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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 2001 Mar;70(3):382–385. doi: 10.1136/jnnp.70.3.382

Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation

J Stone 1, A Franks 1, J Guthrie 1, M Johnson 1
PMCID: PMC1737241  PMID: 11181863

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.



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