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Anti basal ganglia antibodies (ABGA) have been associated with 100%of acute cases and 69%of persistent cases of Sydenhams chorea. We describe two cases o f late recurrences of Sydenhams chorea with absence of ABGA. Both patients had several childhood episodes of Sydenhams chorea. MRI imaging of the basal gang lia and exhaustive investigations for other causes of chorea were normal or nega tive. The absence of ABGA may be evidence against an autoimmune pathology in lat e and some persistent recurrences. We suggest the likely pathophysiology to be d opamine hypersensitivity of chronically damaged basal ganglia neurones possibly following induction of an autoimmune antibody response in childhood.
Antibasal ganglia antibodies (ABGA) have been associated with 100% of acute cases and 69% of persistent cases of Sydenham’s chorea. We describe two cases of late recurrences of Sydenham’s chorea with absence of ABGA. Both patients had several childhood episodes of Sydenham’s chorea. MRI imaging of the basal gang lia and exhaustive investigations for other causes of chorea were normal or nega tive. The absence of ABGA may be evidence against an autoimmune pathology in lat e and some persistent recurrences. We suggest the likely pathophysiology to be d opamine hypersensitivity of chronically damaged basal ganglia neurones can follow induction of an autoimmune antibody response in childhood.