Objective: To discuss neurological involvement of Behçet's disease and therapeutical possibilities.
Patients: Among 110 patients with Behçet's disease, 16 were found to have neurological involvement. All these patients fulfilled Japanese and International Study Group criteria for diagnosis of Behçet's disease.
Results: Central nervous system involvement was found in 15 patients and peripheral nervous system involvement in one. Meningoencephalitis and/or transverse myelitis were the most frequent features (69%) followed by tumor-like manifestations (13%); cerebral venous thrombosis was identified in 1 patient with occlusion of the left lateral sinus. Focal deficits were the major presenting signs and cranial paralysis were present in 19% of patients. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level. Cerebral CT Scan, performed in 6 patients, was normal in 33% of cases. MRI, performed in 4, showed abnormal signals distributed over hemispheric white matter, the brainstem and the thalamus in one patient, an occlusion of the left lateral sinus in the second one and a tumor-like lesion in the third. MRI abnormalities were associated with concording clinical deficits.
Conclusion: Neurological involvement in Behçet's disease con be classified into 3 clinical aspects: meningoencephalitis (and/or myelitis), cerebral venous thrombosis and tumor-like features. Corticosteroids, when prescribed early, are useful and are associated with better prognosis.