[en] The Locked-In syndrome(LIS) is defined by: (i) the presence of sustained eye opening (bilateral ptosis should be ruled out as a complicating factor);(ii) preserved awareness; (iii) aphonia or hypophonia; (iv) quadriplegia or quadriparesis; and (v) a primary mode of communication that uses vertical or lateral eye movement or blinking. Acute ventral pontine lesions are its most common cause. Following such brainstem lesions patients may remain comatose for sometime and then gradually awaken, remaining paralyzed and voiceless, superficially resembling the vegetative state.
Background. – It has been shown that more than half of the time physicians fail to recognize early signs of awareness in LIS. Given appropriate medical care,life expectancy may be several decades but the chances of good motor recovery remain small. Eye-controlled computer technology now allows LIS patients to communicate and control their environment. Recent studies show that most LIS patients self-report meaningful quality of life and the demand for euthanasia is infrequent.
Conclusion. – Patients suffering from LIS should not be denied the right to die – and to die with dignity –but also they should not be denied the right to live–and to live with dignity and the best possible pain and symptom management and revalidation. [fr] Le Locked-In Syndrome (LIS) est défini par : (i) la présence d’une ouverture
continue des paupières ; (ii) des capacités cognitives relativement intactes ; (iii) une aphonie
ou hypophonie sévère ; (iv) une quadriplégie ou quadriparésie ; et (v) une communication
basée principalement sur les mouvements oculopalpébraux. L’étiologie la plus commune du
LIS est une pathologie vasculaire, soit une occlusion de l’artère basilaire, soit une hémorragie
pontique. Les personnes avec de telles lésions du tronc cérébral restent souvent dans le
coma quelque temps, puis, peu à peu, s’éveillent, mais restent paralysées et muettes, cet
état ressemblant fortement à celui des patients en état végétatif.
Etat des connaissances. – Les études récentes ont démontré que, dans plus de la moitié des
cas, la première personne à réaliser que le patient était conscient était un membre de la
famille et non pas le médecin. Lorsque le patient LIS est médicalement stable, l’espérance de
vie peut être de plusieurs décennies.
Conclusions. – Nous devrions accorder aux patients LIS le droit de mourir – avec dignité –
mais il faut également leur donner le droit de vivre dignement en leur assurant la meilleure
rééducation possible et une prise en charge optimale de la douleur et des divers symptômes.
Il est donc urgent de revoir le cadre éthique et médical de la prise en charge des patients
atteints du Locked-In Syndrome.
Disciplines :
Neurology
Author, co-author :
Bruno, Marie-Aurélie ; Université de Liège - ULiège > Centre de recherches du cyclotron
Pellas, F.; Hôpital Caremeau, CHU de Nîmes - Nîmes - France > Médecine Rééducative
Schnakers, Caroline ; Université de Liège - ULiège > Centre de recherches du cyclotron
Van Eeckhout, P.; Hôpital Pitié-Salpétrière - Paris - France > Association Locked-In Syndrome (Alis) > Départzmznr de thérapie du langage
Bernheim, J.; Free University of Brussels, VUB > Faculty of Medicine and Pharmacy > Human Ecology Department
Pantke, K. H.; Evangelischen Krankenhaus köningin Elisabeth Herzberge gGmbg Berlin > German Association Locked-In Syndrome LIS e.V.
