Purpose: To analyse and discuss neurological involvement of Behçet' disease and therapeutical possibilities giving special attention to unusual forms.
Methods: We retrospectively reviewed the medical records of 40 cases with neurological manifestations among 300 cases of Behçet's disease seen over a 20 years period who met the international study group of Behçet's disease criteria.
Results: Central nervous system involvement was found in 39 patients and peripheral nervous involvement in one. Dominant symptoms in 40 patients with neurological manifestations of Behçet's disease were meningo-encephalitis (27 cases), intracranial hypertension (9 cases), psychic disturbances (2 cases), and polyneuritis of the lower limb (1 case). Headache and focal deficits were the major presenting signs (87%). Cerebral venous thrombosis was identified in 8 patients (6 certain and 2 probable). These could be differentiated from meningoencephalitis by their symptomatology predominaly that of intracranial hypertension. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level cerebral CT scan, performed in 20 patients, was normal in 30% of cases. MRI performed in 2 patients, showed in one case a tumor like lesion.
Conclusion: Neurobehçet's disease can be classified into 2 clinical aspects: meningoencephalitis and cerebral venous thrombosis. Treatment is aimed initially at reducing the inflammation with corticosteroids and preventing relapse with adjunction of an immunosuppressor.