Chorea in juvenile primary antiphospholipid syndrome : Reversible decreased circulation in the basal ganglia visualised by single photon emission computed tomography

• Published in: Scandinavian journal of rheumatology Vol. 28; no. 5; pp. 324 - 327
• Main Authors: NORDAL, E. B, NIELSEN, J, MARHAUG, G
• Format: Journal Article
• Language: English
• Published: Colchester Taylor & Francis 1999
• Subjects: Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - drug therapy; Antiphospholipid Syndrome - physiopathology; Antirheumatic Agents - therapeutic use; Basal Ganglia - blood supply; Basal Ganglia - diagnostic imaging; Biological and medical sciences; Child; Chorea - complications; Chorea - diagnostic imaging; Chorea - physiopathology; Cyclophosphamide - therapeutic use; Female; Glucocorticoids - therapeutic use; Humans; Medical sciences; Regional Blood Flow; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Tomography, Emission-Computed, Single-Photon; Radionuclide study; Chorea; Cardiovascular disease; Autoimmune disease; Hemopathy; Involuntary movement; Vascular disease; Primary; Diagnosis; Neurological disorder; Child; Human; Immunopathology; Nervous system diseases; Antiphospholipid antibody syndrome; Exploration; Photon; Cerebral disorder; Case study; Symptomatology; Platelet; Treatment; Central nervous system disease; Medical imagery; Extrapyramidal syndrome; Emission tomography
• ISSN: 0300-9742; 1502-7732

Chorea was observed in a 12-year-old girl with primary antiphospholipid syndrome (APS). She developed severe chorea in a few weeks. On immunosuppressive treatment, including high doses of glucocorticoids and cyclophosphamide, she had a rapid clinical recovery. Single photon emission computed tomography (SPECT) of the brain showed decreased circulation in the basal ganglia and in the medial parts of both temporal lobes. One month after treatment, SPECT was completely normalised. APS in children has a variety of clinical manifestations, and should be suspected in cases of unexplained thromboembolic disease or obscure neurological symptoms.