Post-streptococcal autoimmune neuropsychiatric disease presenting as paroxysmal dystonic choreoathetosis.
Paroxysmal dystonic choreoathetosis (PDC) is an episodic, non-kinesogenic, extrapyramidal movement disorder. It is postulated that PDC is an ion channel disorder. We describe a sporadic case of paroxysmal dystonic choreoathetosis occurring after streptococcal pharyngitis. The episodes were characterized by abrupt-onset dystonic posturing, choreoathetosis, visual hallucinations and behavioral disturbance. Each episode lasted between 10 minutes and 4 hours, and occurred up to 4 times per day. In between attacks, examination was normal. The episodes waxed and waned in frequency during a 6-month illness. Magnetic resonance imaging of the brain was normal. Post-streptococcal neuropsychiatric disease has a proposed autoimmune etiology, which is supported by the presence of serum antibasal ganglia antibodies. Western immunoblotting of this case's serum demonstrated antibody binding to a basal ganglia antigens of molecular weight 80 kDa and 95 kDa. Immunohistochemistry examination demonstrated specific antibody binding to large striatal neurones. We propose that autoantibodies produced in post-streptococcal neuropsychiatric disease cause alteration in neurotransmission, possibly secondary to ion channel binding.
Dale
Russell C
RC
Department of Neurology, Great Ormond Street Hospital NHS Trust and Institute of Child Health, London, United Kingdom. R.Dale@ion.ac.uk
Church
Andrew J
AJ
Surtees
Robert A H
RA
Thompson
Edward J
EJ
Giovannoni
Gavin
G
Neville
Brian G R
BG
D000918:Antibody Specificity / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D001479:Basal Ganglia / Q000276:immunology; D002648:Child; D002819:Chorea / Q000175:diagnosis* / Q000276:immunology; D003342:Corpus Striatum / Q000276:immunology; D003704:Dementia / Q000175:diagnosis* / Q000276:immunology; D003937:Diagnosis, Differential; D004421:Dystonia / Q000175:diagnosis* / Q000276:immunology; D006801:Humans; D008297:Male; D010612:Pharyngitis / Q000150:complications* / Q000276:immunology; D013290:Streptococcal Infections / Q000150:complications* / Q000175:diagnosis* / Q000276:immunology
D001323:Autoantibodies