Hyperkinetic Movement Disorder as First Manifestation of an Autoimmune Disease

Introduction: Suddenly onset hyperkinetic movement disorders are rare in children without primary neurological disease. The causes of chorea minor are often autoimmunological diseases. We report on three patients with autoimmune hyperkinetic movement disorders.

Case Summary: A 16-year-old girl presented with acute right sided hemichorea. Retrospective, the SLE-typical symptoms with headache, visual disturbances, joint pain, fever and erythema already present for some weeks. MRI showed multiple infarctions. SLE-specific antibodies were demonstrated. A 16-year-old boy developed a left-sided hemichorea, accompanying by impaired concentration. Immunological tests showed elevated antiphospholipid-AB and antistreptococcal-AB. The third patient, a 16-year-old boy, presented with sudden onset left-sided hemichorea and had also a raised antistreptolysin-AB titer. His symptoms responded only temporarily to penicillin therapy. Patients 1 and 2 were initially treated with high dose prednisolone, followed by intravenous 7s-immunoglobulin and rituximab. Both patients showed complete restitution of symptoms. The third patient is currently undergoing intravenous 7s-immunoglobulin therapy at monthly intervals and after 2 months shows some improvement. Two of the three affected adolescents developed a depressive episode during the acute phase of the disease, which regressed with improvement of the movement disorder.

Conclusion: In otherwise healthy children with a sudden-onset hyperkinetic movement disorders, immunological diseases must be rapidly sought for after exclusion of other causes such as bleeding or CNS infections. Antiinflammatory therapy should be initiated early and aggressively.