studies showed necrosis of the medulla and swelling of the entire spinal cord. The patient persisted in a locked-in state until his death 3 weeks later, after removal of life support systems. Autopsy confirmed extensive necrosis of the lower medulla, optic chiasm, cranial nerves I and IV, and spinal cord. This case was unusual for its severity. The temporal relationship to ara-C instillation favors a toxic idiosyncratic response to chemotherapy. The authors advocate caution when bolus intrathecal and intravenous ara-C are administered to a patient within a short time of each other.

Haig AJ
Locked-in syndrome [letter; comment]
Journal of Neuroscience Nursing 24:4, 1992


Sepcic J, Sepic-Grahovac D, Strenja-Grubesic J, Antonelli L and Andrasevic D
Locked-in syndrome
Neurologia Croatica 41:157-61, 1992

Abstr.: A patient, young fisherman, with a locked-in syndrome is reported, in whom intact consciousness, quadriplegia of spastic type, voluntary eye blinking, (de)sursumvergence and anarthria were observed. Thrombosis of the basilar artery and slightly disturbed bioelectrogenesis of the cerebral cortex were proved by clinical examination. The patient died after 41 days. At the autopsy thrombosis a. basilaris and ventrobasal pontine infarction were confirmed. Differential diagnosis of this and similar syndromes has been discussed.

Thadani VM, Rimm DL, Urquhart L, Fisher L, Williamson PD, Enriquez R, Kim JH and Levy LL
'Locked-in syndrome' for 27 years following a viral illness: clinical and pathologic findings [see comments]
Neurology 41:498-500, 1991

Abstr.: We describe a man who, after a presumed encephalitic illness, was "locked-in" for 27 years. His CT and autopsy findings showed atrophy of the brainstem and a cystic lesion at the base of the pons. He survived longer than most other patients in a similar state.

Ania Lafuente BJ, Hernandez Gallego J and Cubero Gonzalez A
[Locked-in syndrome (letter)]. [Spanish]
Neurologia 6:34-5, 1991


Fox C and Lavin M
Vertebral artery dissection resulting in locked-in syndrome [see comments]
Journal of Neuroscience Nursing 23:287-9, 1991

Abstr.: A 30 year-old man admitted to the hospital two weeks following a motor vehicle accident developed "locked-in" syndrome resulting from traumatic vertebral artery dissection. Aggressive nursing interventions during the acute and early recovery periods were implemented with the hope that both physical and emotional deficits might be alleviated. Although the prognosis for this type of injury is often poor, this patient has improved steadily over a 16-month period.

Herishanu YO, Abarbanel JM, Frisher S, Farkash P, Berginer J and Amir-Schechter D
Spontaneous vertical eye movements associated with pontine lesions
Israel Journal of Medical Sciences 27:320-4, 1991

Abstr.: Ocular bobbing, dipping and "reversed" ocular dipping were observed and recorded in two patients presenting a severe neurological symptomatology: quadruplegia and coma in one and locked-in syndrome in the other. CT scan showed a pontine infarction in both these patients. This is the first time that the ocular dipping and "reversed" ocular dipping are related to such an anatomic structure.

Mauss-Clum N, Cole M, McCort T and Eifler D
Locked-in syndrome: a team approach [see comments]. [Review] [12 refs]
Journal of Neuroscience Nursing 23:273-85, 1991

Abstr.: A person who suffers brainstem infarction resulting in quadriplegia, lower cranial nerve paralysis and mutism with intact cortical function may survive in a condition aptly termed "locked-in syndrome" (LIS). Literally a mind locked inside the body, the person remains fully aware without the ability to move or communicate. Because of the awesome nature of the condition and its likely permanent basis, family and staff may feel hopeless and incapacitated when planning care. Anatomical findings, etiologies, manifestations and outcomes are discussed in the literature but offer few suggestions for case management. Our clinical team has worked intensively with several LIS patients and has found that an interdisciplinary team approach is essential for providing creative patient care. [References: 12]

Jurmann MJ, Haverich A, Demertzis S, Schaefers HJ, Wagner TO and Borst HG
Extracorporeal membrane oxygenation as a bridge to lung transplantation
European Journal of Cardio Thoracic Surgery 5:94-7, 1991

Abstr.: The occurrence of severe graft failure after lung transplantation which appears refractory to conventional treatment represents a difficult situation with regard to the therapeutic strategies available. Of 17 patients undergoing single lung transplantation at our center, 2 developed early graft failure. In both, temporary artificial cardiopulmonary support by means of extracorporeal membrane oxygenation became necessary as a bridge to retransplantation. Both patients were successfully retransplanted after 8 h and 232 h, respectively, of extra-corporeal support. Postoperatively, there was a variety of complications. The first patient completely recovered from temporary severe cerebral dysfunction diagnosed as "locked-in syndrome". She was discharged from hospital on the 93rd postoperative day and remains alive and well 10 months after her operation. The other patient recovered well early after retransplantation. Later, however, airway problems developed, requiring the implantation of endotracheal stents. Cachexia and several episodes of viral pneumonia contributed to the progressive deterioration of her clinical status. She finally died after being hospitalized for 5 months after the original operation. These two cases illustrate the feasibility of using extracorporeal membrane oxygenation as a bridge to pulmonary transplantation.

Oksenberg A, Soroker N, Solzi P and Reider-Groswasser I
Polysomnography in locked-in syndrome
Electroencephalography & Clinical Neurophysiology 78:314-7, 1991

Abstr.: Sleep patterns were evaluated in a case of 'locked-in' syndrome. This patient had an ischemic infarction involving the ventral portion of the upper half of the pons bilaterally, with a posteromedial extension into the tegmentum. Reticular structures, notably the median raphe nuclei, supposed to play a major regulatory role in sleep, were most probably involved. Unexpectedly, repeated polysomnographic studies revealed sleep patterns with only minor abnormalities.

Corral I, Botia E, Orts E and Lopez F
[Transitory locked-in syndrome (letter)]. [Spanish]
Neurologia 6:34, 1991


Behr AU, Munari M, Baratto F, Dona B and Facco E
[Locked-in syndrome: value of multimodal evoked potentials]. [Italian]
Minerva Anestesiologica 57:991-2, 1991


Zanardi M, Bonamini M and Rossi M
Neuroradiological, neurophysiological and anatomopathological study of a case of locked-in syndrome
Italian Journal of Neurological Sciences 12:317-21, 1991

Abstr.: We report an anatomoclinical case of locked-in syndrome together with the neuroradiological and neurophysiological findings. MRI confirmed its value in the diagnosis of posterior cranial fossa pathology while the neurophysiological work-up (BAEPs, SEPs, MAPs and TEPs) revealed that the lesion may have broader functional implications than may be gauged from the neuroradiological and pathological evidence. Evoked potentials may therefore be of considerable importance in pinpointing brainstem lesions.

Pentore R and Nichelli P
[De-afferented state syndrome (locked-in syndrome) following sudden cervical sprain trauma during a karate training session]. [Italian]
Rivista di Neurologia 61:66-70, 1991

Abstr.: The paper describes a patient, who developed a "locked-in" syndrome following a cervical trauma occurred during karate training. The neurological picture was determined by an ischemic lesion of the ventral pontine region. The pathophysiological aspects concerning the association between cervical trauma and athletic activities are discussed.

Bockenheimer S, Reinhuber F and Mohs C
[Intra-arterial thrombolysis of vessels supplying the brain]. [German]
Radiologe 31:210-5, 1991

Abstr.: Intraarterial thrombolytic therapy can be life-saving in patients with vertebrobasilar occlusion. Thrombotic occlusions of the internal carotid artery or middle cerebral artery often result in disabling ischemic cerebral infarctions. Local fibrinolysis may help to minimize the neurological sequelae. Indications and contraindications for such a therapy are not yet well established, and more discussion is needed. During the last 2 years we have treated 18 patients with occlusions of vessels supplying the brain by means of local intraarterial thrombolytic therapy with urokinase. These included 5 patients who presented with internal carotid/middle cerebral artery occlusions, 3 of whom left the hospital with only minor neurological deficits while 2 died despite therapy. The other 13 patients had acute vertebrobasilar occlusion: 6 patients survived, 3 with a good general condition and 2 with locked-in syndrome. Technique, dosage of urokinase and patient selection are discussed as well as the outcome.

Blumbergs PC, Oatey PE, Sandhu A, Thomas AC and Simpson DA
Pontomedullary tear in a speedboat accident. Report of a case with MRI diagnosis
Zentralblatt fur Neurochirurgie 52:89-93, 1991

Abstr.: A case of atlanto-occipital dislocation with incomplete pondomedullary brainstem tear is reported; it resulted from a high-speed motorboat accident. The 43 year old man exhibited a locked-in syndrome; the diagnosis was made with MRI and verified by autopsy. With modern methods of life support, prolonged survival after such injuries is unfortunately sometimes possible.

Chia LG
Locked-in syndrome with bilateral ventral midbrain infarcts
Neurology 41:445-6, 1991

Abstr.: We report a patient who was quadriplegic and mute, but retained consciousness and communicated by eye movements and blinks. EEG and SEPs were normal. BAEPs showed slight prolongation of latencies for waves IV and V. MRI showed bilateral infarcts only in the middle and lateral portions of the cerebral peduncles.

Kurthen M, Moskopp D, Linke DB and Reuter BM
The locked-in syndrome and the behaviorist epistemology of other minds. [Review] [30 refs]
Theoretical Medicine 12:69-79, 1991

Abstr.: In this paper, the problem of correct ascriptions of consciousness to patients in neurological intensive care medicine is explored as a special case of the general philosophical 'other minds problem'. It is argued that although clinical ascriptions of consciousness and coma are mostly based on behavioral evidence, a behaviorist epistemology of other minds is not likely to succeed. To illustrate this, the so-called 'total locked-in syndrome', in which preserved consciousness is combined with a total loss of motor abilities due to a lower ventral brain stem lesion, is presented as a touchstone for behaviorism. It is argued that this example of consciousness without behavioral expression does not disprove behaviorism specifically, but rather illustrates the need for a non-verificationist theory of other minds. It is further argued that a folk version of such a theory already underlies our factual ascriptions of consciousness in clinical contexts. Finally, a non-behaviorist theory of other minds for patients with total locked-in syndrome is outlined. [References: 30]

Durrani Z and Winnie AP
Brainstem toxicity with reversible locked-in syndrome after intrascalene brachial plexus block
Anesthesia & Analgesia 72:249-52, 1991


Schmidt JA, Krause A, Feddersen CO, Kohl FV, Mariss G, Lutcke A and von Wichert P
[Central pontine myelinolysis following severe hyponatremia]. [German]
Klinische Wochenschrift 68:191-8, 1990

Abstr.: Central pontine myelinolysis is a process of demyelinisation with variable neurological symptoms related to the localization. Predisposing factors are alcoholism and malnutrition. Rapid correction of severe hyponatremia is suspected to be a primary cause for central pontine myelinolysis. We report a 43 year old chronic alcoholic and polytoxicomanic female patient, who was admitted comatose with a serum sodium level of 94 mmol/l, caused by a syndrome of inappropriate ADH secretion. After initial improvement under careful sodium correction, the patients neurologic condition degraded progressively and within 4 weeks she developed a "locked-in"-syndrome. Only then the suspected central pontine myelinolysis could be demonstrated in nuclear magnetic resonance and computer tomography. We presume that, although sodium correction was done relatively slowly in this patient, it probably contributed to her development of central pontine myelinolysis all the same. Due to this case we review the literature on correction of hyponatremia, which shows growing evidence that it should start early but be continued very slowly (rise in serum-Na: max. 0.6 mmol/l/h) and requires frequent laboratory controls.

Krieger D, Leibold M and Bruckmann H
[Dissections of the vertebral artery following cervical chiropractic manipulations]. [German]
Deutsche Medizinische Wochenschrift 115:580-3, 1990

Abstr.: In a 37-year-old female patient complaining of increasing pain in the neck and occiput, chiropractic manipulations at the cervical vertebral column were associated with ischaemias of the brain stem presenting as vertigo, transient "locked-in" syndrome followed by vomiting, and sensorimotor hemiparesis. Digital subtraction angiography (DSA) revealed complete obstruction of the right and slight dissection of the left vertebral artery. The symptoms receded within a few days after heparinisation with 1000 IU/h intravenously. A 39-year-old female patient developed vertigo, nystagmus, tetraparesis and dysarthria two days after chiropractic intervention because of refractory pain in the neck and occiput. DSA showed embolism of the basilar artery and extensive dissections of the vertebral arteries. The basilar artery was completely recanalized after local intraarterial fibrinolysis with 50,000 IU urokinase. During the further course of treatment the symptoms receded under heparin and phenprocoumon over a period of 8 months, except for hemiparesis on the left side especially affecting the arm. Trivial traumas can result in dissections of the vertebral arteries. Severe neck pain is a frequent, typical early symptom. Hence, patients with cervical vertebral column syndromes should receive chiropractic treatment only after careful diagnosis.

