Reference : Locked-in syndrome in children: report of five cases and review of the literature
Scientific journals : Article
Human health sciences : Neurology
Locked-in syndrome in children: report of five cases and review of the literature
Bruno, Marie-Aurélie mailto [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 mailto [Université de Liège - ULg > > Centre de recherches du cyclotron - Département des sciences cliniques >]
Pediatric Neurology
Elsevier Science
Yes (verified by ORBi)
New York
[en] Locked-in syndrome
[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
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

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