Introduction: Behçet's disease is a systemic, chronic, relapsing inflammatory disease of unknown etiology characterized by recurrent oral aphthae, genital ulcerations and uveitis. The main pathological mechanism is leukocytoclastic vasculitis.
Clinical case: We present the case of a patient with Behçet's disease that presented as a relapsing meningoencephalitis with lymphocytic pleocytosis and high protein levels in cerebrospinal fluid. The MRI revealed a lesion in the brain stem and part of diencephalon.
Conclusions: The central nervous system is involved in up to 5%-20% of patients with Behçet's disease, this being the first manifestation in 3% of the cases. Clinical course includes: patients with an isolated episode with complete or incomplete recovery, relapsing and remitting forms, progressive forms or asymptomatic neurological involvement. The most frequent presentation is relapsing meningoencephalitis, with progressive course localized in the brain stem. Prognosis varies according to the type of neurologic process. The worse outcome is in the parenchymal forms with brain stem and spinal cord involvement. It also depends on the pathological findings in the cerebrospinal fluid, and the number of total events or recurrences after tapering the glucocorticoids. Mortality rate is high, up to 20% of cases after 7 years in patients with neurological involvement.