BEHCETS-SYNDROME PRIMARILY INVOLVING CERE BRAL VESSELS

History and clinical findings: Two months after the onset of mainly fr ontal headaches a 25-year-old man of Turkish descent additionally deve loped double vision. Fundoscopy revealed bilateral choked discs and ri ght trochlear paralysis. Computed tomography and digital subtraction a ngiography demonstrated thrombosis of the superior sagittal sinus. On admission to hospital the patient was fully conscious but had marked m eningismus, bilaterally positive Lasegue's sign (painful straight leg raising) at a 50 degrees angle, and multiple oral aphthous ulcers. The sinus thrombosis suggested a chronic inflammatory process, while the oral ulcers pointed to Behcet's syndrome. Investigations: Inflammatory parameters (erythrocyte sedimentation rate, C-reactive protein and wh ite blood cell count) were increased and the HLA-B27 test was positive . Other laboratory tests, cerebrospinal fluid, chest radiogram and ECG were unremarkable. Treatment and course: Despite intravenous administ ration of heparin and cefuroxim for one week the sagittal sinus thromb osis spread to the straight sinus. In the third week scrotal ulceratio ns were noted and taken to confirm Behcet's syndrome. Immunosuppressiv e treatment with methylprednisolone (initially 80 mg daily) and 2 week s later together with chlorambucil (0.1 mg/kg daily) was started. Four weeks later the patient was free of symptoms and discharged. Conclusi on: Neurological signs are not adequately stressed by the Internationa l Study Group for Behcet's Disease among its listed diagnostic criteri a.