Neuropediatrics 2005; 36 - P38
DOI: 10.1055/s-2005-868023

Severe autoimmune chorea with positive antibasalganglia antibodies (ABGA) following herpesencephalitis: Treatment with plasmapheresis and corticosteroids

B Plecko 1, U Gruber-Sedlmayr 1, I Marschitz 2, S Rödl 2, C Mache 3, A Church 4, G Giovannoni 4, HM Grubbauer 2, G Zobel 2
  • 1Univ. Klinik für Kinder- und Jugendheilkunde, Ambulanz für Neuropädiatrie, Graz, Österreich
  • 2Univ. Klinik für Kinder- und Jugendheilkunde, Pädiatrische Intensivstation & Brandverletzteneinheit, Graz, Österreich
  • 3Univ. Klinik für Kinder-und Jugendheilkunde, Amb. für Urologie und Nephrologie, Graz. Österreich
  • 4Institute of Neurology, Queen Square, Department of Neuroinflammation, London, GB

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.