Background:

Group-C-Streptococcus(GCS) is normal flora of the human upper airway and is implicated in pharyngitis, endocarditis, meningitis, osteomyelitis and puerperal infections. Group A hemolytic streptococcus is typically causative of acute rheumatic fever and chorea. To date there are rare descriptions of CNS manifestations with GCS.

Results:

20-year-old woman with history of autism, presented with acute onset choreiform movement 4 days after she developed acute pharyngitis with fever. The choreiform movements involved her face, tongue, bilateral arms and legs. Examination revealed classic chorea including motor impersistence. Chorea subsided during her sleep. She had a throat culture that was positive for Streptococcus C. Of note, there was no history of prior chorea, or use of dopamine blocking medications. There was no family history of movement disorders or neurodegenerative conditions. She was treated with a course of amoxicillin. Her work up for the chorea included a pregnancy test which was negative, ANA, autoimmune work up, copper and ceruloplasmin all of which were unremarkable. She had an elevated CRP and normal ESR. Her ASLO-titers were negative. Cardiac ultrasound did not show carditis.  MRI of the brain showed a small colloid cyst of the pineal gland and otherwise normal. Her symptoms improved with antibiotics and Haldol. She was recommended long term prophylaxis with Penicillin.

Sydenham chorea is a neurological disorder that occurs in up to a third of patients with acute rheumatic fever and is a major manifestation. It often occurs later than other manifestations typically, 1 to 6 months after acute pharyngitis with Streptococcus group A. In our case, however it occurred a few days after active Streptococcus C pharyngitis. We believe the association is real due the temporal relationship. Although rare, the diagnosis of Sydenham chorea must be considered in patient who presents with chorea even with negative rapid streptococcal test.