Damas, François ; Université de Liège - ULiège > Anesthésie et réanimation
Faymonville, Marie ; Université de Liège - ULiège > Anesthésie et réanimation
Moonen, Gustave ; Université de Liège - ULiège > Département des sciences cliniques > Neurologie - Doyen de la Faculté de Médecine
Goldman, S.; Hôpital Erasme - Université Libre de Bruxelles - ULB > Unité Biomédicale PET
Laureys, Steven ; Université de Liège - ULiège > Centre de recherches du cyclotron - Département des sciences cliniques
Acharya V.Z., Talwar D., and Elliott S.P. Enteroviral encephalitis leading to a locked-in state. J Child Neurol 16 (2001) 864-866
Allain P., Joseph P.A., Isambert J.L., Le Gall D., and Emile J. Cognitive functions in chronic locked-in syndrome: a report of two cases. Cortex 34 (1998) 629-634
Allen C.M. Conscious but paralysed: releasing the locked-in. Lancet 342 (1993) 130-131
American Congress of Rehabilitation Medicine. Recommendations for use of uniform nomenclature pertinent to patients with severe alterations of consciousness. Arch Phys Med Rehabil 76 (1995) 205-209
Anderson C., Dillon C., and Burns R. Life-sustaining treatment and locked-in syndrome. Lancet 342 (1993) 867-868
Anonymous. Awareness during anaesthesia. Lancet 2 (1973) 1305
Bach J.R. Threats to "informed" advance directives for the severely physically challenged?. Arch Phys Med Rehabil 84 Suppl. 2 (2003) S23-S28
Baddeley A.D., Emslie H., and Nimmo-Smith I. Doors and people: a test of visual and verbal recall and recognition (1994), Thames Valley Test Company, Flempton, Bury St. Edmunds
Bassetti C., Mathis J., and Hess C.W. Multimodal electrophysiological studies including motor evoked potentials in patients with locked-in syndrome: report of six patients. J Neurol Neurosurg Psychiatry 57 (1994) 1403-1406
Batavia A.I. Disability and physician-assisted suicide. N Engl J Med 336 (1997) 1671-1673
Bauby J.D. Le scaphandre et le pappillon (1997), Éd. Robert Laffont, Paris
Bauer G., Gerstenbrand F., and Rumpl E. Varieties of the locked-in syndrome. J Neurol 221 (1979) 77-91
Bernat J.L., Cranford R.E., Kittredge Jr. F.I., and Rosenberg R.N. Competent patients with advanced states of permanent paralysis have the right to forgo life-sustaining therapy. Neurology 43 (1993) 224-225
Bernheim J.L. How to get serious answers to the serious question: "How have you been?": subjective quality of life (QOL) as an individual experiential emergent construct. Bioethics Q 13 (1999) 272-287
Breen P., and Hannon V. Locked-in syndrome: a catastrophic complication after surgery. Br J Anaesth 92 (2004) 286-288
Brighouse D., and Norman J. To wake in fright. BMJ 304 (1992) 1327-1328
Britt R.H., Herrick M.K., and Hamilton R.D. Traumatic locked-in syndrome. Ann Neurol 1 (1977) 590-592
Calder A.J., Lawrence A.D., and Young A.W. Neuropsychology of fear and loathing. Nat Rev Neurosci 2 (2001) 352-363
Cappa S.F., Pirovano C., and Vignolo L.A. Chronic "locked-in" syndrome: psychological study of a case. Eur Neurol 24 (1985) 107-111
Cappa S.F., and Vignolo L.A. Locked-in syndrome for 12 years with preserved intelligence. Ann Neurol 11 (1982) 545
Carroll W.M., and Mastaglia F.L. "Locked-in coma" in postinfective polyneuropathy. Arch Neurol 36 (1979) 46-47
Casanova E., Lazzari R.E., Lotta S., and Mazzucchi A. Locked-in syndrome: improvement in the prognosis after an early intensive multidisciplinary rehabilitation. Arch Phys Med Rehabil 84 (2003) 862-867
Chang B., and Morariu M.A. Transient traumatic "locked-in" syndrome. Eur Neurol 18 (1979) 391-394
Cherington M., Stears J., and Hodges J. Locked-in syndrome caused by a tumor. Neurology 26 (1976) 180-182
Christakis N.A., and Asch D.A. Biases in how physicians choose to withdraw life support. Lancet 342 (1993) 642-646