Morlan L, Rodriguez E, Gonzalez J, Jimene-Ortiz C, Escartin P and Liano H
Central pontine myelinolysis following correction of hyponatremia: MRI diagnosis
European Neurology 30:149-52, 1990

Abstr.: A 37-year-old woman with hepatic failure developed a locked-in syndrome after correction of a severe symptomatic hyponatremia. Magnetic resonance imaging showed a lesion involving the basis pontis and extending into the midbrain, consistent with central pontine myelinolysis. In this patient the rate of correction of hyponatremia was within the range considered sure by several authors, but factors such as hepatic encephalopathy, a single generalized seizure and correction of hyponatremia in 42 h with a change in serum sodium concentration of 34 mmol/l were present, and they could have been etiologically relevant. This case, like other recent reports, suggests new views about the pathogenesis of cerebral demyelinating lesions in patients with hyponatremia.

Sereni C and Degos CF
[Lesions of the brain stem and cerebellum of alcoholic and nutritional deficiency origin]. [French]
Revue du Praticien 40:1193-6, 1990

Abstr.: Lesions of the brain stem and cerebellum due to toxic substances or nutritional deficiencies are mostly seen in chronic alcohol abuse and, more rarely, in severe malnutrition. Four clinical entities are described: Wernicke's encephalopathy is the most frequent of these, with progressive development of disorders of consciousness, oculomotor palsies and ataxia. The condition regresses under massive vitamin BA therapy, but it often leaves neuropsychological sequelae (e. g. Korsakoff's syndrome). The best treatment is prevention by giving thiamine to alcoholics and to malnourished subjects who receive glucose infusions. Cerebellar atrophy of alcoholic origin is responsible for subacute ataxia which, as a rule, is definitive. Central pontine myelinolysis is rare and presents as sudden quadriplegia with pseudobulbar palsy and sometimes "locked-in" syndrome. Beside alcohol, a frequent cause is major ionic disorders, such as hyponatraemia or its excessively rapid correction. Pellagra is a classical disease rarely seen in this country. It is due to nicotinamide (vitamin PP) deficiency.

Arboix A and Marti-Vilalta JL
[Locked-in syndrome of reversible clinical course]. [Review] [28 refs] [Spanish]
Neurologia 5:137-9, 1990

Abstr.: A 72-year-old female developed a "locked-in" syndrome due to bilateral ventral pontine infarction. The symptoms improved 72 hours after the beginning of the clinical features. At the end of the acute phase of the disease, only a pseudobulbar syndrome of pontocerebellar type persisted. Usually, the evolution of "locked-in" syndrome is irreversible. However, our case shows that a satisfactory outcome can be possible, with a partial regression of the initially established neurological symptoms. [References: 28]

Ohry A
The locked-in syndrome and related states [editorial]
Paraplegia 28:73-5, 1990


Striano S, Luciano R, Meo R, Bilo L, Orefice G and Grasso A
[The physiopathology of supranuclear structures in oculomotor disorders]. [Review] [53 refs] [Italian]
Acta Neurologica 12:151-71, 1990

Abstr.: Several structures, located at different levels in the Central Nervous System (CNS), collaborate to the control and realization of ocular movements (O.M.) Therefore, alterations of oculomotricity, of varying degree and of different kinds, may be found in a lot of diseases of the CNS. The study of O.M., expecially when carried out by means of specific techniques such as electro-oculography, may represent an useful tool in the diagnosis (both from a topographic and an etiological standpoint) and in the follow-up of patients, possibly also allowing a precise evaluation of therapy effectiveness. In this review the Authors briefly examine the role of the supranuclear structures mostly involved in the control and in the execution of oculomotricity and the characteristics of the different types of O.M. (rapid and slow, vergence movements). Successively, they analyze the semeiological features of supra-nuclear disturbances of O.M., describing alterations of saccadic movements (slowing, dysmetria) and of smooth pursuit, fixation instability (square waves, flutter, opsoclonus, nystagmus,...) and other alterations of O.M. (ocular bobbing, see-saw nystagmus, skew deviation,...). Successively, disturbances of O.M. are analyzed in relation to their topodiagnostic significance, describing oculomotor involvements due to focal lesions of different areas of CNS (frontal cortex, parieto-occipital cortex, basal ganglia, cerebellum, brainstem) and particular syndromes (Foville syndromes, locked-in syndrome, Parinaud syndrome, anterior internuclear ophthalmoplegia, "one and a half" syndrome, Balint syndrome,...). Finally, particular attention is drawn to the oculomotor disturbances observed in degenerative cerebellar and multisystemic diseases, and several abnormal "oculomotor patterns", which seem to be specifically related to particular diseases, are described. In particular, the oculomotor patterns of Steele-Richardson-Olszewsky disease (slowing and hypometria of horizontal saccades, loss of upward saccades with preservation of reflex movements) and of Friedreich ataxia (fixation instability mostly due to the occurrence of square waves, saccadic dysmetria, impairment of smooth pursuit) are stressed. [References: 53]

Knepper L, Biller J, Adams H Jr., Yuh W, Ryals T and Godersky J
MR imaging of basilar artery occlusion
Journal of Computer Assisted Tomography 14:32-5, 1990

Abstr.: We studied six patients with basilar artery occlusion using magnetic resonance imaging. Two patients also had arteriography. All had signs consistent with pontine infarction; three had a "locked in" syndrome. Magnetic resonance imaging (five done within 24 h of onset of progression of neurologic symptoms) revealed a linear structure of increased signal intensity in the pontine cistern on T1-weighted parasagittal images and absence of flow void phenomena. Hyperintense signals at various brain stem levels corresponding to the course of the basilar artery were noted on T2-weighted images. Multiple parenchymal abnormalities were noted in the brain stem (six), cerebellum (four), occipital lobes (two), and thalamus (two). Magnetic resonance performed early in the course of basilar artery occlusion is a high-yield, safe procedure and might eliminate the need for arteriography.

Jacome DE and Morilla-Pastor D
Unreactive EEG: pattern in locked-in syndrome
Clinical Electroencephalography 21:31-6, 1990

Abstr.: Locked-in syndrome (LIS) is a state of complete paralysis except for ocular movements in a conscious individual, normally resulting from brainstem lesions. We report three patients with acute brainstem strokes and LIS whose repeated EEG recordings exhibited an "alpha coma" pattern including an unreactive alpha rhythm to multimodal stimuli. This finding is of importance since preservation of consciousness is often difficult to ascertain in LIS and presence of unreactive alpha on unresponsive patients is normally equated with vegetative states and discards LIS. Failure to diagnose LIS results in abandonment of communication efforts with these tragically affected individuals. Alpha reactivity cannot define consciousness.

Dollfus P, Milos PL, Chapuis A, Real P, Orenstein M and Soutter JW
The locked-in syndrome: a review and presentation of two chronic cases. [Review] [45 refs]
Paraplegia 28:5-16, 1990

Abstr.: The locked-in syndrome (LIS) is a state of an upper motor neurone quadriplegia involving the cranial nerve pairs with usually a lateral gaze palsy, paralytic mutism, full consciousness and awareness by the patient of his environment. A historical presentation of the LIS is given as well as a short description of the clinicoanatomic lesion causing LIS. The usual cause is vascular and corresponds to a pontine infarction due to an obstruction of the basilar artery but other lesions in the brainstem can also be the cause. Non-vascular aetiologies, especially traumatic, are reviewed. The use of electroencephalography (EEG), brain auditory evoked potentials (BAEP) and somesthesic evoked potentials (SEP) are discussed as well as the use in the acute stage of computed tomography (CT), angiography, and magnetic resonance imagery (MRI). The last method may show well delineated ischaemic lesions some time after the event. The communication disability is probably the most difficult to overcome. Two cases of LIS are presented. [References: 45]

Orenstein M, Real P, Stierle F and Fuchs C
[A post-traumatic locked-in syndrome with delayed onset]. [French]
Cahiers d Anesthesiologie 38:87-90, 1990

Abstr.: We report a case of locked-in syndrome occurring two days after a cranio-cervical trauma in a 28 years old male patient. It was a consequence of basilar thrombosis after left vertebral artery dissection secondary to a vertebral fracture at the C6 level. Decerebrate rigidity and apparent unconsciousness led to misdiagnosing it for post traumatic coma. It took several days to correct this mistake. To avoid this pitfall, we insist on the clinical features of this syndrome and discuss which investigations are needed and which therapy is available.

Iida H, Kawano N, Endo M, Saito M, Hirose R, Ohwada T and Yada K
[Blunt injury of the vertebral artery: report of three cases]. [Japanese]
No Shinkei Geka Neurological Surgery 17:1051-6, 1989

Abstr.: The authors reported three cases, whose vertebral arteries had been injured by blunt trauma to the neck which was followed by cerebello-brainstem infarctions. Case 1: a 32-year-old man, who developed severe vertigo and nausea 7 days after a traffic accident. He showed neck pain and horizontal nystagmus on admission. Three days later, he became drowsy. CT scan of the head demonstrated right-side cerebellar infarction, and the angiography revealed an occlusion of the right vertebral artery at C4-5 level. After the removal of the right cerebellar hemisphere, he recovered neurologically and was discharged from the hospital, able to walk. Case 2: a 47-year-old man, who suddenly became comatose 6 hours after an accident. Plain CT demonstrated a highly dense basilar artery. Angiography revealed the occlusion of the left vertebral artery, and severe stenosis of the right vertebral artery. The basilar artery was not visualized. Anticoagulant therapy was started immediately. He survived, but he developed locked-in syndrome. Case 3: a 53-year-old man, who developed transient apnea after an injury. On admission, neurological examination showed horizontal nystagmus, weakness of his right upper limb, and sensory disturbance in the left side of the body. Neck traction was done for spinal C1 and C2 fractures. Twenty-one hours after the injury, he became comatose suddenly. The four-vessels angiography revealed the occlusion of both vertebral arteries. The basilar artery was visualized through the posterior communicating arteries. He died on the 6th day after the trauma.(ABSTRACT TRUNCATED AT 250 WORDS)

Hayashi Y, Tachibana O, Oki H, Kadoya M and Gabata T
[MRI findings of locked-in syndrome--a case report]. [Japanese]
Rinsho Hoshasen Japanese Journal of Clinical Radiology 34:253-5, 1989

Abstr.: We report a case of Locked-in syndrome caused by thrombus in basilar artery resulting in a brainstem infarction in the ventral pons, magnetic resonance imaging (MRI) shows abnormal signal intensity regions in the ventral pons and basilar artery clearly.

Sato M, Kuroda R, Ioku M, Kim A, Tanaka S, Nakakita K, Kohama A, Fujii C, Ono S, Fukunaga M and et a
Regional cerebral blood flow in the persistent vegetative state
Neurologia Medico Chirurgica 29:389-94, 1989

Abstr.: Regional cerebral blood flow (CBF) in eight patients in a persistent vegetative state was measured and compared with that in five healthy volunteers. The patients were classified into three groups: Group 1 (locked-in syndrome) consisted of a single patient, Group 2 (typical vegetative state) of five patients, and Group 3 (prolonged coma) of two patients. CBF was measured early after onset by single photon emission computed tomography with 123I-N-isopropyl-p-iodo-amphetamine and/or 99mTc-hexamethyl-propyleneamine oxime. The regions of interest (ROIs) were the bilateral frontal, temporal, parietal, occipital, and cerebellar areas and basal ganglia. The values obtained in these areas were averaged, and the ratio for each ROI [(the value in the ROI/the mean value) x 100] was calculated. "Hyperfrontal distribution" of CBF was found to be rare in both the normal condition and the vegetative state. Higher CBF values were noted in the left than in the right frontal area in four of the five volunteers but in only four of the eight patients. CBF distribution in the frontal lobe was characteristic for each group: Group 1 showed high CBF bilaterally, although the elevation was statistically significant only on the right side, and Group 3 exhibited significantly low values. In Group 2, CBF was variable but, for the most part, within normal limits. Awareness was closely correlated with frontal lobe function and alteration of CBF in the frontal region.

Yang CC, Lieberman JS and Hong CZ
Early smooth horizontal eye movement: a favorable prognostic sign in patients with locked-in syndrome
Archives of Physical Medicine & Rehabilitation 70:230-2, 1989

Abstr.: Locked-in Syndrome (LIS) is a rare clinical entity consisting of quadriplegia, paralysis of lower cranial nerves, mutism, and bilateral paresis of horizontal gaze. The prognosis is usually poor. Most patients who do survive have severe residual impairment or develop chronic LIS. Review of the literature shows that recovery has been reported but not systematically studied, particularly with respect to early prognostic signs. We report a case of LIS with bilateral pontine infarctions shown by MRI. Severe neurological deficits included quadriplegia, paresis of horizontal gaze, facial paralysis, and bulbar palsy. Horizontal eye movements were present and smooth by the second week after onset. The patient had almost full functional motor recovery within four months, after a comprehensive rehabilitation program. Fourteen other reported cases of LIS with full recovery had documented recovery of lateral gaze in the early stage. In addition, smooth horizontal gaze is the most frequent activity in incomplete LIS. Early recovery of horizontal eye movement may indicate that the brain lesion is limited and may be a good prognostic sign in LIS.

