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Immune mediated chorea encephalopathy syndrome in childhood
Author(s) -
Hartley LM,
Ng SY,
Dale RC,
Church AJ,
Martinez A,
De Sousa C
Publication year - 2002
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/j.1469-8749.2002.tb00804.x
Subject(s) - chorea , encephalopathy , medicine , pediatrics , extrapyramidal symptoms , atrophy , extrapyramidal disorder , stiff person syndrome , cerebral atrophy , movement disorders , pandas , immunology , psychiatry , disease , schizophrenia (object oriented programming) , antipsychotic , biochemistry , chemistry , glutamate decarboxylase , enzyme
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.