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DOI: 10.1055/s-2005-868023
Severe autoimmune chorea with positive antibasalganglia antibodies (ABGA) following herpesencephalitis: Treatment with plasmapheresis and corticosteroids
Introduction: Chorea has been described as a sign of relapse in patients with herpes simplex virus (HSV) encephalitis, while chorea following streptococcal infections is a well known post infectious phenomenon, possibly mediated by anti-basalganglia antibodies (ABGA). We report a case with severe autoimmune-mediated chorea 3 weeks after an episode of HSV 1 encephalitis.
Case report: A 2½ year old girl was diagnosed with HSV 1 encephalitis and was treated with acyclovir, 30mg/kg/day for 15 days with a rapid but incomplete recovery and negative follow-up HSV CSF PCR. 3 weeks after initial onset she developed compulsive behaviour and within days developed a severe choreiforme movement disorder. Herpes PCR in CSF was negative and MRI showed bitemporal cystic lesions but was otherwise unchanged. Readministration of Acyclovir, 60mg/kg/day for 21 days in conjunction with a course of intravenous immunoglobulines 2.0g/kg for 5 days was of no benefit. Treatment with chlorprothixen, risperidone, valproate, dopamine and tiapridhydrochloride were ineffective. 5 weeks after onset of the chorea, nasotracheal intubation and artificial ventilation became necessary. Positive ABGA in CSF and plasma were demonstrated using Western immunoblotting in two pairs of samples taken 2 weeks and 1 month after the onset of the chorea. No benefit was noted after high dose methylprednisolone (20mg/kg/day) for 3 days. Intermittent plasmapheresis, 6 sessions on alternate days, exchanging a plasma volume of 60ml/kg in each procedure and systemic immunosuppression with prednisolone, 2mg/kg/day, resulted in rapid improvement and extubation after 3 more weeks.
Conclusion: While ABGA associated movement disorders in childhood have been reported following streptococcal infections, this is the first report on ABGA positive chorea following HSV 1 encephalitis. An underlying autoimmune mechanism was suggested by the finding of positive ABGA in plasma and CSF.
Systemic immunosuppression and elimination of circulating antibodies using intermittent plasmapheresis resulted in rapid clinical improvement.