Facco E, Caputo P, Fiore D and Giron GP
Sensorimotor and auditory central conduction time in locked-in syndrome
Electroencephalography & Clinical Neurophysiology 73:552-6, 1989

Abstr.: Motor evoked potentials (MEPs) following magnetic cortical stimulation, somatosensory evoked potentials from median nerve (SEP) and auditory brain-stem responses (ABR) were recorded in a patient with locked-in syndrome during the acute and chronic phases of the clinical course. During the acute phase both ABR and SEP were normal. The MEP from the right upper limb showed a delayed and low voltage potential, while that from the left upper limb was absent. The CT scan showed a ponto-mesencephalic hypodensity. In the chronic phase the MEP from the left upper limb had reappeared and showed a normal latency, while the one from the right upper limb worsened; at that time a new small left fronto-parietal hypodensity had appeared on CT scan. The combined use of MEP, SEP and ABR was able to confirm that the brain-stem damage involved the pyramidal but spared the lemniscal pathways, while MEP changes during the clinical course appeared to reflect both clinical and radiological findings.

Towle VL, Maselli R, Bernstein LP and Spire JP
Electrophysiologic studies on locked-in patients: heterogeneity of findings
Electroencephalography & Clinical Neurophysiology 73:419-26, 1989

Abstr.: Somatosensory evoked potentials, brain-stem auditory evoked potentials and electroencephalograms were obtained from 9 patients with the diagnosis of 'locked-in' syndrome. No pattern of evoked potential abnormality was specific to this syndrome, with findings ranging from bilaterally normal to unilaterally or bilaterally absent. The evoked potential studies complemented radiographic findings in defining the extent of the lesion and revealed that a portion of the pontine tegmentum was usually involved. Pathology from 2 patients corroborated the findings of the evoked potential studies. The value of evoked potential studies of patients with locked-in syndrome is to provide early objective evidence of brain-stem involvement independent of the clinical examination, EEG and radiographic studies.

Soria E, Fine E and Hajdu I
Auditory and somatosensory evoked potentials in a case of "locked-in" syndrome: a clinical and pathological study. [Review] [23 refs]
Electromyography & Clinical Neurophysiology 29:235-42, 1989

Abstr.: Brainstem auditory evoked potentials were bilaterally normal, and somatosensory evoked potentials were unilaterally abnormal in a patient with a large pontine infarct causing a "locked-in" syndrome. In the post mortem examination, the lesion extended unilaterally into the pontine tegmentum, partially involving the left medial lemniscus. The P14 potential was absent and the N20 potential was diminished in amplitude with right median nerve stimulation. The origin of the P14 potential has been debated in the literature. This case provides evidence for the P14 generator being located at the pontine level, in relation to a lemniscal area above the decussation of the somatosensory pathway. Evoked potentials can help to determine the tegmental extension of the pontine infarcts in the "locked-in" syndrome, especially in patients unable to cooperate with clinical examination. [References: 23]

Rao N and Costa JL
Recovery in non-vascular locked-in syndrome during treatment with Sinemet
Brain Injury 3:207-11, 1989

Abstr.: A case of successful pharmacological therapy of non-vascular locked-in syndrome (LIS) is described. A 22-year-old male experienced aqueductal stenosis following an episode of viral meningitis 11 months prior to admission. A ventriculo-peritoneal shunt was inserted and subsequently revised three times. Following the last procedure, the patient remained mute, quadriplegic and

Kenny DJ and Luke DA
'Locked-in' syndrome. Occurrence after coronary artery bypass graft surgery
Anaesthesia 44:483-4, 1989

Abstr.: The 'locked-in' syndrome has not previously been described in the immediate period after open-heart surgery. We describe the case of a patient who remained tetraplegic and mute, but alert, after graft surgery. A brief review of the aetiological factors in the development of this type of stroke after coronary artery bypass is presented.

Ohry A and Sadeh M
[Locked-in syndrome: some neurological and rehabilitational aspects]. [Review] [27 refs] [Hebrew]
Harefuah 117:383-4, 1989


Gallo UE and Fontanarosa PB
Locked-in syndrome: report of a case. [Review] [26 refs]
American Journal of Emergency Medicine 7:581-3, 1989

Abstr.: Locked-in syndrome is a unique clinical condition associated with ventral pontine lesions. The locked-in state is characterized by tetraplegia and mutism with maintenance of alertness as well as vertical eye movements and eye blinking, by which communication is possible. A case of locked-in syndrome in a 58-year-old man that demonstrates the clinical manifestations of the disorder is reported. Unless the physician is familiar with the signs and symptoms of the locked-in syndrome, the diagnosis may be missed and the patient may erroneously be considered comatose. [References: 26]

Tang XF
[Clinical and evoked potential studies in 3 cases of "locked-in" syndrome]. [Chinese]
Chung Hua Shen Ching Ching Shen Ko Tsa Chih [Chinese Journal of Neurology & Psychiatry] 22:297-300, 1989

Abstr.: Three cases of "Locked in" Syndrome diagnosed by clinical features and CT scan were studied with brainstem auditory and somatosensory evoked potentials. The results were compared with normal values from 22 healthy subjects. The results suggested that these two evoked potentials may be useful for the localization of "Locked-in" syndrome.

Griffiths ER
Spinal unit long term inpatient management of patients with severe neurological deficit
Paraplegia 27:350-3, 1989

Abstr.: Patients with the 'locked-in syndrome' are now being presented to spinal units for long term care. This is becoming a problem in spinal cord injury units, which have developed to accept spinal cord injury in the acute stage, to manage problems resulting from this, and to conduct a rehabilitation programme. A case history is presented of a patient with Guillain-Barre's recurrent syndrome, already 3 years an inpatient in a spinal unit and with minimal recovery. Problems in care are described in a patient with total bulbar palsy, paralysis of the intercostal and of the diaphragm includes inability to swallow due to bulbar paralysis with nutrition delivered by jejunostomy. The presence of a tracheostomy, as well as the bulbar and laryngeal palsy, render vocal communication impossible. Total care has been provided in a spinal unit for the past 3 years, with no complications occurring during his management. Given continuation of expert care, life expectancy may be that of many years. Is a spinal cord injury unit the proper place for long term management of cases of this nature and many similar cases described as having the 'locked-in syndrome'?

Rae-Grant AD, Lin F, Yaeger BA, Barbour P, Levitt LP, Castaldo JE and Lester MC
Post traumatic extracranial vertebral artery dissection with locked-in syndrome: a case with MRI documentation and unusually favourable outcome
Journal of Neurology, Neurosurgery & Psychiatry 52:1191-3, 1989

Abstr.: A patient with a right vertebral artery dissection occurring after minor head trauma progressed to a massive pontine infarction. An unusually favourable outcome and MRI imaging are discussed.

Maurri S, Lambruschini P and Barontini F
Total mesencephalic "locked-in" syndrome. A case report and review of the literature. [Review] [54 refs]
Rivista di Neurologia 59:211-6, 1989

Abstr.: A 63-year-old man suddenly fell in a state of apparent coma. An electroencephalogram (EEG) performed at a local hospital revealed the maintenance of a reactive alpha rhythm over the posterior regions of the scalp, allowing a diagnosis of alert coma. The persistence of minimal degrees of alertness and ability to communicate made the clinical picture classifiable as a "locked-in" syndrome (LIS). Bilateral ptosis and complete ophthalmoplegia oriented towards a total mesencephalic form of LIS. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed pathological findings at mesencephalic level. Such findings were insufficient to explain the clinical picture. [References: 54]

Fisher CM
The 'herald hemiparesis' of basilar artery occlusion [see comments]
Archives of Neurology 45:1301-3, 1988

Abstr.: In thrombosis of the basilar artery, not infrequently a hemiparesis is present at an early stage, when brain-stem signs may be absent or few, slight or equivocal. It is natural to suspect that such a hemiparesis signifies localization in a cerebral hemisphere, but unexpectedly in a few hours bilateral hemiplegia appears associated with coma or a locked-in syndrome, indicating basilar artery occlusion. I would term this disarming hemiparesis the "herald hemiparesis" of basilar artery occlusion. If the true nature of the condition is recognized early, the use of heparin and other measures may forestall disaster.

Calatayud T, Mongelos J, Fernandez Uria D and Gomez Diaz-Castroverde A
[Locked-in syndrome of mesencephalic origin (letter)]. [Spanish]
Neurologia 3:204-5, 1988


Zampella EJ, Duvall ER and Langford KH
Computed tomography and magnetic resonance imaging in traumatic locked-in syndrome
Neurosurgery 22:591-3, 1988

Abstr.: The first reported case of traumatic locked-in syndrome with computed tomographic and magnetic resonance scan confirmation of the brain stem lesion is presented. The lesion responsible for the patient's condition consisted of a hemorrhage in the ventral pontomedullary junction. The pathophysiology of the production of such lesions is discussed.

Ferbert A, Buchner H, Bruckmann H, Zeumer H and Hacke W
Evoked potentials in basilar artery thrombosis: correlation with clinical and angiographic findings
Electroencephalography & Clinical Neurophysiology 69:136-47, 1988

Abstr.: In 28 patients with vertebro-basilar or basilar artery thrombosis brain-stem auditory evoked potentials (BAEPs) and somatosensory evoked potentials (SEPs) have been recorded. Visual evoked potentials (VEPs) were recorded in 7 of these 28 patients. In 24 patients the diagnosis was angiographically proven and in 4 patients Doppler sonography and computerized tomography suggested this diagnosis. The BAEP and SEP findings were correlated to clinical and angiographical signs. BAEPs could be classified into 6 different patterns. In more than half of the patients different BAEP patterns from the two ears could be found. A pathological IV/V complex was most often found in comatose patients and in patients with a basilar artery occlusion distal to the anterior inferior cerebellar artery. Prolonged interpeak latency of I-III was mainly found in alert or drowsy patients with caudal occlusions. The frequent occurrence of a BAEP with only wave I preserved, or with no waves preserved, in patients with brain-stem functions suggests that BAEPs are not useful in the diagnosis of brain death when basilar artery thrombosis is suspected. SEPs were either absent bilaterally or else severely altered on one side in all comatose patients. In alert patients, including those with 'locked-in' syndrome, SEPs were never absent bilaterally. Increased N13-N20 interpeak latency was an uncommon finding in this series. There was no correlation between the SEP and the angiographically proven location of the occlusion. In the 'locked-in' syndrome both SEP and BAEP findings were non-uniform. Normal SEPs were sometimes found in combination with severely altered BAEPs, suggesting partial deafferentation. Since basilar artery thrombosis is now a treatable condition, early diagnosis and documentation of functional deficits moves into a more important clinical area than heretofore.

Lanotte R, Perrotin D and Ginies G
[Central pontine myelinolysis and hyponatremia]. [French]
Annales Francaises d Anesthesie et de Reanimation 7:339-42, 1988

Abstr.: Central pontine myelinolysis is a rare, but probably underreported, complication of the treatment of severe hyponatraemia. The typical presentation, with pseudobulbar palsy, quadriplegia and locked-in syndrome, made the diagnosis clinically possible in the two new cases reported. It was confirmed by computed tomography (CT). Serial CT scans in one case showed the chronological dissociation, as there was complete resolution of the pontine lucency several months after complete clinical recovery. Osmotic demyelination is suspected, but it remains unproven. Although recent evidence has suggested that too rapid a correction of severe hyponatraemia may be a possible factor, the two cases described occurred after slow correction. It would seem that rapid correction to a level of mild hyponatraemia (128-132 mmol.l-1) may be safe, reducing the morbidity of severe hyponatraemia without having central pontine myelinolysis.

Delacour JL, Floriot C, Wagschal G, Ritz A, Faller JP, Daoudal P and Noirot A
[The locked-in syndrome]. [Review] [21 refs] [French]
Annales Francaises d Anesthesie et de Reanimation 7:433-6, 1988

Abstr.: The locked-in syndrome is poorly understood, and may be misdiagnosed as coma. The syndrome consists of complete paralysis of all four limbs and the lower cranial nerve pairs, associated with an unaltered consciousness. Vertical eye movements and blinking remain intact, so allowing some form of communication by way of eye codes. Obstruction of the vertebral and basilar vascular system is the major cause, but the six cases reported show the aetiological diversity of this syndrome. If the vascular obstruction is diagnosed within the first few hours, fibrinolytic therapy may be considered. Although the patients usually die within a few days or after several months, some rare cases of recovery after several weeks have been reported. Intensive care is required by these patients (tracheostomy, artificial ventilation, intensive nursing care); the physicians and nursing staff looking after them should always keep in mind that these patients are conscious and able to communicate. [References: 21]

Frank C, Harrer G and Ladurner G
[The locked-in syndrome--experience dimensions and possibilities for an enlarged communication system]. [German]
Nervenarzt 59:337-43, 1988


Seeldrayers PA, Borenstein S, Gerard JM and Flament-Durand J
Reversible capsulo-tegmental locked-in state as first manifestation of multiple sclerosis
Journal of the Neurological Sciences 80:153-61, 1987

Abstr.: An 18-year-old girl developed a reversible locked-in state with bilateral ptosis and almost complete ophthalmoplegia. She later presented with a relapsing and remitting course suggestive of multiple sclerosis. Autopsy findings demonstrated bilateral capsular and tegmental demyelinating lesions. In addition to this unusual aetiology, this is the first report with pathological evidence of a locked-in syndrome due to lesions outside the ventral brainstem.

Barbieri F, Sinisi L, Cirillo S, Delehaje L and Mansi D
Long-term observation of two cases of locked-in syndrome with recovery. Clinical and CT-scan features
Clinical Neurology & Neurosurgery 89:177-80, 1987

Abstr.: We report clinical features of two patients suffering from Locked-In Syndrome (LIS) who made a moderate recovery, some years after the onset: a 29-year-old man, with mitral valve stenosis, and a 41-year-old diabetic woman. CT scan findings showing cerebellar and brain stem atrophy, as well as hypodensity areas in the ventral pons, are described.

