Neuro-Behçet disease. A review

Abstract

Background: Behçet disease is a vasculitis with mucocutaneous, ocular, arthritic, vascular, and other manifestations. Its neurologic manifestations (neuro-Behçet disease) are relatively rare, but they must be thoroughly investigated due to their grave prognosis.

Review summary: The frequency of neurologic manifestations, more common in male Behçet patients, is between 5% and 30%. Both the central and peripheral nervous systems can be involved. Central nervous system manifestations can be divided into 2 main groups: (1) parenchymal involvement, which includes brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitic presentations; (2) nonparenchymal involvement, including dural sinus thrombosis, arterial occlusion, and/or aneurysms. Peripheral neuropathy and myopathy are relatively rare. Cerebrospinal fluid analysis reveals pleocytosis and elevated protein levels. Magnetic resonance imaging is the investigation of choice which often reveals iso-/hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images, mostly in the mesodiencephalic junction, cerebellar peduncles, and other parts of the brainstem. Corticosteroids and adjuvant immunosuppressive therapy are used for parenchymal manifestations, and corticosteroids and anticoagulants are used for treatment of dural sinus thrombosis.

Conclusion: Neuro-Behçet disease must be considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial sinovenous occlusive diseases, and other neurologic syndromes.