Neurological manifestations of Behçet’s disease: 161 cases in one moroccan center

Introduction: Behçet’s disease (BD) is a chronic multisystemic inflammatory disease, predominant within the Mediterranean countries and characterized by oral, genital mucous ulcers and uveitis. Neurological involvement is one of the most serious complications of BD and represents a major cause of morbidity. Patients and methods: 161 moroccan cases of neurobehçet disease (NBD) have been categorized into two main groups: “Parenchymal” CNS involvement, which includes hemispheric, brain-stem, spinal, and multifocal presentations; and “non parenchymal” CNS involvement, which includes dural sinus thrombosis and arterial involvement. Results: Parenchymal CNS pattern was observed in 74% of our cases with a predominance of symptoms related to brainstem involvement (30%) such ophthalmoparesis, bulbar and pseudobulbar palsy. Other symptoms have been present with varying frequencies: cerebellar signs(52%), headache(52%), psychiatric and cognitive symptoms (34%), sphincter disturbances (22%), epilepsy (9%). Some features are less common such as optic neuropathy (2%) or peripheral neuropathy(1%). Non-parenchymal form was noted in 26% of our series essentially represented by cerebral venous thrombosis (CVT). Only 4 of cases have cerebral arterial involvement, three with intracranial aneurysm. All patients have cerebral imaging. Magnetic resonance imaging showed a caracteristic lesion in one third of our patients, located at the mesodiencephalic junction and the pontobulbar region. Treatment was based on corticosteroids in the two categories. Long-term maintenance with immunosuppressive agents was administrated in patients with parenchymal CNS involvement and anticoagulants in case of CVT. Conclusion: Neurological involvement represents a major complications of BD which requires early diagnosis and long terme management.