Rizzi DA, Jensen P and Kjaer LU
[Locked-in syndrome. Pontine lesion visualized with magnetic resonance scanning]. [Danish]
Ugeskrift for Laeger 149:3115-6, 1987


Revesz T, Barnard RO and Lipcsey A
[Simultaneous occurrence of the locked-in syndrome and ocular bobbing apropos of 2 cases]. [Hungarian]
Orvosi Hetilap 128:2355-8, 1987


Rodriguez ML and Hankey GJ
The "locked-in" syndrome and alcoholism--a preventable complication [clinical conference]
Medical Journal of Australia 146:487-90, 1987


Garcia-Leal B, Abad-Herrera P, Juarez-Jimenez H and Estanol-Vidal B
[Tomographic findings in the "locked-in" syndrome: report of 3 cases]. [Spanish]
Revista de Investigacion Clinica 39:181-3, 1987


Povlsen UJ, Kjaer L and Arlien-Soborg P
Locked-in syndrome following cervical manipulation
Acta Neurologica Scandinavica 76:486-8, 1987

Abstr.: A case of locked-in syndrome following cervical manipulation in a 36-year-old woman is described. The patient had always been healthy but was born with polydactyly on one hand and had 2 mentally retarded children with progressive leucoencephalopathia. Because of pain in the neck the patient was treated with cervical manipulation, during which she became ill and developed a locked-in syndrome, where she was awake but mutistic and totally paralyzed. Following intensive training the patient became able to perform rocking movements with the head, which were used to activate an electronic system for communication. MR scanning 3 months after admission exhibited a sharply demarcated triangular area stretching through most of the pons, which was compatible with a recent infarction.

Calatayud T, Bermejo F, Cabello A, Escriche D, Fernandez Ortega J and Portera A
[The locked-in syndrome. Description of 8 cases and review of the literature]. [Spanish]
Archivos de Neurobiologia 50:172-82, 1987


Pearce JM
The locked in syndrome [editorial]
British Medical Journal Clinical Research Ed 294:198-9, 1987


McGuire TJ, Hodges DL, Medhat MA and Redford JB
Transient locked-in syndrome and phenobarbital
Archives of Physical Medicine & Rehabilitation 68:566-7, 1987

Abstr.: Locked-in syndrome is a rare syndrome characterized by quadriplegia, intact consciousness, mutism, and communication using vertical eye movements. We report the case history of a patient with transient locked-in syndrome following repeated shunt procedures for idiopathic aqueductal stenosis. Communication was with lateral eye movements. Although she eventually recovered and regained functional independence, high doses of phenobarbital, given to prevent seizures, sedated her and obscured her mental status during her rehabilitation. We urge the careful monitoring of all centrally acting agents in patients with locked-in syndrome.

Momma F, Sabin HI, Symon L and Branston NM
Clinical evidence supporting a subthalamic origin of the P15 wave of the somatosensory evoked potentials to median nerve stimulation
Electroencephalography & Clinical Neurophysiology 67:134-9, 1987

Abstr.: Both somatosensory (SSEPs) and brain-stem auditory evoked potentials (BAEPs) were recorded in 3 patients with localized lesions in the thalamus or brain-stem. In one patient with a right thalamic cystic tumour there was a significant interhemispheric difference in the central conduction time (CCT), but the N14-P15 interval was not affected on either side and BAEPs showed normal wave latencies and interpeak latencies (IPLs). The second patient had a colloid cyst of the third ventricle in which central herniation had occurred resulting in 'locked-in' syndrome. The P15 peak was absent on one side and markedly prolonged in latency on the other. BAEPs showed neither wave IV nor V on either side. The third patient had compression of the right posterior rostral pons by a metastatic tumour, resulting in significant prolongation of the CCT on the side of the tumour because of increase in the N14-P15 interval. Both I-V and III-V interpeak latencies were significantly prolonged bilaterally without prolongation in I-III IPL in this case. There was a correlation between the electrophysiological abnormalities of P15 and of BAEP wave V. These results suggest that the origin of the P15, which is usually recorded as a far-field potential, may be in the subthalamus or the upper part of the brain-stem.

Gomez CR, Malik MM, Wingkun E and Selhorst JB
"Locked-in" syndrome [letter]
Stroke 18:275, 1987


Haig AJ, Katz RT and Sahgal V
Mortality and complications of the locked-in syndrome
Archives of Physical Medicine & Rehabilitation 68:24-7, 1987

Abstr.: The locked-in syndrome is a severe disability consisting of quadriplegia and anarthria with preserved consciousness. No large series of cases have been reported and very few cases of long-term survival have been described. We present a follow-up of 27 patients "locked-in" for more than one year. Twenty-four were still alive up to 12.5 years after onset, with a mean survival of 4.9 years. Significant recovery was noted in only a few patients. Seventeen patients lived at home at the time of study. Eight were never hospitalized after the initial event. Gastrostomy and tracheostomy tubes and indwelling catheters were eventually removed from many patients. Electronic devices were used by ten patients to facilitate communication. We conclude that rehabilitation and medical care must be planned carefully, given the length of survival shown in this group.

Cabezudo JM, Olabe J, Lopez-Anguera A and Bacci F
Recovery from locked-in syndrome after posttraumatic bilateral distal vertebral artery occlusion
Surgical Neurology 25:185-90, 1986

Abstr.: A 55-year-old man developed a delayed locked-in syndrome after a mild head injury. Angiography showed bilateral distal vertebral artery occlusion. Anticoagulant therapy and energetic medical management to promote collateral circulation to the structures in the posterior fossa led to a functional recovery. A review of the reported cases of posttraumatic locked-in syndrome has allowed the authors to differentiate between two types: the first is due to primary brainstem injury and the second is due to secondary brainstem ischemia. Both types have different modes of onset, mechanisms of production, angiographic findings, and prognosis. It is concluded that, with early diagnosis and vigorous medical management, expectations for a useful recovery are high, especially in those cases of posttraumatic locked-in syndrome due to secondary brainstem ischemia.

Tang XF, Huang YN, Du H and Chen L
Clinical and evoked potential studies on 3 cases of "locked-in" syndrome
Proceedings of the Chinese Academy of Medical Sciences & the Peking Union Medical College 1:157-63, 1986


Cochran FF
Locked-in syndrome [letter]
Annals of Emergency Medicine 15:605-6, 1986


Popova LM and Pirogov VN
[Thrombolytic therapy with streptodekase in ischemic strokes]. [Russian]
Zhurnal Nevropatologii i Psikhiatrii Imeni S S Korsakova 86:1777-81, 1986

Abstr.: Thrombolytic agents previously employed in the treatment of ischemic stroke were associated with adverse side-effects and hemorrhagic complications. The use of streptodekase (S), a Soviet trombolytic drug, which is a long-acting immobilized ensyme proved to be highly effective. The efficacy of S in ischemic stroke was not investigated. The authors report the use of S within the first 6-8 hours after an ischemic stroke in thrombosis of the main stem of the middle meningeal artery in one case and of the vertebral and main arteries with the development of the locked-in syndrome in the other. In both cases the administration of S led to thrombolysis and restoration of the blood flow. The authors discuss the results of the use of S and indicate the necessity of the earliest possible administration of the drug with regard to a high sensitivity of the brain to ischemic lesions.

Buchman AS and Wichter MD
Recovery following the "locked-in" syndrome [letter]
Stroke 17:558, 1986


Patterson JR and Grabois M
Locked-in syndrome: a review of 139 cases
Stroke 17:758-64, 1986

Abstr.: Etiology, clinical manifestations and outcome were reviewed in 139 cases of "locked-in syndrome." Six cases were reported from our center and the remaining 133 cases were taken from a review of the literature. The results of this review emphasized the necessity for a comprehensive program of pulmonary management in this population. Furthermore, an effective system of communication for the patient is considered essential in the management of the "locked-in" state. Reported mortality in the cases reviewed was 60%. Overall, the prognosis for survival and recovery was found to be better in the group of patients whose syndrome was nonvascular in origin than those with a vascular etiology. Functional recovery was generally good in those patients with a vascular etiology who survived beyond 4 months while recovery occurred earlier and more completely in the nonvascular group. Thus, a program of intensive rehabilitation should be considered early in both groups in order to assist each patient in attaining the highest level of function possible as recovery progresses.

Tijssen CC and Ter Bruggen JP
Locked-in syndrome associated with ocular bobbing
Acta Neurologica Scandinavica 73:444-6, 1986

Abstr.: Locked-in syndrome together with an ocular bobbing phenomenon was observed in a 41-year-old man, an association which has not been reported earlier. Computerized tomography demonstrated an infarction of the medio-ventral portion of the pons. The patient survived and is still alive ten months after the onset of the disease with severe neurological deficit.

Gam J and Blichfeldt S
[Reversible locked-in syndrome in hypoglycemia]. [Danish]
Ugeskrift for Laeger 148:328-9, 1986


Nemeth G, Hegedus K and Molnar L
Akinetic mutism and locked-in syndrome: the functional-anatomical basis for their differentiation
Functional Neurology 1:128-39, 1986

Abstr.: The accurate location and extent of brainstem lesions that cause akinetic mutism (a.m.) and/or locked-in syndrome (LiS) are reviewed. We compared the data of our morphological analysis with recent neurochemical findings and speculated on the possible pathomechanism that results in the clinical state. There seems to be an anatomic correspondence between pathways of dopamine containing neurons and the lesions associated with a.m. We argue that mesocoeruleo, diencephalospinal and/or mesocorticolimbic dopaminergic systems are selectively damaged in a.m. but spared in LiS.

Keane JR
Locked-in syndrome after head and neck trauma
Neurology 36:80-2, 1986

Abstr.: Ten patients became locked-in after head and neck trauma. Five had a prompt onset due to either direct trauma or secondary infarction of the brainstem. A delay of 6 to 48 hours in four other patients was probably the interval between vertebral artery damage and vertebrobasilar arterial occlusion. The last patient sustained ischemia and compression of the cerebral peduncles from tentorial herniation.

Cordero Sanchez M, Sanchidrian Velayos I, Dominguez Moronta F and Alonso Gutierrez D
[Locked-in syndrome. Presentation of 2 new cases]. [Spanish]
Revista Clinica Espanola 179:26-7, 1986


de Graaf AS and Rybnikar MD
'Locked-in' but not 'locked-out'. A case report
South African Medical Journal 69:839-40, 1986

Abstr.: The application of modern electronic apparatus in patients with the 'locked-in' syndrome can significantly improve communication.

Erokhina LG, Gubskii LV and Khalatov VS
[Locked-in syndrome in vascular pathology of the brain]. [Russian]
Zhurnal Nevropatologii i Psikhiatrii Imeni S S Korsakova 86:1284-90, 1986

Abstr.: Seven patients with vascular abnormalities of the brain (infarct of the ventral portions of the pons varolii and/or the midbrain--5, hemorrhage into the base of the pons varolii--1 and transient impairment of the cerebral circulation in the vertebro-basilar system--presented the locked-in syndrome with such underlying disorders as tetraplegia with the maintenance of tendon and the appearance of abnormal plantar muscle reflexes, pseudobulbar syndrome with intact consciousness and the absence of any significant changes in the EEG. Other findings included disorders of eye movement, mainly in the horizontal plane and involuntary muscular activity such as forced crying, decerebrational and decorticational muscular spasms, as well as masticatory automatisms. The authors emphasize the identity of the given syndrome and Filimonov's syndrome which was repeatedly described in Soviet literature before 1966 when the term "locked-in syndrome" was proposed. They also discuss the pathogenetic mechanisms of the development of the locked-in syndrome in occlusion of the basilar artery.

Verhagen WI, Huygen PL and Schulte BP
Clinical and electrophysiological study in a patient surviving from locked-in syndrome
Clinical Neurology & Neurosurgery 88:57-61, 1986

Abstr.: The results of clinical and electrophysiological investigations in a patient with a locked-in syndrome due to a pontine infarction, mainly on the right side, are presented. EEG showed only slight disturbances, while BAER and SSER revealed response alterations as could be expected from physical examination. BAER revealed altered central conduction after stimulation of either side. After median nerve stimulation on the right side SSER was slightly delayed, while no reproducible cortical response was seen after stimulation on the left side. Our patient survived and showed partial recovery.

Mikhailidis DP, Hutton RA and Dandona P
"Locked in" syndrome following prolonged hypoglycemia [letter]
Diabetes Care 8:414, 1985


Rana PV, Malhotra RM and Narula HS
Transient brain stem dysfunction manifesting as locked-in syndrome secondary to alcoholism
Journal of the Association of Physicians of India 33:301-2, 1985


Keane JR
Locked-in syndrome with deafness [letter]
Neurology 35:1395, 1985


Keane JR and Itabashi HH
Locked-in syndrome due to tentorial herniation
Neurology 35:1647-9, 1985

Abstr.: A 28-year-old man had a chronic locked-in syndrome following tentorial herniation caused by an epidural hematoma. Postmortem examination revealed bilateral corticospinal tract degeneration caudal to the midbrain, with infarction of the right internal capsule just rostral to the cerebral peduncle and pressure necrosis of the pyramidal portion of the left cerebral peduncle.

