Affiliations: Department of Pediatric Neurology, Shaare Zedek
Medical Center, Jerusalem, Israel | Department of Pediatric Rheumatology, Shaare Zedek
Medical Center, Jerusalem, Israel
Note: [] Correspondence: Dr. Hilla Ben-Pazi, Shaare Zedek Medical Center, P.O. 3235, Jerusalem 91031, Israel.
Tel.: +972 2 6666641; Fax: +972 2 6555672; E-mail: benpazi@gmail.com
Abstract: Sydenham's chorea (SC) is, thought to be an autoimmune
neuropsychiatric disorder that may have a prolonged fluctuating course. In some
children, associated psychiatric symptoms such as depression, anxiety and
obsessive-compulsive symptoms are unresponsive to neuroleptic therapy and may
cause significant disability. We describe two adolescents with no prior
neuropsychiatric disorders who developed SC and psychiatric symptomatology that
did not respond to conventional psychiatric drugs: a 13-year-old boy with
hallucinations and a 14-year-old girl with abulia. They received
immunosuppressive therapy for 1 year. Hallucinations in the boy resolved
following the yearlong regimen of corticosteroids and did not recur during the
5-year follow-up period. The abulia resolved after 14 weeks of prednisone
treatment but recurred several months later; further treatment with prednisone
and azathioprine resulted in complete resolution of the abulia over the next
year. This report of immunosuppressive-responsive psychiatric symptoms in SC
suggests an underlying autoimmune disorder causing behavioral changes and
offers a therapeutic option in neuroleptic resistant cases.