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Scleroderma “en coup de sabre”: pathological evidence of intracerebral inflammation
  1. J Stonea,
  2. A J Franksb,
  3. J A Guthriec,
  4. M H Johnsond
  1. aDepartment of Clinical Neurosciences, Western General Hospital, Crewe Rd, Edinburgh EH4 2XU, UK, bDepartment of Public Health, Division of Laboratory, Radiology and Pharmacy Services, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK, cDepartment of Radiology, St James's University Hospital, Beckett St, Leeds LS9 7TF, UK, dDepartment of Neurology
  1. Dr J Stone jstone{at}skull.dcn.ed.ac.uk

Abstract

Linear scleroderma “en coup de sabre” (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. Analysis of CSF and pathology obtained at brain biopsy provides evidence of an inflammatory process which may be amenable to immunosuppressive treatment.

  • scleroderma “en coup de sabre”
  • central nervous system inflammation
  • progressive facial hemiatrophy

https://doi.org/10.1136/jnnp.70.3.382

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