A dystonic syndrome associated with anti-basal ganglia antibodies
Abstract
Anti-basal ganglia antibodies (ABGA) have been associated with movement disorders (usually tics and chorea) and psychiatric disturbance in children. This report describes five adult and adolescent patients (one male, four females; mean age of onset, 16 years (range, 13–35)) who presented subacutely with a clinical syndrome dominated by dystonia and had ABGA binding to antigens of similar molecular weights to those seen in Sydenham’s chorea. Three patients had a clear history of respiratory infection before the onset of their symptoms. Three patients received immunosuppressive treatment, with three showing a notable reduction in symptoms. It is hypothesised that dystonia in adults or adolescents may be part of the clinical spectrum of the post-infectious syndrome associated with ABGA.
Anti-basal ganglia antibodies (ABGA) have been found in association with both Sydenham’s chorea (SC),1 and a condition in children characterised by a movement disorder (tics and chorea most commonly) and psychiatric problems following streptococcal infection, known as paediatric autoimmune neuropsychiatric disorder associated with streptococcal infection (PANDAS).2 A proportion of patients with a diagnosis of Tourette’s syndrome (TS) have also been found to have ABGA, and it is possible that PANDAS and TS have considerable overlap,3 although this remains controversial. Group A β haemolytic streptococcus is thought to be the causative organism involved in SC and PANDAS, and ABGA in SC are crossreactive with antigen extracts of streptococcus.4 The pathophysiological mechanism is thought to be autoimmune targeting of the basal ganglia via molecular mimicry, triggered by streptococcal infection.4 There have been no previous reports of a dystonic syndrome in adults or adolescents associated with ABGA.
Related to Dystonia?
Dopa-Responsive Dystonia
Cervical Dystonia and Exhaustion