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Catastrophic primary antiphospholipid syndrome presenting as status epilepticus
  1. L J Coward1,2,
  2. D M Kullmann1,
  3. N P Hirsch2,
  4. R S Howard2,
  5. S B Lucas3
  1. 1Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK
  2. 2Harris Neuromedical Intensive Care Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK
  3. 3GKT/GST Dept of Pathology, St Thomas’ Hospital, Lambeth Palace Road, London, SE17EH, UK
  1. Correspondence to:
 Dr R S Howard
 Box 33 Medical Intensive Care Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG;

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Antiphospholipid syndrome (APS) is defined as the occurrence of arterial or venous thrombosis or recurrent miscarriage, with raised titres of antiphospholipid antibodies, namely lupus anticoagulant (LA) or anti-cardiolipin antibodies (aCL). An accelerated form of APS is catastrophic antiphospholipid syndrome (CAPS) or Asherson’s syndrome. Proposed diagnostic criteria for definite CAPS are: multiorgan failure, development of manifestations within 1 week, demonstration of antiphospholipid antibodies and histopathological evidence of microthrombosis (positive predictive value 99.4%).1 Precipitants include infection, surgery, and childbirth. Patients typically develop widespread thrombotic vasculopathy with marked thrombocytopaenia. Clinically apparent cerebral infarction occurs much less frequently than in uncomplicated APS but the major pathological manifestation of CAPS is cerebral microthrombosis at postmortem examination.

Case report

A 30 year old woman delivered her first child following a full term uncomplicated pregnancy. Three weeks later, she developed headache and transient hemiparesis. The following day she had an episode of speech arrest, bit her tongue, and held both arms stiffly in the air for about 1 minute. On admission to hospital, she was pyrexial and developed generalised tonic clonic seizures. There was no relevant previous medical history and she was not taking any medication. Blood tests. including erythrocyte sedimentation rate (ESR) and C reactive protein (CRP). were normal. Computed tomography …

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  • Competing interests: none