Cappa SF, Pirovano C and Vignolo LA
Chronic 'locked-in' syndrome: psychological study of a case
European Neurology 24:107-11, 1985

Abstr.: We describe a 31-year-old man who has been living in a 'locked-in' condition for the past 12 years. Extensive testing, using both the patient's residual eye movements and a writing apparatus, showed preserved cognitive abilities in spite of the long-standing de-efferentation. The emotional consequences of this condition are discussed in relation with the results of personality testing.

Kobayashi N, Nakagawa Y, Sakuragi M, Tashiro K, Abe H, Tsuru M, Nomura M and Ueno K
[Two cases of giant fusiform aneurysm presenting ischemic attacks]. [Japanese]
No Shinkei Geka Neurological Surgery 13:919-25, 1985

Abstr.: It is said that major clinical sign of giant fusiform aneurysm is so-called mass sign compressing surrounding tissues and ischemic attack caused by the aneurysm is very rare. The authors experienced such uncommon two patients with partially thrombosed fusiform aneurysm who developed ischemic attack. Case 1: A 68-year-old man was admitted to our hospital in semicomatous state with ocular bobbing and decorticate posture of sudden onset. Enhanced CT scans taken at admission showed a presence of homogenously enhanced mass continuing to bilateral posterior cerebral arteries, which compressed the pons from right ventro-lateral side (Fig. 1 a,b). Plain CT scan taken 10 days after admission demonstrated extensive low density area in the pons (Fig. 1c). Basilar artery and P1 segment of the right posterior cerebral artery are ectatic, tortuous and elongated (Fig. 2a). The ectatic basilar artery was displaced upward and posteriorly (Fig. 2b). He developed locked-in syndrome and died of pneumonia six months after admission. At autopsy, large aneurysm measuring 3.5 X 2.0 X 1.9 cm was verified in the basilar artery (Fig. 3a). Cross section of the basilar artery aneurysm showed that laminated thrombosis nearly occupied the lumen with partially unobstructed portion (Fig. 3b). Also, horizontal section of the pons demonstrated a presence of old infarction located in the perforating arteries territory (Fig. 3c). Case 2: A 42-year-old man was initially hospitalized in 1978 because of multiple intracranial meningiomas and subtotal removal of meningiomas located in the tuberculum sellae and the foramen magnum was performed.(ABSTRACT TRUNCATED AT 250 WORDS)

Ebinger G, Huyghens L, Corne L and Aelbrecht W
Reversible "locked-in" syndromes
Intensive Care Medicine 11:218-9, 1985

Abstr.: Two young patients are described who made good recoveries from a "locked-in" syndrome presumed to be due to ventral pontine ischemia. The first patient recovered completely from quadriplegia and mutism. In the second patient the only permanent sequellae were slight dysarthria and mild spasticity. Since patients may recover nearly completely from a "locked-in" syndrome, aggressive supportive therapy seems justified during the initial weeks or months.

Ide M, Yamamoto M, Jimbo M and Enomoto N
[Cheiro-oral syndrome with pontine infarct--report of a case]. [Japanese]
No to Shinkei Brain & Nerve 37:181-6, 1985

Abstr.: A case of cheiro-oral syndrome presumably due to brain stem infarction was reported. A 41-year-old man suddenly experienced the numbness of the palm and the lips on the left side at 2 p. m. March 30, 1982 and was admitted to our clinic at 5 p.m. on that day. Neurological examination on admission revealed hypesthesia of the palm and the lips on the left side, but pain and temperature sensibilities were preserved. Neither astereognosia nor extinction phenomenon was there. CT scan showed no abnormal findings. At 9 p.m. his speaking became somewhat obscure and he became unable to swallow fluid. And after ten hours or so, he became quadriplegic, anarthric and lost almost all voluntary movement below the eye, while his consciousness was preserved ("Locked-in" syndrome). CT scan taken on the 9th hospital day revealed brain stem infarction. He expired on the 58 th hospital day because of the exacerbation of pneumonia. At autopsy both vertebral arteries and basilar artery were markedly sclerotic. The rostral two-thirds of the basilar artery was occluded by organized thrombus. The serial microscopic sections through the midbrain and the medulla oblongata showed extensive infarction of the basis pontis. The tegmentum pontis remained free, except that a small offshoot of the infarct reached into the medial part of the medial lemniscus.(ABSTRACT TRUNCATED AT 250 WORDS)

Dehaene I, Dom R, Marchau M and Geens K
Locked-in syndrome with bilateral ptosis: combination of bilateral horizontal pontine gaze paralysis and nuclear oculomotor nerve paralysis
Journal of Neurology 232:366-7, 1985

Abstr.: A patient with a classical locked-in syndrome is described, in whom bilateral ptosis was observed on the 2nd day. On the 4th day, ophthalmoplegia was complete with the exception of a voluntary downward movement of the right eye. The syndrome can be explained by the association of bilateral horizontal pontine gaze palsy and a unilateral nuclear oculomotor nerve palsy, and approximates the "total locked-in syndrome".

Tanaka K, Yokoyama M, Katayama N, Terui H and Abukawa H
[Experience in nursing of patients with locked-in syndrome--establishment of communication with the patients]. [Japanese]
Kango Gijutsu Japanese Journal of Nursing Art 31:396-402, 1985


Who speaks for the patient with the locked-in syndrome?
Hastings Center Report 15:13-5, 1985


Wu J
[Experience in the care of a patient with locked-in syndrome]. [Chinese]
Chung Hua Hu Li Tsa Chih Chinese Journal of Nursing 20:20-2, 1985


Rafalowska J, Kwiecinski H and Jamrozik Z
[Rare vascular diseases of the brain stem. The "locked-in" syndrome]. [Polish]
Neuropatologia Polska 22:509-22, 1984


Ringelstein EB, Sobczak H, Pfeifer B and Hacke W
[Polyradiculomeningoencephalitis caused by Epstein-Barr virus infection--description of a case with fatal outcome]. [German]
Fortschritte der Neurologie Psychiatrie 52:73-82, 1984

Abstr.: A 21 years old high school student, who, since early childhood, suffered from complete spinal paraplegia of unknown cause at D 10, suddenly, after a three week period with influenca -like prodromal symptoms, presented with acute polyradiculomeningoencephalitis . Positive reactions following Paul-Bunnell- Davidsohn -test and tests for specific antivirus IgG as well as specific IgM revealed acute Epstein-Barr-Virus infection, although no glandular signs could be found. The clinical findings consisted of peripheral sensory-motor tetraparesis, ataxia, nystagmus and disturbances of the caudal cranial nerves. Initially, the diagnosis of Fisher's syndrome was made. The orbicularis oculi reflex, however, indicated a circumscribed brainstem lesion in the medial reticular formation. This finding lead to the additional clinical diagnosis of encephalitis. Shortly after admission, a heart-arrest occurred, and, after successful reanimation, the patient presented with a "locked-in syndrome". On the 12th day after admission, he succumbed during a sudden drop of blood-pressure. Necropsy revealed septic shock as the cause of death, due to perforation of a duodenal ulcer. In the lower thoracic spinal cord a neurocytoma was found, which once lead to the paraplegia. Additionally, moderate inflammatory infiltrations were found in the basal leptomeninges, in the walls of several subcortical vessels and in the hypoglossal nerve. The infiltrations, at some sites, contained a large number of atypical lymphoid cells in the inflammatory meningeal exsudate . Microglial proliferation with satellitosis was found in the inferior olives, thus confirming the clinical diagnosis of polyradiculomeningoencephalitis . This case report should emphasize special features of EBV-infection with neurological complications: (1) in inflammatory diseases of the peripheral and central nervous system, infectious mononucleosis should always be drawn into the diagnostic considerations, even if heterophil antibodies and glandular symptoms are lacking. (2) In Guillain-Barre-syndrome, a transient external heart stimulator should be administered, in order to prevent heart arrest from acute autonomic neuropathy.

[Locked-in syndrome and arterial hypertension in a young woman (clinical conference)]. [Spanish]
Medicina 44:413-21, 1984


Negreiros dos Anjos M
"Locked in" syndrome following prolonged hypoglycemia [letter]
Diabetes Care 7:613, 1984


Gilsanz FJ, Manrique J, Martinez R, Alvarez Escudero J, Lora-Tamayo JI and Vaquero J
[The locked-in syndrome in the postoperative period of infratentorial surgery]. [Spanish]
Revista Espanola de Anestesiologia y Reanimacion 31:205-7, 1984


Pou-Serradel A, De Paiva VJ and Codima-Puiggros A
[Basilar trunk obstruction: report of 3 cases]. [Portuguese]
Arquivos de Neuro Psiquiatria 42:152-7, 1984

Abstr.: Three cases of complete basilar obliteration are reported, ocurring at ages arteriosclerosis is unusual. The obliteration was verified angiographically in these three cases. The evolution was quite different in each one: "restitutio ad integrum", death, and definitive persistency of a locked-in syndrome.

Virgile RS
Locked-in syndrome. Case and literature review. [Review] [33 refs]
Clinical Neurology & Neurosurgery 86:275-9, 1984

Abstr.: A case of locked-in syndrome is described and the literature is reviewed. The disorder consists of quadriplegia and mutism with preserved consciousness. The only remaining voluntary movements are usually vertical eye movements and eyelid blinking. Most cases are caused by basilar artery occlusion resulting in a brainstem infarction in the ventral pons. Health care personnel must be aware that: 1) cognition and some cutaneous sensation is intact and 2) while for most patients the prognosis is poor, there is some chance for partial to almost complete recovery. [References: 33]

Davous P, Bianco C, Duval-Lota AM, de Recondo J, Vedrenne C and Rondot P
[Aphasia caused by left paramedian thalamic infarction. Anatomo-clinical case]. [French]
Revue Neurologique 140:711-9, 1984

Abstr.: A 67 year-old right-handed man presented with an aphasia of acute onset associated to paralysis of vertical eye movements, mild cerebellar right dysmetria and right hemiparesis without hemianopia. CT scan showed two low density areas in the left thalamus and left occipital lobe. Neuropsychological examination revealed a non fluent aphasia with normal repetition, semantic paraphasias, perseverations and good comprehension of verbal and written commands. There was no alexia. Writing was impaired only by motor disturbances. Three weeks after the onset, tetraparesis and impairment of consciousness followed by a locked in syndrome supervened. Death occurred four months after onset. Neuropathological examination showed a left paramedian thalamic infarct involving the ventrolateral, dorso-medial and intralaminar nuclei, sparing the pulvinar. There was an occipital infarct sparing the calcarine scissura and multiple infarctions in the pons and the cerebellum. Broca's and Wernicke's areas were spared. We suggest that the involvement of medial nuclei could be partly responsible of language disturbances in thalamic aphasia.

Bogousslavsky J, Miklossy J, Regli F, Deruaz JP and Despland PA
One-and-a-half syndrome in ischaemic locked-in state: a clinico-pathological study
Journal of Neurology, Neurosurgery & Psychiatry 47:927-35, 1984

Abstr.: Five patients with "locked-in" syndrome and dysconjugate palsy of horizontal gaze were studied. In all cases internuclear ophthalmoplegia due to dysfunction or destruction of the median longitudinal fasciculus was combined with an ipsilateral gaze palsy, producing the "one-and-a-half" syndrome. Clinical and electro-oculographic examination suggested involvement of the paramedian pontine reticular formation when all ipsilateral saccades were abolished, when exotropia of the contralateral eye was present, and when vestibular stimulation showed full conjugate deviation to the damaged side. Involvement of the abducens nucleus was suggested when the palsy of ipsilateral gaze was not dissociated on vestibular stimulation. In three cases these clinical deductions were confirmed by the pathological study, which showed a corresponding destruction of the median longitudinal fasciculus, paramedian pontine reticular formation and abducens nucleus. In one case the one-and-a-half syndrome evolved into a total horizontal gaze palsy, which corresponded to involvement of the abducens nucleus contralateral to the initially destroyed paramedian pontine reticular formation. Vertical oculocephalic response disappeared, because of destruction of the median longitudinal fasciculus on both sides (bilateral internuclear ophthalmoplegia). Patients with the locked-in syndrome provide a unique situation in which complex pontine oculomotor disturbances may be studied, because consciousness is preserved. In these patients, dissociated and dysconjugate oculomotor palsy may have been underestimated.

Dehaene I, Dom R and Martin JJ
[The locked-in syndrome. Clinical study of 10 cases]. [French]
Acta Neurologica Belgica 84:12-20, 1984

Abstr.: Ten cases of locked-in syndrome of syndrome of ischaemic nature are described. Seven of them are conform to the original description. In the other three, the oculomor disturbances are less severe. The clinical spectrum of this syndrome is discussed, special attention being paid to the gaze palsies.

Loeb C, Mancardi GL and Tabaton M
Locked-in syndrome in acute inflammatory polyradiculoneuropathy
European Neurology 23:137-40, 1984

Abstr.: A clinicopathological study of a patient with a 'locked-in syndrome' due to an acute inflammatory polyradiculoneuropathy is reported.

Landrieu P, Fromentin C, Tardieu M, Menget A and Laget P
Locked in syndrome with a favourable outcome
European Journal of Pediatrics 142:144-5, 1984

Abstr.: The locked in syndrome seldom occurs in children, is rarely due to trauma and only in exceptional cases has a favourable outcome. The case reported below is unusual in all these respects, and its evolution might be an example of successful axonal regeneration in the central nervous system.

