Locked-in syndrome in children: report of five cases and review of the literature

[en] The locked-in syndrome is a rare neurologic disorder defined by (1) the presence of sustained eye opening; (2) preserved awareness; (3) aphonia or hypophonia; (4) quadriplegia or quadriparesis; and (5) a primary mode of communication that uses vertical or lateral eye movement or blinking. Five cases are reported here, and previous literature is reviewed. According to the literature, the most common etiology of locked-in syndrome in children is ventral pontine stroke, most frequently caused by a vertebrobasilar artery thrombosis or occlusion. In terms of prognosis, 35% of pediatric locked-in syndrome patients experienced some motor recovery, 26% had good recovery, 23% died, and 16% remained quadriplegic and anarthric. These findings raise important ethical considerations in terms of quality of life and end-of-life decisions in such challenging cases.

Disciplines :

Neurology

Author, co-author :

Bruno, Marie-Aurélie ; Université de Liège - ULiège > Centre de recherches du cyclotron

Schnakers, Caroline

Damas, François ; Université de Liège - ULiège > Anesthésie et réanimation

Pellas, F.

Lutte, I.

Bernheim, J.

Majerus, Steve ; Université de Liège - ULiège > Département des sciences cognitives > Psychopathologie cognitive

Moonen, Gustave ; Université de Liège - ULiège > Neurologie Sart Tilman

Goldman, Serge; Université Libre de Bruxelles - ULB > Hopital Erasme - Unite PET

Laureys, Steven ; Université de Liège - ULiège > Centre de recherches du cyclotron - Département des sciences cliniques

Language :

English

Title :

Locked-in syndrome in children: report of five cases and review of the literature

Publication date :

2009

Journal title :

Pediatric Neurology

ISSN :

0887-8994

Publisher :

Elsevier Science, New York, United States - New York

Volume :

Pages :

237-246

Peer reviewed :

Peer Reviewed verified by ORBi

Additional URL :

http://www.coma.ulg.ac.be/papers/locked-in_syndrome.html

Funders :

French Association for Locked-In Syndrome
F.R.S.-FNRS - Fonds de la Recherche Scientifique
JSMF - James S McDonnell Foundation
MSF - Mind Science Foundation
EU - European Commission
Concerted Research Action

Available on ORBi :

since 27 October 2009

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