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Systemic Lupus Erythematosus and Chorea Amanda Bevan

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

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Systemic Lupus Erythematosus and Chorea Amanda Bevan

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  1. Systemic Lupus Erythematosus and Chorea Amanda Bevan

  2. Systemic Lupus Erythematosus and Chorea Amanda Bevan Southampton University Hospitals NHS Trust

  3. 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.

  4. 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

  5. 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

  6. The Case • Day 19 transferred to PHDU, anxious, distressed and increasing choreoid movements • All treatment continues • Day 20 seen by paed pharmacist, stop hydroxychloroquine.

  7. 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.

  8. Discussion • Disease related or was this drug induced? • 11% of aPL, but • Temporal relationship • Published evidence • Rechallenge

  9. Any Questions??????

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