Minguetti G and Ratton JF
[Akinetic pseudomutism (locked-in syndrome) in accidental Chagas' disease]. [Portuguese]
Arquivos de Neuro Psiquiatria 42:59-63, 1984

Abstr.: Chagas' disease is a major problem in Central and South America. The disease is caused by Trypanosoma cruzi, a protozoon which may inhabit the blood and tissues of both man and animals. Although it may produce marked enlargement of several hollow viscera, myocardial involvement is the hallmark of chronic Chagas' disease. The clinical picture of chronic Chagas myocardites is one of insidious, progressive, prolonged congestive heart failure and cardiac enlargement. The almost invariable arrhythmias make syncope and sudden death common in patients both with p6 without congestive heart failure. Electrocardiographic abnormalities occur in 87 percent of patients with chronic Chagas' disease and are often the initial manifestation of illness . Pathological findings include mural trombosis which often results in pulmonary and central nervous system embolization. Trypanosoma cruzi is in general transmitted to human beings by the bite of haematophagus insects of the reduviidae family, but in the last years reports pointing out the importance of blood transfusions as infectious sources of Trypanosoma cruzi are becoming more and more frequent. The authors present a case of a male young architect which contrated Chagas' disease probably by a blood transfusion and latter developed "locked-in" syndrome. It is discussed the importance of blood transfusion as a source of transmission of the disease even in non-endemic areas and the need to controlling all blood transfusions in countries where Chagas' disease is endemic. The authors also point out the importance of computed tomography in the diagnosis of lesions in the pons.

Schoenmaker RT
Locked-in syndrome caused by a megadolicho vascular malformation of the basilar artery
Clinical Neurology & Neurosurgery 86:159-62, 1984

Abstr.: A 41-year-old patient is described who was initially considered to be suffering from a space-occupying lesion in the posterior fossa. He was eventually found to be suffering from a megadolicho vascular malformation of the basilar artery, which caused a locked-in syndrome. The patient survived and improved considerably.

Horn S2
The "Locked-In" syndrome following chiropractic manipulation of the cervical spine
Annals of Emergency Medicine 12:648-50, 1983

Abstr.: Reported is a case of a 34-year-old man, previously in good health, who underwent chiropractic manipulation of the cervical spine for treatment of a recent whiplash injury. Immediately following manipulation, the patient became unresponsive and was found to have sustained a brain stem infarction resulting in the "Locked-In" syndrome. Ten months following the initial insult, he remained tetraplegic and mute but able to communicate by eye blinking and vertical eye movements.

Sulkava R and Kovanen J
Locked-in syndrome with rapid recovery: a manifestation of basilar artery migraine?
Headache 23:238-9, 1983


[Locked-in syndrome--a clinical and pathological report of 3 cases]. [Chinese]
Chung Hua Shen Ching Ching Shen Ko Tsa Chih [Chinese Journal of Neurology & Psychiatry] 16:14-6, 1983


Pogacar S, Finelli PF and Lee HY
Locked-in syndrome caused by a metastasis
Rhode Island Medical Journal 66:147-50, 1983


Kobayashi K, Matsubara R, Kurachi M, Sano J, Isaki K, Yamaguchi N, Matsubara S and Nakanishi I
[Locked-in syndrome with bilateral midbrain infarcts--report of an autopsy]. [Japanese]
No to Shinkei Brain & Nerve 35:115-21, 1983

Abstr.: A case of "Locked-in" syndrome with bilateral midbrain infarcts was reported. A 51-year-old man had a memory disturbance and a change of his personality after head trauma, and then became rapidly "Locked-in" state. He was quadriplegic and bulbar paralytic, but ocular movements were preserved. Left VAG showed the narrowing of the basilar artery and there was a striking anastomosis between the SCA and PICA. Neuropathological findings were as the following; 1) The lateral two third of bilateral cerebral peduncles were extensively infarcted. 2) There were contusion of the left frontal orbital surface and the ischemic infarction of the left frontal white matter. 3) Two small infarcted lesions were found in the ventral pons without involving the pyramidal tracts. 4) Additional findings were retrograde degeneration of the medial nucleus of the left thalamus from the left frontal orbital lesion, and were loss of the Purkinje cells and neurons of pontine nucleus from the infarction of the middle cerebellar peduncle. Tegmentum of the midbrain, pons and medulla were preserved, which was considered to be due to the anastomosis of the SCA and PICA. Bilateral midbrain infarcts are responsible lesions in this case and it seems that "Locked-in" state is not synonymous with the "Ventral pontine syndrome".

Sternfeld M, Eliraz A and Pecket P
[Locked-in syndrome]. [Hebrew]
Harefuah 105:13-5, 1983


Bauer G, Gerstenbrand F and Aichner F
[Locked-in syndrome: pseudocoma from pons sections]. [German]
Psychiatrie, Neurologie und Medizinische Psychologie Beihefte 29:139-45, 1983


Bugiani O, Piola P and Tabaton M
Nontraumatic dissecting aneurysm of the basilar artery
European Neurology 22:256-60, 1983

Abstr.: A case of nontraumatic dissecting aneurysm of the basilar artery in association with hypertension, smoke, and oral contraceptives is reported in a young female patient with a locked-in syndrome.

Rana PV, Malik RK, Narayanaswamy AS and Bhattacharya K
Locked in syndrome
Journal of the Association of Physicians of India 31:378-9, 1983


Minnick A
Locked-in syndrome
Journal of Neurosurgical Nursing 15:77-9, 1983


Pamela F, Beaugerie L, Couturier M, Duval G and Gaudy JH
[Locked-in syndrome caused by thrombosis of the basilar trunk after spinal manipulation (letter)]. [French]
Presse Medicale 12:1548, 1983


Sano J, Matsubara R, Yamaguchi N, Matsubara S and Kimura A
[EEG changes in the "locked-in" syndrome]. [Japanese]
Seishin Shinkeigaku Zasshi Psychiatria et Neurologia Japonica 85:379-92, 1983


Drabek P, Stefanova J and Dostal J
[Pontine infarcts with manifestations of the "locked-in" syndrome and alpha coma]. [Czech]
Ceskoslovenska Neurologie a Neurochirurgie 46:400-5, 1983


Hernandez EI
[The locked-in syndrome (letter)]. [Spanish]
Medicina 43:117-8, 1983


Reznik M
Neuropathology in seven cases of locked-in syndrome
Journal of the Neurological Sciences 60:67-78, 1983

Abstr.: The neuropathology of 7 cases of the locked-in syndrome has been reviewed and compared with 3 cases of akinetic mutism, all of them occurring after brainstem vascular disease (9 infarctions, 1 hematoma). Including other published cases of verified locked-in syndrome, the extent of lesions has been studied in order to understand, among other symptoms, the preservation of consciousness, voluntary eye movements, auditory comprehension and vital functions. It is concluded that, at the time of autopsy, although most of the clinical signs can be explained by the pathological findings, it is not yet possible to individualize specific brainstem lesions responsible for this syndrome.

Tamura K, Karacan I, Williams RL and Meyer JS
Disturbances of the sleep-waking cycle in patients with vascular brain stem lesions
Clinical Electroencephalography 14:35-46, 1983

Abstr.: Continuous polygraphic recordings of six patients with vascular brain stem lesions were evaluated to investigate the relationship between 1) the localization or extent of the lesions and the alteration of the sleep-waking cycle, and 2) the evolution of the clinical condition and the sleep-waking cycle. Absent REM sleep and severely disorganized NREM sleep were associated with extensive pontine lesions involving the pontine tegmentum. In "locked-in" syndrome, the neural mechanisms responsible for both REM and NREM sleep continued to function, but the sleep-waking cycle was disturbed and marked by sleep-onset REM periods. Lesions in the lower brain stem resulted in absent slow-wave sleep and sleep-onset REM, suggesting that vascular lesions may involve the neural mechanisms responsible for slow-wave sleep. In general, the acute stage of illness was marked by increased awakenings and time spent awake, absent REM sleep, and little or no slow-wave sleep. These results indicate that continuous polygraphic recordings can reveal severe abnormalities due to vascular lesions of the brain stem whereas the waking EEG can only detect mild abnormalities. Improvement in the organization of the sleep-waking cycle in successive polygraphic examinations is likely to be followed by an improvement of the clinical condition. Continuous polygraphic recording is thus a valuable prognostic indicator for vascular brain stem lesions.

Belloni AR, Gallo P, Neri Serneri L and Bozzao L
[Cerebro-bulbo-spinal disconnection or "locked-in syndrome"]. [Review] [30 refs] [Italian]
Recenti Progressi in Medicina 73:380-96, 1982


Bharani AK and Mehta RS
'Locked-in' syndrome
Journal of the Association of Physicians of India 30:391-2, 1982


Seifer JG, Faccio EJ and Charchaflie RJ
[Locked-in syndrome]. [Spanish]
Medicina 42:179-84, 1982


Bauer G, Prugger M and Rumpl E
Stimulus evoked oral automatisms in the locked-in syndrome
Archives of Neurology 39:435-6, 1982


Larmande P, Henin D, Jan M, Elie A and Gouaze A
Abnormal vertical eye movements in the locked-in syndrome
Annals of Neurology 11:100-2, 1982


Hammond EJ and Wilder BJ
Short latency auditory and somatosensory evoked potentials in a patient with "locked-in" syndrome
Clinical Electroencephalography 13:54-6, 1982


Delcour C, Mouchette R, Desoignies J and Reznik M
[Locked-in syndrome]. [French]
Revue Medicale de Liege 37:465-8, 1982


Farage Filho M and Gomes MD
[Locked-in syndrome. Report of a case and review of the literature]. [Portuguese]
Arquivos de Neuro Psiquiatria 40:296-300, 1982

Abstr.: The case of a 46 year-old man who had a locked-in syndrome is reported. The patient had a haemorragic vascular accident with tetraplegia, anartria, vertical ocular movements and preservation of the consciousness. The EEG showed a projected disturbance possibly caused by a brain-stem lesion, that was confirmed by the CAT SCAN. Cerebral angiography showed high level of aterosclerosis. The lesion is located in the ventral part of the pons above the nucleus of the abducent nerve, without damaging the substantia reticularis of the mesencephalon and can be caused by thrombosis of the basilar artery and tumor, myastenia gravis, polyneurites, heroin overdose, after swine-flu inoculation and post-infectious polyneurites. The patient is still alive 2 years after the stroke with a good recovery. He can walk and speak fluently, but with cordonal ataxy of the limbs.

Dehaene I and Dom R
A mesencephalic locked-in syndrome
Journal of Neurology 227:255-9, 1982


Forti A, Ambrosetto G, Amore M, De Maria R, Michelucci R, Omicini E, Rizzuto N, Fenzi F and Tassinari CA
Locked-in syndrome in multiple sclerosis with sparing of the ventral portion of the pons
Annals of Neurology 12:393-4, 1982


Kovanen J and Sulkava R
[The "locked-in" syndrome with loss of consciousness]. [Finnish]
Duodecim 98:1079-83, 1982


Cappa SF and Vignolo LA
Locked-in syndrome for 12 years with preserved intelligence [letter]
Annals of Neurology 11:545, 1982


McCusker EA, Rudick RA, Honch GW and Griggs RC
Recovery from the 'locked-in' syndrome
Archives of Neurology 39:145-7, 1982

Abstr.: Four patients made substantial recovery following the locked-in syndrome of vascular origin. Clinical and radiologic features supported the presence of ventral pontine infarction secondary to basilar artery occlusion. Quadriplegia and mutism persisted for one to 12 weeks before recovery of motor function began. Improvement continued over several years. All patients regained functional though dysarthric speech. Three of the four patients are ambulatory, one without assistance. As a few patients make a notable recovery from the locked-in syndrome resulting from ventral pontine infarction, aggressive supportive therapy should be considered in the early months of the syndrome.

Werder M, Ruppert RK, Bajc O and Truniger B
[Central pontine myelinolysis and Schwartz-Bartter syndrome]. [German]
Schweizerische Medizinische Wochenschrift. Journal Suisse de Medecine 112:765-9, 1982

Abstr.: Central pontine myelinolysis (CPM) occurred in a typical patient with severe alcoholism presenting with severe initial hyponatremia and a rapidly progressive pontomesencephalic syndrome. A review on 141 cases of CPM is presented and the ties between CPM, hyponatremia/hypoosmolality and the syndrome of inadequate secretion of ADH are discussed. Correct interpretation of the patient's history (alcoholism, severe wasting disease) and the clinical picture (initial hypoosmolality with acute or subacute pontomesencephalic syndrome, possibly locked-in syndrome) should permit correct in-vivo diagnosis more frequently than hitherto.

