Post-streptococcal autoimmune neuropsychiatric disease presenting as paroxysmal dystonic choreoathetosis

Mov Disord. 2002 Jul;17(4):817-20. doi: 10.1002/mds.10169.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibody Specificity / immunology
  • Autoantibodies / blood
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / immunology
  • Basal Ganglia / immunology
  • Child
  • Chorea / diagnosis*
  • Chorea / immunology
  • Corpus Striatum / immunology
  • Dementia / diagnosis*
  • Dementia / immunology
  • Diagnosis, Differential
  • Dystonia / diagnosis*
  • Dystonia / immunology
  • Humans
  • Male
  • Pharyngitis / complications*
  • Pharyngitis / immunology
  • Streptococcal Infections / complications*
  • Streptococcal Infections / diagnosis*
  • Streptococcal Infections / immunology


  • Autoantibodies