IMMUNE MEDIATED CHOREA ENCEPHALOPATHY AND PARVOVIRUS INFECTION

Objectives: To describe the clinical, radiological, neurophysiological and immunological features of Immune Mediated Chorea Encephalopathy syndrome in a series of patients and to highlight the association with Parvovirus infection in a single case.

Methods: Retrospective collection and analysis of clinical, radiological and laboratory data. Results: Five previously healthy female children presented with encephalopathy and an extrapyramidal movement disorder (chorea in 5, rigidity in 2 and oculogyric crisis in 2). In addition, an acute behavioural disturbance occurred in three patients and mutism in two others. Seizures heralded the onset of the illness in three cases. Acute magnetic resonance neuroimaging (MRI) was either normal or initially normal with later generalised cerebral atrophy. All five cases had oligoclonal bands in the cerebrospinal fluid (CSF) but not the serum, indicating intrathecal immunoglobulin synthesis. In one patient genomic human Parvovirus B19 DNA was detected in her serum and cerebrospinal fluid. All other infective (including streptococcus), biochemical and metabolic investigations were normal in the remaining 4 patients. All five children made a full recovery within four months of the onset.

Conclusion: These cases represent an immune-mediated movement disorder and encephalopathy syndrome. One of our patients represents the first described case of childhood chorea-encephalopathy associated with and probably caused by human Parvovirus B19 infection.