PO244 Case report: ‘en coup de sabre’ and cerebral vasculitis

Introduction ‘En coup the sabre’, a linear form of facial scleroderma, is rarely complicated by neurological symptoms. The aetiology of the underlying cerebral lesions is still obscure, but there is evidence in favour of a vasculitic process. Our case, with neurological symptoms presenting over ten years, illustrates this picture. Case report A 60 year old woman with history of facial linear scleroderma and migraine, presented to casualty with acute left sided weakness. Two months prior to this, she had been admitted for new onset of seizures. Seven years earlier she had developed acute left hemianopia and a brain scan had revealed a possible arterio-venous malformation (AVM) and gliotic changes in the right hemisphere. A new MRI showed progression of the right sided lesions. A cerebral angiogram did not confirm AVM, but showed signs of vasculitis. Identical oligoclonal bands were found in cerebrospinal fluid (CSF) and blood. ANA and ANCA were positive. She was treated with steroids and started on mycophenolate. She improved and remains stable at 2 years. Conclusions The association between linear scleroderma and neurological symptoms is well known, but its pathological nature is still debated. Vasculitis is a possible underlying process, and needs consideration and treatment in case of symptoms progression.