Introduction: Behçet disease is a systemic form of vasculitis which presents with neurological symptoms with a frequency varying between 16 and 40%. Involvement of the parenchyma has been found to worsen the prognosis in patients with neuroBehçet (NB).
Objective: To review the clinical features and course of patients with NB involving the parenchyma of the central nervous system (CNS).
Clinical cases: Seven patients with Behçet disease and neurological localizing signs were seen in our hospital between 1989 and 1996. The initial diagnosis was of ischemic ictus in five of the seven patients. Both neuroimaging studies and investigation of the cerebrospinal fluid were always pathological in all cases. Vascular studies (arteriography and echo-Doppler of the supra-aortic trunks) were normal. One patient died. Four patients had serious sequelae following treatment.
Conclusion: NB should be included in the different diagnosis of ictus. Involvement of the parenchyma of the CNS was accompanied by lymphocytic meningitis, perhaps also leading to a worse functional prognosis.