학술논문

100 Antiphospholipid-related chorea
Document Type
Article
Source
Journal of Neurology, Neurosurgery, & Psychiatry (JNNP); 2022, Vol. 93 Issue: 9 pe2-ax-e2-ax, 1p
Subject
Language
ISSN
00223050; 1468330X
Abstract
Chorea of subacute onset is a movement disorder that typically has a monophasic course. Chorea can be associated with autoimmune diseases such as antiphospholipid syndrome and has been associated with the isolated presence of antiphospholipid antibodies (aPL). Here, we report a patient with isolated aPL-related chorea that unusually followed a relapsing-remitting course.A female in her forties developed chorea gravidarum during her first pregnancy, eight years previously, and again during her second pregnancy five years ago. Investigations showed high titre triple positivity of aPL. No basal ganglia lesion or signal change was identified on MRI brain. There was no history of arterial or venous thromboembolism or pregnancy morbidity. Her symptoms continued post-partum and she received treatment with low-molecular-weight-heparin, prednisolone and intravenous immunoglobulin, while breastfeeding. Tetrabenazine was trialled, which helped to suppress chorea, though lowered her mood. The chorea remitted. She developed a recurrence of chorea three years later, when she was not pregnant or taking hormone therapy. She had significant irritability. Olanzapine was trialled with benefit and her movements and mood improved. She relapsed with a fourth recurrence of chorea two years later and recommenced olanzapine, with no symptomatic benefit. She is currently trialling pulsed oral methylprednisolone followed by azathioprine.