Article (Scientific journals)
Locked-in syndrome in children: report of five cases and review of the literature
Bruno, Marie-Aurélie; Schnakers, Caroline; Damas, François et al.
2009In Pediatric Neurology, 41, p. 237-246
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Keywords :
Locked-in syndrome
Abstract :
[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 :
41
Pages :
237-246
Peer reviewed :
Peer Reviewed verified by ORBi
Funders :
French Association for Locked-In Syndrome
F.R.S.-FNRS - Fonds de la Recherche Scientifique [BE]
JSMF - James S McDonnell Foundation [US-MO]
MSF - Mind Science Foundation [US-TX]
EU - European Commission
Concerted Research Action
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since 27 October 2009

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