Davis L.E., Wesley R.B., Juan D., and Carpenter C.C. "Locked-in syndrome" from diazepam toxicity in a patient with tetanus. Lancet 1 (1972) 101
De Partz M.P., Bilocq V., De Wilde V., Seron X., and Pillon A. Lexis : test pour le diagnostic des troubles lexicaux chez le patient aphasique (2001), éditions Solal, Paris
Doble J.E., Haig A.J., Anderson C., and Katz R. Impairment, activity, participation, life satisfaction, and survival in persons with locked-in syndrome for over a decade: follow-up on a previously reported cohort. J Head Trauma Rehabil 18 (2003) 435-444
Dunn L.M., Thérault-Whalen C.M., and Dunn L.M. Échelles de vocabulaire en images Peabody [adaptation française du Peabody Picture Vocabulary Test-Revised (1993), Psycan ed., Toronto (Ontario)
Durrani Z., and Winnie A.P. Brainstem toxicity with reversible locked-in syndrome after intrascalene brachial plexus block. Anesth Analg 72 (1991) 249-252
Ebinger G., Huyghens L., Corne L., and Aelbrecht W. Reversible "locked-in" syndromes. Intensive Care Med 11 (1985) 218-219
Ethics and Humanities Subcommittee of the AAN. Position statement: certain aspects of the care and management of profoundly and irreversibly paralyzed patients with retained consciousness and cognition. Report of the Ethics and Humanities Subcommittee of the American Academy of Neurology. Neurology 43 (1993) 222-223
Feldman M.H. Physiological observations in a chronic case of "locked-in" syndrome. Neurology 21 (1971) 459-478
Fitzgerald L.F., Simpson R.K., and Trask T. Locked-in syndrome resulting from cervical spine gunshot wound. J Trauma 42 (1997) 147-149
Fred H.L. "Helen". South Med J 79 (1986) 1135-1136
Gallo U.E., and Fontanarosa P.B. Locked-in syndrome: report of a case. Am J Emerg Med 7 (1989) 581-583
Ghorbel S. Statut fonctionnel et qualité de vie chez le locked-in syndrome à domicile. Montpellier, France: Université Jean-Monnet Saint-Étienne (2002).
Golubovic V., Muhvic D., and Golubovic S. Posttraumatic locked-in syndrome with an unusual three day delay in the appearance. Coll Antropol 28 (2004) 923-926
Guerra M.J. Euthanasia in Spain: the public debate after Ramon Sampedro's case. Bioethics Q 13 (1999) 426-432
Gutling E., Isenmann S., and Wichmann W. Electrophysiology in the locked-in-syndrome. Neurology 46 (1996) 1092-1101
Haig A.J., Katz R.T., and Sahgal V. Mortality and complications of the locked-in syndrome. Arch Phys Med Rehabil 68 (1987) 24-27
Hall K.M., Knudsen S.T., Wright J., Charlifue S.W., Graves D.E., and Werner P. Follow-up study of individuals with high tetraplegia (C1-C4) 14 to 24 years postinjury. Arch Phys Med Rehabil 80 (1999) 1507-1513
Hawkes C.H., and Bryan-Smyth L. Locked-in syndrome caused by a tumor. Neurology 26 (1976) 1185-1186
Hayashi H., and Kato S. Total manifestations of amyotrophic lateral sclerosis. ALS in the totally locked-in state. J Neurol Sci 93 (1989) 19-35
Humbert V. Je vous demande le droit de mourir (2003), Éd. Michel Lafon, Paris
Inci S., and Ozgen T. Locked-in syndrome due to metastatic pontomedullary tumor - case report. Neurol Med Chir (Tokyo) 43 (2003) 497-500
Jacome D.E., and Morilla-Pastor D. Unreactive EEG: pattern in locked-in syndrome. Clin Electroencephalogr 21 (1990) 31-36
Katz R.T., Haig A.J., Clark B.B., and DiPaola R.J. Long-term survival, prognosis, and life-care planning for 29 patients with chronic locked-in syndrome. Arch Phys Med Rehabil 73 (1992) 403-408
Keane J.R. Locked-in syndrome after head and neck trauma. Neurology 36 1 (1986) 80-82
Kennedy P.R., and Bakay R.A. Restoration of neural output from a paralyzed patient by a direct brain connection. Neuroreport 9 (1998) 1707-1711
Kleinschmidt-DeMasters B.K., and Yeh M. "Locked-in syndrome" after intrathecal cytosine arabinoside therapy for malignant immunoblastic lymphoma. Cancer 70 (1992) 2504-2507