Xu XH, Li SW, Tan MX and Zang X
Locked-in syndrome (pontopseudocoma): report of 3 clinical and pathologic cases
Chinese Medical Journal 94:821-6, 1981


Seales DM, Torkelson RD, Shuman RM, Rossiter VS and Spencer JD
Abnormal brainstem auditory evoked potentials and neuropathology in "locked-in" syndrome
Neurology 31:893-6, 1981

Abstr.: Brainstem auditory evoked potentials (BAEPs) were studied in a "locked-in" syndrome resulting from multiple occlusions of paramedian pontine arteries. Abnormality of BAEPs (first recorded 10 days after infarction) indicated that brainstem damage extended beyond the basis pontis, where, typically, interruption of corticospinal pathways results in the "locked-in" syndrome. Later, partial BAEP recovery suggested that the technique can be used to distinguish between permanent and transient brainstem involvement. Autopsy examination revealed close correspondence between permanent brainstem damage and persistent BAEP abnormalities. Persistent abnormal prolongation of III-V conduction time and aberration of wave IV were associated with damage near the lateral lemniscus contralateral to the stimulated ear.

Udaka F and Kameyama M
[A case of reversible "locked-in syndrome" like state due to pufferfish poisoning (author's transl)]. [Japanese]
Rinsho Shinkeigaku Clinical Neurology 21:762-6, 1981


Aimard G, Vighetto A, Confavreux C, Ventre JJ and Duquesnel J
[Diagnostic and therapeutic value of Seldinger's technique angiography in 200 neurological patients (author's transl)]. [French]
Nouvelle Presse Medicale 10:223-7, 1981

Abstr.: A retrospective study of 200 patients with various neurological diseases who had been investigated by Seldinger's technique cerebral or medullar angiography (STA) was undertaken with the view of evaluating the effective contribution of this technique to diagnosis and treatment. On the whole, STA gave positive results in 106 cases, doubtful results in 15 cases and negative, yet often useful, results in 79 cases. It proved more helpful in establishing the cause (120/200 cases) than the location (117/200 cases) of the lesions and contributed to the therapeutic decision in only 36 patients, latrogenic complications included 1 death from locked-in syndrome, 10 neurological disorders and 4 local reactions. However, the diagnostic and therapeutic value of Seldinger's technique angiography cannot be properly assessed owing to uncertainties surrounding each criterium of judgement.

Delpuech F, Habib M, Tafani B, Pellissier JF and Khalil R
[The "locked-in syndrome": report on three cases, with pathological findings in two of them (author's transl)]. [French]
Revue d Oto Neuro Ophtalmologie 53:215-24, 1981


Kawamura J, Matsubayashi K, Fukuyama H and Kitanaka H
Paired anterior spinal arteries in a case of locked-in syndrome
Neuroradiology 22:107-10, 1981

Abstr.: Paired anterior spinal arteries have rarely been demonstrated angiographically, although several anatomical studies have shown that they are not uncommonly observed. This report describes the angiographic and autopsy findings of such a variation, which was observed in a 65-year-old man with a locked-in syndrome. The paired trunks of the anterior spinal artery were visualized in a retrograde fashion through the left inferior thyroid artery and a radical branch at the 5th cervical level by left retrograde brachial angiography. The uppermost segments of either vertebral artery and the lower portion of the basilar artery were opacified through these channels. The autopsy confirmed the paired trunks of the anterior spinal artery, occlusion of the vertebral arteries just caudal to the origin of the main branches of the anterior spinal artery, and an old infarct involving the pontine tegmentum and cerebellum.

Ottomo M, Okada Y and Daidohji S
["Locked-in syndrome" after aortic valve replacement with Bjork-Shiley prosthesis--report of a case (author's transl)]. [Japanese]
Nippon Kyobu Geka Gakkai Zasshi Journal of the Japanese Association for Thoracic Surgery 29:1944-7, 1981


Takahashi K, Lee B, Ono G and Matsushita K
[Auditory evoked brain-stem response (BSR) in locked-in syndrome--an autopsy case (author's transl)]. [Japanese]
No to Shinkei Brain & Nerve 32:179-83, 1980


Goto N, Iwabuchi S and Yoshikura N
[Changes in motor nuclei and anterior horns in a case of basilar artery thrombosis with locked-in syndrome--an investigation with serial sections after a new fixation (author's transl)]. [Japanese]
No to Shinkei Brain & Nerve 32:257-64, 1980


Khurana RK, Genut AA and Yannakakis GD
Locked-in syndrome with recovery
Annals of Neurology 8:439-41, 1980

Abstr.: We report three patients who recovered from "locked-in" syndrome of presumed vascular origin. One patient narrated her experience of the "locked-in" phase and its termination. Another demonstrated palatal myoclonus and mirror movements upon recovery. These patients deserve considerate handling and vigorous medical support.

McCusker EA, Rudick RA, Honch GW and Griggs RC
Recovery from the locked-in syndrome
Transactions of the American Neurological Association 105:17-21, 1980


Bauer G, Gerstenbrand F and Hengl W
Involuntary motor phenomena in the locked-in syndrome
Journal of Neurology 223:191-8, 1980

Abstr.: Several involuntary movements were observed in patients who were totally immobile except for vertical gaze due to bilateral pyramidal transverse lesion at the pontine or midbrain level. In those conscious locked-in patients extensor spasms and flexor spasms could be elicited by nonspecific stimulation. Mimic pain reactions, pathological crying, and primordial screaming ("cat crying') were also noted. Other motor patterns were whining, moaning, groaning, sighing, and yawning. The pathophysiological implications of these observations are discussed.

Gauger GE
Communication in the locked-in syndrome
Transactions American Society for Artificial Internal Organs 26:527-9, 1980


Britt RH, Herrick MK, Mason RT and Dorfman LJ
Traumatic lesions of pontomedullary junction
Neurosurgery 6:623-31, 1980

Abstr.: An autopsied series of 162 consecutive fatal head injuries was analyzed for location and type of primary brain stem injury. Of 41 cases with primary brain stem injury, 24 (59%) demonstrated 22 tears and 2 complete transections at the level of the pontomedullary junction. Three cases are presented in detail to illustrate the range of structural and functional damage seen with trauma at this level of the brain stem. A pathologically verified case of traumatic "locked-in" syndrome is reported, including serial brain stem auditory evoked responses that are correlated with the neuropathological findings. In addition, two cases of primary traumatic damage at the pontomedullary junction (one tear and one total transection) are described; these were associated with instantaneous death at the time of injury. Pathophysiologically, the tearing or disruption occurring at this level results from rapid hyperextension, which produces stretching at the pontomedullary junction.

Prosiegel M and Konig NH
[The locked-in syndrome. A case report]. [German]
Fortschritte der Medizin 98:1489-94, 1980

Abstr.: Based on five of our own cases and the literature on hand the etiological, clinical, diagnostic and intensive therapeutic aspects of the syndrome are represented. Special emphasis is placed on the possible role of the oral contraceptives in triggering this syndrome.

Newman RP and Manning EJ
Hyperbaric chamber treatment for "locked-in' syndrome
Archives of Neurology 37:529, 1980


Reisecker F and Wagner O
[The locked-in-syndrome (author's transl)]. [German]
Wiener Medizinische Wochenschrift 129:331-3, 1979

Abstr.: On the basis of two cases some problems of the locked-in-syndrome are discussed. In most cases the cause of the syndrome is an infarct in the ventral part of the pons due to an occlusion of the vertebral or basilar artery. There is a total loss of movement exept for the movement of the eyes in all directions and the eye-closing. Although vigilance is almost not disturbed the complete loss of movement often results in a false diagnosis of cerebral coma. The EEG is an important diagnostic aid.

O'Donnell PP
'Locked-in syndrome' in postinfective polyneuropathy [letter]
Archives of Neurology 36:860, 1979


Stefan H
["Locked-in" syndrome in intensive care patients (author's transl)]. [German]
Praktische Anaesthesie, Wiederbelebung und Intensivtherapie 14:217-20, 1979

Abstr.: The "locked-in" syndrome constitutes a potential source of diagnostic and prognostic errors, especially in intensive care patients. The pathologicoanatomical, pathophysiological and clinical features of the syndrome are described and differentiated from other syndromes. Means of establishing contact are reviewed which would allow simple wishes on the part of the patient to be satisfied and thus prevent the psychic isolation of the (conscious) patient.

Bauer G, Gerstenbrand F and Rumpl E
Varieties of the locked-in syndrome
Journal of Neurology 221:77-91, 1979

Abstr.: The locked-in syndrome (LiS) was broken down on the basis of neurological symptoms in 12 patients. The criteria of classical LiS are total immobility except for vertical eye movements and blinking. If any other movements are present one should consider the condition as incomplete LiS. Total immobility, including all eye movements, combined with signs of undisturbed cortical function in the EEG led to the concept of total LiS. The anatomical basis for this condition consists of lesions in both cerebral peduncles which interrupt the pyramidal and corticobulbar tracts, the supranuclear fibers for horizontal gaze and the postnuclear oculomotor fibers. As to the course, chronic and transient LiS have been described.

Chang B and Morariu MA
Transient traumatic "locked-in" syndrome
European Neurology 18:391-4, 1979

Abstr.: The first clinical case of transient traumatic locked-in syndrome is presented. The locked-in state lasted for 3 months. The patient's neurologic deficit and the abnormal auditory evoked responses pointed to a lesion involving the left pons and ventral midbrain. The locked-in syndrome caused by a traumatic damage of the brain stem structures can be transient and thus it may have a better prognosis than the locked-in syndrome secondary to the occlusion of the basilar artery.

Murphy MJ, Brenton DW, Aschenbrener CA and Van Gilder JC
Locked-in syndrome caused by a solitary pontine abscess
Journal of Neurology, Neurosurgery & Psychiatry 42:1062-5, 1979

Abstr.: The clinical and pathological findings in a patient with locked-in syndrome caused by a solitary pontine abscess are reported for the first time. Successful treatment of brainstem abscess rests on early and accurate diagnosis.

Meienberg O, Mumenthaler M and Karbowski K
Quadriparesis and nuclear oculomotor palsy with total bilateral ptosis mimicking coma: a mesencephalic 'locked-in syndrome"?
Archives of Neurology 36:708-10, 1979

Abstr.: Upper lid and vertical eye movements are usually preserved in patients with the "locked-in" syndrome, a condition manifested by anarthria, spastic quadriparesis, and intact mental function, and caused by lesions in the ventral portion of the pons. We describe a presumed mesencephalic "locked-in" syndrome in a patient who had total bilateral ptosis at the onset of his disease and later on experienced complete bilateral oculomotor nerve paralysis.

Messert B, Orrison WW, Hawkins MJ and Quaglieri CE
Central pontine myelinolysis. Considerations on etiology, diagnosis, and treatment
Neurology 29:147-60, 1979

Abstr.: Pontine myelinolysis can be suspected clinically on the basis of the following criteria: (1) Electrolyte disturbance manifested mainly by hyponatremia; (2) progressive neurologic deficits resulting in a "locked-in" syndrome; (3) usually, but not necessarily, alcohol abuse; and (4) frequent iatrogenic precipitation of the syndrome by inappropriate rehydration of patients at risk. A major pathophysiologic mechanism for this disorder seems to be the anatomic grid structure of the base of the pons, which is more vulnerable to edema than the cerebral hemispheres. Treatment should be focused on rapid reversal of electrolyte imbalance and judicious use of dehydrating agents. Early diagnosis and treatment might reverse an otherwise malignant syndrome.

Krutchik AN, Blumenschein GR, Tashima CK and Buzdar AU
Cerebromedullospinal disconnection after intraventricular methotrexate
Southern Medical Journal 71:476-7, 1978

Abstr.: A case of cerebromedullospinal disconnection or "locked-in" syndrome after intraventricular methotrexate is described. This is an unusual complication of intraventricular methotrexate instillation. Ventral pontine infarction is postulated to be responsible for the syndrome.

Lim HS and Tong HI
Locked-in syndrome--a report of three cases
Singapore Medical Journal 19:166-8, 1978


Cherington M
Locked-in syndrome after "swine flu" inoculation [letter]
Archives of Neurology 34:258, 1977


Cummings JL and Greenberg R
Sleep patterns in the "locked-in" syndrome
Electroencephalography & Clinical Neurophysiology 43:270-1, 1977

Abstr.: Sleep patterns in two chronically "locked-in" patients were studied. The first patient had sleep activity similar to other quadriparetic patients with reduced physical activity. The second patient experienced no REM sleep and only short bursts of non-REM sleep. Clinical evidence suggested extension of his lesion dorsally into the pontine tegmentum. This correlates well with experimental evidence indicating that structures in the tegmental area are essential for normal sleep.

Flugel KA, Fuchs HH and Druschky KF
[The "locked-in" syndrome: pseudocoma in thrombosis of the basilar artery (author's transl)]. [German]
Deutsche Medizinische Wochenschrift 102:465-70, 1977

Abstr.: In pontine lesions with tetraplegia and cranial-nerve disturbances--including trismus--and with speech disability paralytic akinesia and loss of reactivity may be misinterpreted as coma. "Pseudocoma" of this kind may be associated with normal consciousness or relatively mild psychological disorders. This explains the discrepancy between a normal EEG (only minor abnormalities) and presumed unconsciousness. The term "locked-in" expresses the patient's inability to communicate. In typical cases there may be only vertical eye movements and blinking. Using remaining innervation facilities, those examining and treating the patient must try to communicate with him. The locked-in state may be mistaken for coma particularly if there had been a preceding episode of unconsciousness. Moreover, the "locked-in" syndrome often passes into a state of unconsciousness or coma. The degree of communication can markedly differ from hour to hour. Most of the cases of this syndrome are caused by basilar artery thrombosis.

