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Immune mediated chorea encephalopathy syndrome in childhood

Published online by Cambridge University Press:  09 May 2002

LM Hartley ,
SY Ng ,
RC Dale ,
AJ Church ,
A Martinez  and
C de Sousa
LM Hartley
Affiliation:
Department of Neurology, Great Ormond Street Hospital for Children NHS Trust, and Institute of Child Health, London, UK.
SY Ng
Affiliation:
Department of Neurology, Great Ormond Street Hospital for Children NHS Trust, and Institute of Child Health, London, UK.
RC Dale
Affiliation:
Department of Neurology, Great Ormond Street Hospital for Children NHS Trust, and Institute of Child Health, London, UK.
AJ Church
Affiliation:
Neuroimmunology Unit, Institute of Neurology, UK.
A Martinez
Affiliation:
Department of Paediatric Neurology, Royal London Hospital, Garden House, Whitechapel, UK.
C de Sousa
Affiliation:
Department of Neurology, Great Ormond Street Hospital for Children NHS Trust, and Institute of Child Health, London, UK.
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Abstract

We report four previously healthy female children, aged between 3 and 8 years, who presented with encephalopathy and an extrapyramidal movement disorder (chorea n=4, rigidity n=2, oculogyric crisis n=2). In addition, an acute behavioural disturbance occurred in two patients and mutism in two others. Seizures heralded the onset of the illness in three patients. Acute MRI was either normal or initially normal with later generalized cerebral atrophy. All infective (including streptococcus), biochemical, and metabolic investigations were normal, although all four patients had oligoclonal bands in the (CSF) but not the serum, indicating intrathecal immunoglobulin synthesis. All four children made an apparently full recovery within four months of the onset.We suggest that these patients represent an immune-mediated movement disorder and encephalopathy syndrome.

Type
Case Reports
Information
Developmental Medicine and Child Neurology , Volume 44 , Issue 4 , April 2002 , pp. 273 - 277
Copyright
© 2002 Mac Keith Press

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