Journal de vascularite

Neuro-Behçet’s Disease: A Review of Neurological Manifestations and Its Treatment

Abstract

Livia Almeida Dutra and Orlando Graziani Povoas Barsottini

Behçet’s disease (BD) is an inflammatory disorder characterized by recurrent oral and genital ulcers, ocular inflammation, arthritis and skin lesions. Neuro-Behçet’s disease (NBD) is found in 5–30% of patients and is classified into parenchymal and non-parenchymal manifestations. Most common parenchymal NBD manifestation is brainstem meningoencephalitis and patients may also have cranial nerve palsies, myelitis, epilepsy and peripheral neuropathy. Non-parenchymal NBD manifestations are cerebral venous thrombosis and aseptic meningitis. NBD usually develop abruptly and generally clear completely within weeks, however a third of patients evolve with progressive course. Moreover, patients with BD without overt neurological manifestations may present silent neurological involvement, with abnormal findings on neuropsychological, neurophysiological and neuroimaging studies. NBD is an adverse prognostic factor. Herein we review NBD manifestations and its treatment.