Severe childhood encephalopathy with dyskinesia and prolonged cognitive disturbances: evidence for anti-N-methyl-d-aspartate receptor encephalitis.

Details

Serval ID
serval:BIB_2A49379425B4
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Severe childhood encephalopathy with dyskinesia and prolonged cognitive disturbances: evidence for anti-N-methyl-d-aspartate receptor encephalitis.
Journal
Developmental Medicine and Child Neurology
Author(s)
Poloni Claudia, Korff Christian M., Ricotti Valeria, King Mary D., Perez Eliane Roulet, Mayor-Dubois Claire, Haenggeli Charles-Antoine, Deonna Thierry
ISSN
1469-8749[electronic], 0012-1622[linking]
Publication state
Published
Issued date
2009
Volume
52
Number
5
Pages
78-82
Language
english
Abstract
Aim We report four cases of acquired severe encephalopathy with massive hyperkinesia, marked neurological and cognitive regression, sleep disturbance, prolonged mutism, and a remarkably delayed recovery (time to full recovery between 5 and 18mo) with an overall good outcome, and its association with anti-N-methyl-d-aspartate (anti-NMDA) receptor antibodies. Method We reviewed the four cases retrospectively and we also reviewed the literature. Results Anti-NMDA receptor antibodies (without ovarian teratoma detected so far) were found in the two children tested in this study. Interpretation The clinical features are similar to those first reported in 1992 by Sebire et al.,(1) and rarely recognized since. Sleep disturbance was not emphasized as part of the disorder, but appears to be an important feature, whereas coma is less certain and difficult to evaluate in this setting. The combination of symptoms, evolution (mainly seizures at onset), severity, paucity of abnormal laboratory findings, very slow recovery, and difficult management justify its recognition as a specific entity. The neuropathological substrate may be anatomically close to that involved in encephalitis lethargica, in which the same target functions (sleep and movement) are affected but in reverse, with hypersomnolence and bradykinesia. This syndrome closely resembles anti-NMDA receptor encephalitis, which has been reported in adults and is often paraneoplastic.
Keywords
Basal Ganglia Antibodies, Movement-Disorder, Abnormal Movements, Lethargica, Chorea, Coma
Pubmed
Web of science
Create date
06/05/2010 14:43
Last modification date
20/08/2019 13:09
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