Neuropediatrics 2012; 43 - VS11_05
DOI: 10.1055/s-0032-1307161

Behavioural changes and unilateral movement disorder in 2 adolescents with Chorea Sydenham

A Hackenberg 1, D Wille 1, E Boltshauser 1
  • 1Kinderspital Zürich, Zürich, Switzerland

Aims: Acute rheumatic fever (ARF) is rare in industrialized countries with an incidence below 1/10000, while in developing countries it can be as high as 6/1000. Chorea Sydenham occurs in up to 30% of the cases of ARF, perferably in adolescents, in many cases as the only symptom. Diagnosis is made by exclusion. Unilateral preponderance and accompanying psychiatric symptoms are common. The finding of high streptococcal antibody titres may be absent as chorea occurs 1 to 7 months after Group A streptococcal tonsillopharyngitis.

We report on a 11 year old girl and a 14 year old boy presenting with restlessness, bad temper and school problems that developed within a few days. At the same time unilateral weakness and involuntary movements emerged with distal accentuated irregular jerks spreading to the trunk and the face. The movements were aggravated by motor activity and subsided during sleep. As movements could not be suppressed the affected limb was intermittently held tight. The physical examination was otherwise normal, cardiac involvement was excluded. Treatment with prednisolon (1mg/kg per day) over 4 weeks led to resolution of symptoms within 10 days and sustained remission.

Methods: case report

Results: The videos show 2 adolescents with unilateral chorea during neurologic exam.

Conclusion: A subacute choreatiform movement disorder should not be mistaken as a somatoform disorder, presentation can be unilateral. Most cases of chorea occur postinfectious after Group A streptococcal tonsillopharyngitis. Before making the diagnosis of Chorea Sydenham Lupus erythematodes, Antiphospholipid-Antibody-Syndrome, M. Wilson and a tumour need to be excluded. The course is self limiting, treatment with steroids leads to a shortened disease duration of more than 50%, relapses occur in about 20%. Prophylaxis with penicillin prevents booster effects by reinfections.

Chorea Sydenham - chorea minor - acute rheumatic fever - streptococci