[Behçet disease with primary involvement of the cerebral vessels]

Abstract

History and clinical findings: Two months after the onset of mainly frontal headaches a 25-year-old man of Turkish descent additionally developed double vision. Fundoscopy revealed bilateral choked discs and right trochlear paralysis. Computed tomography and digital subtraction angiography demonstrated thrombosis of the superior sagittal sinus. On admission to hospital the patient was fully conscious but had marked meningism, bilaterally positive Lasègue's sign (painful straight leg raising) at a 50 degree angle, and multiple oral aphthous ulcers. The sinus thrombosis suggested a chronic inflammatory process, while the oral ulcers pointed to Behçet's syndrome.

Investigations: Inflammatory parameters (erythrocyte sedimentation rate, C-reactive protein and white 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 administration of heparin nad cefuroxim for one week the sagittal sinus thrombosis spread to the straight sinus. In the third week scrotal ulcerations were noted and taken to confirm Behçet's syndrome. Immunosuppressive treatment with methylprednisolone (initially 80 mg daily) and 2 weeks later together with chlorambucil (0.1 mg/kg daily) was started. Four weeks later the patient was free of symptoms and discharged.

Conclusion: Neurological signs are not adequately stressed by the International Study Group for Behçet's Disease among its listed diagnostic criteria.