ORBi: Bruno Marie-Aurélie - Locked-in syndrome in children: report of five cases and review of the literature

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


	Document type :	Scientific journals : Article
	Discipline(s) :	Human health sciences : Neurology
	To cite this reference:	http://hdl.handle.net/2268/27015

Title :	Locked-in syndrome in children: report of five cases and review of the literature
Language :	English
Author, co-author :	Bruno, Marie-Aurélie [Université de Liège - ULg > > Centre de recherches du cyclotron >]
	Schnakers, C. [ > > ]
	Damas, François [Université de Liège - ULg > > Anesthésie et réanimation >]
	Pellas, F. [ > > ]
	Lutte, I. [ > > ]
	Bernheim, J. [ > > ]
	Majerus, Steve [Université de Liège - ULg > Département des sciences cognitives > Psychopathologie cognitive >]
	Moonen, Gustave [Université de Liège - ULg > > Neurologie Sart Tilman >]
	Goldman, Serge [Université Libre de Bruxelles - ULB > Hopital Erasme - Unite PET > > >]
	Laureys, Steven [Université de Liège - ULg > > Centre de recherches du cyclotron - Département des sciences cliniques >]
Publication date :	2009
Journal title :	Pediatric Neurology
Publisher :	Elsevier Science
Volume :	41
Pages :	237-246
Peer reviewed :	Yes (verified by ORBi)
Audience :	International
ISSN :	0887-8994
City :	New York
Country :	NY
Keywords :	[en] 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.
Funders :	French Association for Locked-In Syndrome ; Fonds de la Recherche Scientifique (Communauté française de Belgique) - F.R.S.-FNRS ; James S. McDonnell Foundation, ; Mind Science Foundation ; European Commission ; Concerted Research Action
Permalink :	http://hdl.handle.net/2268/27015
Other URL :	http://www.coma.ulg.ac.be/papers/locked-in_syndrome.html

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