Kotchoubey B., Lang S., Winter S., and Birbaumer N. Cognitive processing in completely paralyzed patients with amyotrophic lateral sclerosis. Eur J Neurol 10 (2003) 551-558
Kubler A., and Neumann N. Brain-computer interfaces - the key for the conscious brain locked into a paralyzed body. Prog Brain Res 150 (2005) 513-525
Landi A., Fornezza U., De Luca G., Marchi M., and Colombo F. Brain stem and motor evoked responses in "locked-in" syndrome. J Neurosurg Sci 38 (1994) 123-127
Landrieu P., Fromentin C., Tardieu M., Menget A., and Laget P. Locked in syndrome with a favourable outcome. Eur J Pediatr 142 (1984) 144-145
Laureys S., Owen A.M., and Schiff N.D. Brain function in coma, vegetative state, and related disorders. Lancet Neurol 3 (2004) 537-546
Laureys S., Perrin F., Faymonville M.E., Schnakers C., Boly M., Bartsch V., et al. Cerebral processing in the minimally conscious state. Neurology 63 (2004) 916-918
Laureys S., Pellas F., Van Eeckhout P., et al. The locked-in syndrome: what is it like to be conscious but paralyzed and voiceless?. Prog. Brain Res. 150 (2005) 495-511
Leon-Carrion J., van Eeckhout P., and Dominguez-Morales Mdel R. The locked-in syndrome: a syndrome looking for a therapy. Brain Inj 16 (2002) 555-569
Leon-Carrion J., van Eeckhout P., Dominguez-Morales Mdel R., and Perez-Santamaria F.J. The locked-in syndrome: a syndrome looking for a therapy. Brain Inj 16 (2002) 571-582
Levy D.E., Sidtis J.J., Rottenberg D.A., et al. Differences in cerebral blood flow and glucose utilization in vegetative versus locked-in patients. Ann Neurol 22 (1987) 673-682
Lilje C.G., Heinen F., Laubenberger J., Krug I., and Brandis M. Benign course of central pontine myelinolysis in a patient with anorexia nervosa. Pediatr Neurol 27 (2002) 132-135
Loeb C., Mancardi G.L., and Tabaton M. Locked-in syndrome in acute inflammatory polyradiculoneuropathy. Eur Neurol 23 (1984) 137-140
Majerus S., Gill-Thwaites H., Andrews K., and Laureys S. Behavioral evaluation of consciousness in severe brain damage. Prog Brain Res 150 (2005) 397-413
McCusker E.A., Rudick R.A., Honch G.W., and Griggs R.C. Recovery from the "locked-in" syndrome. Arch Neurol 39 (1982) 145-147
Meienberg O., Mumenthaler M., and Karbowski K. Quadriparesis and nuclear oculomotor palsy with total bilateral ptosis mimicking coma: a mesencephalic "locked-in syndrome"?. Arch Neurol 36 (1979) 708-710
Messert B., Orrison W.W., Hawkins M.J., and Quaglieri C.E. Central pontine myelinolysis. Considerations on etiology, diagnosis, and treatment. Neurology 29 (1979) 147-160
Mikhailidis D.P., Hutton R.A., and Dandona P. "Locked in" syndrome following prolonged hypoglycemia. Diabetes Care 8 (1985) 414
Morlan L., Rodriguez E., Gonzalez J., Jimene-Ortiz C., Escartin P., and Liano H. Central pontine myelinolysis following correction of hyponatremia: MRI diagnosis. Eur Neurol 30 (1990) 149-152
Murphy M.J., Brenton D.W., Aschenbrener C.A., and Van Gilder J.C. Locked-in syndrome caused by a solitary pontine abscess. J Neurol Neurosurg Psychiatry 42 (1979) 1062-1065
Negreiros dos Anjos M. "Locked in" syndrome following prolonged hypoglycemia. Diabetes Care 7 (1984) 613
New P.W., and Thomas S.J. Cognitive impairments in the locked-in syndrome: A case report. Arch Phys Med Rehabil 86 (2005) 338-343
Oda Y., Okada Y., Nakanishi I., Kajikawa K., Kita T., Hirose G., et al. Central pontine myelinolysis with extrapontine lesions. Acta Pathol Jpn 34 (1984) 403-410
Ohry A. The locked-in syndrome and related states. Paraplegia 28 (1990) 73-75
Onofrj M., Thomas A., Paci C., Scesi M., and Tombari R. Event related potentials recorded in patients with locked-in syndrome. J Neurol Neurosurg Psychiatry 63 (1997) 759-764
Pantke K.H. Locked-in. Gefangen im eigenen Körper (1999), Mabuse Verlag, Frankfurt
