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Follow the challenging case of a 16-year-old girl with SLE who developed chorea. Explore treatment strategies, drug-induced effects, and the role of anticoagulation in this rare manifestation.
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Systemic Lupus Erythematosus and Chorea Amanda Bevan
Systemic Lupus Erythematosus and Chorea Amanda Bevan Southampton University Hospitals NHS Trust
The Case • 16y old girl diagnosed with SLE • Day3 prescribed hydroxychloroquine and prednisolone and discharged home • Day13 presented to A&E with facial spasms and involuntary body movements • Treated with procyclidine and lorazepam; no response.
The Case • Send for CT venogram under GA • Post CT – increasingly violent movements • Re-intubated and admitted to PICU • Day14 treated with iv methylprednisolone • Continue hydroxychloroquine, • Consider cyclophosphamide • ?cortical vein thrombosis – anticoagulated with heparin
The Case • Day 15 extubated, still having significant involuntary movements and tongue thrusting • Prescribed high dose carbamazepine and levetiracetam • Day 17 no definite evidence of thrombosis • Day 18 increasing chorea treated with midazolam boluses, prescribed clonazepam regularly and as required chloral hydrate
The Case • Day 19 transferred to PHDU, anxious, distressed and increasing choreoid movements • All treatment continues • Day 20 seen by paed pharmacist, stop hydroxychloroquine.
The Case • Day 21 Definite reduction in involuntary movements. • Day 23 All involuntary movements stopped. Discharged to medical ward, wean anticonvulsants. • Day 37 Discharged home.
Discussion • Disease related or was this drug induced? • 11% of aPL, but • Temporal relationship • Published evidence • Rechallenge