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  • Updated 03.22.2022
  • Released 10.14.1994
  • Expires For CME 03.22.2025

Behcet disease

Introduction

Overview

Behcet disease is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin that can affect the nervous system. Clinical and imaging evidence suggests that primary neurologic involvement may be subclassified into two major phenotypes. The first, which is seen in most patients, is characterized by a vascular-inflammatory central nervous system disease with focal or multifocal parenchymal involvement. The second is caused by isolated cerebral venous sinus thrombosis and intracranial hypertension. In this article, the author describes the various clinical manifestations, summarizes the diagnosis and differential diagnosis, and gives an update on the emerging views on its epidemiology, pathogenesis, suggested diagnostic MRI patterns, and other biomarkers as well as current management issues for the neurologic disease, including the use of anti-TNF-alpha agents and other new alternatives.

Key points

• Behcet disease is a disorder of unclear pathogenesis. Several inflammatory markers and the association of Behcet disease with particular major histocompatibility complex antigens point to inflammatory mechanisms inducing the manifestations of the disease in a genetically predisposed host.

• Neurologic involvement, or neuro-Behcet syndrome (NBS), is heterogeneous with two major clinical subtypes: a central nervous system inflammatory parenchymal disease and a less common nonparenchymal form that involves large cerebral vessels, mainly venous dural sinuses.

• A history of oral or genital ulcers and uveitis should be sought in young patients who present with stroke-like syndromes or with symptoms suggestive of inflammatory-demyelinating diseases of the central nervous system as well as increased intracranial pressure or dural sinus venous thrombosis.

• New anti-inflammatory agents, in particular TNF-alpha inhibitors, currently are accepted to be the most efficient therapeutic alternative to conventional immunosuppressive drugs.

Historical note and terminology

The earliest known observations of oral and genital ulcerations associated with uveitis are in Hippocrates' notes (41). The disease was named after Dr. Hulusi Behcet (17). Since then, the manifestations of the disease have become more widely known and have been extended to include multiple systems.

Dr. Hulusi Behcet
Dr. Hulusi Behcet, a Turkish dermatologist, was the first to recognize that orogenital ulcerations and eye inflammation were the manifestations of a single disease, which came to be known as Behcet disease. (Contributed by Dr. Ban...

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