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

Poloni, Claudia
Ricotti, Valeria
King, Mary D.
Perez, Eliane Roulet
Mayor-Dubois, Claire
Deonna, Thierry
Published in Development Medicine and Child neurology. 2010, vol. 52, no. 5, p. e78-82
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 Autoantibodies/bloodBrain/pathology/physiopathologyChildChild, PreschoolCognition Disorders/*etiology/physiopathologyDyskinesias/*etiology/physiopathologyElectroencephalographyEncephalitis/*complications/diagnosis/immunology/pathology/physiopathologyFemaleHumansMagnetic Resonance ImagingMutism/etiologyPrognosisReceptors, N-Methyl-D-Aspartate/*immunologyRetrospective StudiesSeverity of Illness IndexSleep Disorders/*etiology/physiopathologyTime Factors
PMID: 20041934
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Research group Groupe Menache Starobinski Caroline (881)
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POLONI, Claudia et al. Severe childhood encephalopathy with dyskinesia and prolonged cognitive disturbances: evidence for anti-N-methyl-D-aspartate receptor encephalitis. In: Developmental Medicine and Child Neurology, 2010, vol. 52, n° 5, p. e78-82. doi: 10.1111/j.1469-8749.2009.03542.x https://archive-ouverte.unige.ch/unige:21225

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Deposited on : 2012-05-23

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