Gschwend J
[Yawning in a case with transsecting glioma of the pons (author's transl)]. [German]
Fortschritte der Neurologie, Psychiatrie und Ihrer Grenzgebiete 45:652-5, 1977

Abstr.: A patient with ponsglioma got completely tetraplegic and plegic in all muscles innervated by the caudal brainstem nerves (locked-in syndrome). He could not open or close the mouth, but he was able to yawn. It is concluded that the motivation of yawning stems from the sleep inducing system in the region of the raphe-nuclei and is projected directly to the nuclei of the caudal brainstem nerves.

Marti-Vilalta JL and Roig-Arnall C
Transitory, post-traumatic "locked-in" syndrome [letter]
Lancet 2:1351-2, 1977


Gilroy J, Lynn GE, Ristow GE and Pellerin RJ
Auditory evoked brain stem potentials in a case of "locked-in" syndrome
Archives of Neurology 34:492-5, 1977

Abstr.: Auditory evoked brain stem potentials were measured in a patient with occlusion of the basilar artery about 7 mm above its origin, resulting in the "locked-in" syndrome due to infarction at the junction of the lower one third and upper two thirds of the pons. The first three waves of the evoked response originating from the acoustic nerve and auditory nuclei in the caudal pons were normal in wave form, peak latency, and voltage level. Waves IV and V, generated in the region of the lateral lemniscus and inferior colliculus in rostral pons and caudal midbrain, demonstrated prolonged peak latency and reduced voltage, indicative of slowed neural conduction in the pons above the level of the superior olivary complex. These findings suggest that auditory evoked potential recordings may have considerable value in the localization of brain stem disorders.

Kumar S, Mann KS and Gulati DR
Locked-in syndrome: a case report
Neurology India 25:45-6, 1977


Britt RH, Herrick MK and Hamilton RD
Traumatic locked-in syndrome
Annals of Neurology 1:590-2, 1977


Pagni CA, Giovanelli M, Tomei G, Zavanone M, Signoroni G and Cappricci E
Long-term results in 62 cases of post-traumatic complete apallic syndrome
Acta Neurochirurgica 36:37-45, 1977

Abstr.: After the transition state of decerebrate coma, at least four different kinds of the so-called apallic status can be identified: 1. Complete apallic syndrome: coma vigil, alertness without any awareness, mass movements only, impairment of sleep rhythm, absence of any emotional responses, postural abnormalities, some primitive motor responses, tetraplegia, and alteration of muscle tone. 2. Incomplete apallic syndrome: some of the features of the complete apallic syndrome are lacking, and the patient shows emotional reactions with appropriate grimacing and some appropriate motor responses. 3. False apallic syndrome: most signs of complete apallic syndrome are present, but the patient is in touch with the environment. This condition is somewhat similar to the so-called locked-in syndrome. 4. Functional apallic syndrome: full clinical picture of the complete apallic syndrome but full recovery within a few days. Long-term results in 62 patients, aged between 4 and 62, affected by a post-traumatic complete apallic syndrome are reported. Thirty two patients were operated upon and 30 were not operated upon. Out of these cases, 38 died after weeks or months; 3 patients entered a chronic apallic status; 2 patients are improving; 10 recovered with severe neurological or psychic sequels or both; 4 recovered with minimal sequels, and 5 without sequels; no patients in these two last groups were aged more than 20.

Baldy-Moulinier M, Billiard M, Escuret E, Roquefeuil B, Fuentes JM and Passouant P
[A polygraphic, haemodynamic and metabolic study of 2 cases of the "locked-in" syndrome (author's transl)]. [French]
Revue d Electroencephalographie et de Neurophysiologie Clinique 7:473-8, 1977

Abstr.: The sleep-waking pattern, cerebral blood flow (CBF) and cerebral metabolism have been studied in 2 cases of the "locked-in" syndrome, one traumatic, the other vascular in origin. Studies were performed in the third week in the first case and the seventh in the second. A lesion of the ventral pons was suggested clinically and neuroradiologically. The waking EEG was composed of alpha and theta activity. Different stages of slow wave sleep were diminished in quantity (NREM) as was paradoxical sleep (REM). The basal cerebral blood flow (CBF) was increased by 30 p. 100 compared to normals. Cerebral oxygen consumption was normal. The cerebral hyperaemia was evenly distributed and was associated with a failure of autoregulation. The vascular response to CO2 was normal, however. The E.E.G. findings, haemodynamic and metabolic studies confirmed a pontine lesion as the cause of the "locked-in" syndrome in contrast to akinetic mutism where the lesion is in the mid-brain.

Mannen T
[Locked-in syndrome (author's transl)]. [Japanese]
No Shinkei Geka Neurological Surgery 5:121-4, 1977


Kameyama M
[Locked-in syndrome]. [Japanese]
Nippon Rinsho Japanese Journal of Clinical Medicine 1:614-5, 1977


Reeves AG and Jenkyn LR
Letter: Eye findings in the "locked-in" syndrome
Archives of Neurology 33:215-6, 1976


Cherington M, Stears J and Hodges J
Locked-in syndrome caused by a tumor
Neurology 26:180-2, 1976

Abstr.: A case of locked-in syndrome is described. Clinical and radiologic evidence suggested the presence of a brain-stem tumor. Autopsy showed that the patient had a reticulum cell sarcoma. This is the first reported case of locked-in syndrome caused by a tumor.

Dehaene I and Martin JJ
"Locked-in" syndrome. A clinico-pathological study of two cases
European Neurology 14:81-9, 1976

Abstr.: Two new examples of "locked-in" syndrome are reported. The patients are mute and quadriplegic; they present a bilateral horizontal gaze palsy. Contact can be established through preserved vertical eye movements demonstrating therefore that the patients are not comatose. Postmortem examination reveals in both cases a thrombosis of the basilar artery with an infarction of the ventral rostral pons extending dorsally into the paramedian tegmental area. The main clinical features of this syndrome and their anatomical correlates are discussed.

Markand ON
Eectroencephalogram in "locked-in" syndrome
Electroencephalography & Clinical Neurophysiology 40:529-34, 1976

Abstr.: Twenty-three EEG studies in eight patients with "locked-in" syndrome were reviewed. The EEG was either normal or minimally slow in all except one patient. All showed reactivity to various stimuli. Photic stimulation elicited a photic driving response at one time or another in all the seven patients where it was attempted. Althouth the basic activity may be similar, it is the preservation of alertness and the presence of reactivity of the EEG which distinguishes the "locked-in" patients from those comatose due to an extensive brain stem lesion. In an unresponsive patient, an EEG resembling that of the waking state, although highly suggestive of an intrinsic brain stem insult, may also occur at times in association with diffuse cortical damage, secondary to prolonged cardio-pulmonary arrest.

Markand ON and Dyken ML
Sleep abnormalities in patients with brain stem lesions
Neurology 26:769-76, 1976

Abstr.: Seven patients with "locked-in" syndrome were studied by prolonged polygraphic recordings. Severe alterations in the sleep pattern were observed in five patients who had bilateral extensive pontine lesions resulting in tetraplegia, facial and pseudobulbar paralysis, and absence of conjugate horizontal gaze. Rapid eye movement (REM) sleep was entirely absent while non-rapid eye movement (NREM) sleep was absent, reduced, or altered. The remaining two patients, both of whom had relative sparing of horizontal gaze and apparently either no or minimal pontine tegmental involvement, showed both REM and NREM sleep with only a minimal alteration in the sleep pattern. The study suggests that, in human beings as in animals, pontine structures near the midline are essential for control of sleep states.

Hawkes CH and Bryan-Smyth L
Locked-in syndrome caused by a tumor (letter)
Neurology 26:1185-6, 1976


Golden GS, Leeds N, Kremenitzer MW and Russman BS
The "locked-in" syndrome in children
Journal of Pediatrics 89:596-8, 1976


Jennett B
Letter: "Locked-in" syndrome
British Medical Journal 1:334, 1975


Noel P and Desmedt JE
Somatosensory cerebral evoked potentials after vascular lesions of the brain-stem and diencephalon
Brain 98:113-28, 1975

Abstr.: Clinical and electrophysiological observations are described in 7 patients with clinically well-identified vascular lesions of the brain-stem or diencephalon. In the patients of Group A with a thalamic syndrome, the somatosensory cerebral evoked potentials had a reduced voltage and increased latency on the affected side. No significant anomalies were recorded in the patients of Group B with a Wallenberg or Weber syndrome. In patients of group C with a locked-in syndrome, the cerebral evoked potentials presented marked bilateral anomalies which provided interesting data about the extension of the pontine vascular lesions into the tegmentum. The pathophysiological mechanisms involved in the changes of average cerebral evoked potentials and in the slowing of corticipetal conduction are discussed.

Nomura N, Matsue M, Terao A, Araki S and Shirabe T
[Autopsy case of basilar artery thrombosis with locked-in syndrome]. [Japanese]
Rinsho Shinkeigaku Clinical Neurology 15:286-93, 1975


Al-Wardi DA, Adams AH and Hamilton AE
Four cases of "locked-in" syndrome and review of the literature
Bulletin of the Los Angeles Neurological Societies 40:60-70, 1975

Abstr.: The clinical features, pathology, the autopsy studies, EEG correlation, and the cause of death were discussed. The difference between the locked-in syndrome and classical akinetic mutism was discussed. The importance of reaching a correct diagnosis was stressed to allow a different nursing care program for the conscious "locked-in" patient. Two of our cases were short-term survival, two were long-term survival, one of whom (Case 4) is still living and is the longest survival reported in the literature.

Mikkelsen B and Tolstrup IM
[The "locked in" syndrome. Solution of the communication problem of a severely motor-handicapped patient]. [Danish]
Ugeskrift for Laeger 137:737-9, 1975


Harness B and Bental E
[The locked-in syndrome]. [Hebrew]
Harefuah 88:55-7, 1975


Valenstein E
Nonlanguage disorders of speech reflect complex neurologic apparatus
Geriatrics 30:117-21, 1975

Abstr.: The mute or nearly mute patient who is alert and has good understanding of speech and a right hemiparesis could have Broca's aphasia, akinesia of speech (transcortical motor aphasia), or aphemia. The patient who has Broca's aphasia does not write well, and his speech does not improve greatly with repetition. The speech of a patient with akinesia of speech improves with repetition. The aphemic patient writes normally, but his speech does not improve with repetition. The mute patient whose eyes are open but who is poorly responsive and moves little or not at all could be an akinetic mute (with either a cingulate or a thalamomesencephalic lesion) or have a locked-in syndrome. The latter is diagnosed by asking the patient to look up and down or to open and close his eyes. If he obeys these commands, the physician questions him using a code of eye movement responses. If the patient fails to respond at all, he is an akinetic mute; intense stimulation may result in speech or movement. If the patient is drowsy and has third nerve involvement, the lesion is in the thalamomesencephalic reticular formation. If the patient appears alert and has episodes of agitation, he probably has bilateral lesions in the gyri cinguli. Patients with weakness of the bulbar musculature (facial, palatal, and tongue weakness and dysphonia) may have either upper motor neuron or lower motor neuron lesions. Only bilateral upper motor neuron lesions produce permanent dysarthria. As a typical example, a patient has a transient left hemiparesis with dysarthria and almost completely recovers. Later, however, a right hemiparesis develops and the patient experiences severe bilateral facial weakness, drooling, dysphagia, and severe dysarthria. The absence of atrophy of the bulbar musculature, a hyperactive jaw jerk and gag reflex and, sometimes, inappropriate laughing or crying episodes indicate that the lesion is located above the medulla in the corticobulbar tracts. Flaccid paralysis, absence of the jaw jerk or gag reflex, and absence of other upper motor neuron signs, such as upgoing toes, indicate a lower motor neuron or neuromuscular junction problem. Appropriate tests to rule out myasthenia gravis should be done. The other conditions discussed here are often obvious from their clinical presentation. Although the specific disorder of speech sometimes is helpful in localizing the cause, in most patients, the associated deficits on neurologic examination are of greatest value.

Suda E, Hashiguchi K, Yamanouchi H, Tohgi H and Tomonaga M
[Clinical and pathological studies of infartion of the brain stem and locked-in syndrome]. [Japanese]
Nippon Ronen Igakkai Zasshi Japanese Journal of Geriatrics 11:402-7, 1974


Hawkes CH and Bryan-Smyth L
The electroencephalogram in the "locked-in" syndrome
Neurology 24:1015-8, 1974


Noda Y, Okayama M and Ogata J
[Case of locked-in syndrome]. [Japanese]
Rinsho Shinkeigaku Clinical Neurology 14:431-6, 1974


Hawkes CH
"Locked-in" syndrome: report of seven cases
British Medical Journal 4:379-82, 1974


Caraceni T, Broggi G and Allegranza A
[Locked-in syndrome: anatomo-clinical study]. [Italian]
Acta Neurologica 28:409-14, 1973


Davis LE, Wesley RB, Juan D and Carpenter CC
"Locked-in syndrome" from diazepam toxicity in a patient with tetanus
Lancet 1:101, 1972


Feldman MH
Physiological observations in a chronic case of "locked-in" syndrome
Neurology 21:459-78, 1971


Nordgren RE, Markesbery WR, Fukuda K and Reeves AG
Seven cases of cerebromedullospinal disconnection: the "locked-in" syndrome
Neurology 21:1140-8, 1971