Post-Streptococcal autoimmune neuropsychiatric disease presenting as paroxysmal dystonic choreoathetosis
✍ Scribed by Russell C. Dale; Andrew J. Church; Robert A.H. Surtees; Edward J. Thompson; Gavin Giovannoni; Brian G.R. Neville
- Publisher
- John Wiley and Sons
- Year
- 2002
- Tongue
- English
- Weight
- 99 KB
- Volume
- 17
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
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.