Patterson J.R., and Grabois M. Locked-in syndrome: a review of 139 cases. Stroke 17 (1986) 758-764
Pecket P., Landau Z., and Resnitzky P. Reversible locked-in state in postinfective measles encephalitis. Arch Neurol 39 (1982) 672
Peduto V.A., Silvetti L., and Piga M. An anesthetized anesthesiologist tells his experience of waking up accidentally during the operation. Minerva Anestesiol 60 (1994) 1-5
Perrin F., Garcia-Larrea L., Mauguiere F., and Bastuji H. A differential brain response to the subject's own name persists during sleep. Clin Neurophysiol 110 (1999) 2153-2164
Plum F., and Posner B. The diagnosis of stupor and coma. 1st ed. (1966), Davis FA, Philadelphia
Plum F., and Posner B. The diagnosis of stupor and coma. 3rd ed. (1983), Davis FA, Philadelphia
Pogacar S., Finelli P.F., and Lee H.Y. Locked-in syndrome caused by a metastasis. R I Med J 66 (1983) 147-150
Rae-Grant A.D., Lin F., Yaeger B.A., Barbour P., Levitt L.P., Castaldo J.E., et al. Post traumatic extracranial vertebral artery dissection with locked-in syndrome: a case with MRI documentation and unusually favourable outcome. J Neurol Neurosurg Psychiatry 52 (1989) 1191-1193
Ragazzoni A., Grippo A., Tozzi F., and Zaccara G. Event-related potentials in patients with total locked-in state due to fulminant Guillain-Barre syndrome. Int J Psychophysiol 37 (2000) 99-109
Richard I., Pereon Y., Guiheneu P., Nogues B., Perrouin-Verbe B., and Mathe J.F. Persistence of distal motor control in the locked in syndrome. Review of 11 patients. Paraplegia 33 (1995) 640-646
Sandin R.H., Enlund G., Samuelsson P., and Lennmarken C. Awareness during anaesthesia: a prospective case study. Lancet 355 (2000) 707-711
Schnakers C, Majerus S, Goldman S, et al. (In press). Cognitive function in the locked in syndrome. J Neurol.
Sigalovsky N. Awareness under general anesthesia. AANA J 71 5 (2003) 373-379
Steffen G.E., and Franklin C. Who speaks for the patient with the locked-in syndrome?. Hastings Cent Rep 15 (1985) 13-15
Stumpf S.E. A comment on "Helen". South Med J 79 (1986) 1057-1058
Stuss D.T., Levine B., Alexander M.P., Hong J., Palumbo C., Hamer L., et al. Wisconsin Card Sorting Test performance in patients with focal frontal and posterior brain damage: effects of lesion location and test structure on separable cognitive processes. Neuropsychologia 38 (2000) 388-402
Tavalaro J., and Tayson R. Look up for yes (1997), Kodansha America, Inc, New York, NY
Thadani V.M., Rimm D.L., Urquhart L., Fisher L., Williamson P.D., Enriquez R., et al. "Locked-in syndrome" for 27 years following a viral illness: clinical and pathologic findings. Neurology 41 (1991) 498-500
Towle V.L., Maselli R., Bernstein L.P., and Spire J.P. Electrophysiologic studies on locked-in patients: heterogeneity of findings. Electroencephalogr Clin Neurophysiol 73 (1989) 419-426
Trail M., Nelson N.D., Van J.N., Appel S.H., and Lai E.C. A study comparing patients with amyotrophic lateral sclerosis and their caregivers on measures of quality of life, depression, and their attitudes toward treatment options. J Neurol Sci 209 (2003) 79-85
van Eeckhout P. Le locked-in syndrome. Rééducation Orthophonique 35 (1997) 123-135
Vigand P., and Vigand S. Only the eyes say yes (original title: Putain de silence) (2000), Arcade Publishing
Vigand P. Promenade immobiles (2002), Le livre de poche, Paris
Vigand P., and Vigand S. Putain de silence (1997), Anne Carrière, Paris
Ware J.E., Snow K.K., and Kosinski M. SF-36 Health survey manual and interpretation guide (1993), The Health Institute, New England Medical Center, Boston, MA
Wechsler D. MEM-III : échelle clinique de mémoire de Wechsler (2001), ECPA éd, Paris
Zimmermann P., and Fimm B. Tests d'évaluation de l'attention (TEA) (1994), Psytest éd., Würselen
