Behcet disease

Aksel Siva MD (Dr. Siva of the Istanbul University Cerrahpasa School of Medicine received honorariums from Bayer-Schering AG and Teva for speaking engagements, fees from Novartis as a consultant and advisory committee member, consultation fees and honorariums from Biogen as an advisory committee member, honorariums and an unrestricted research grant from Merck-Serono for speaking engagements, international congress travel and registration reimbursements from Gen Ilac and Genzyme, and honorariums from Genzyme for consulting.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Express Scripts, Isis, and Merck.)
Originally released October 14, 1994; last updated August 12, 2016; expires August 12, 2019

This article includes discussion of Behcet disease, multiple symptom complex of Behcet, Morbus Behcet, Behcet recurrent disease, oculobuccogenital syndrome, Adamantiades-Behcet disease, triple symptom complex, silk route disease, neurobehçet, neurobehcet, neuro-Behçet, neuro-Behcet, vasculo-Behcet, entero-Behcet, incomplete Behcet disease, complete Behcet disease, mucocutaneous form of Behcet disease, and neuro-ocular form of Behcet disease. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

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 2 major forms. The first form, which is seen in the majority of patients, may be characterized by a vascular-inflammatory central nervous system disease with focal or multifocal parenchymal involvement. The other form 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 experience-based management issues for the neurologic disease.

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 2 major clinical subtypes with distinct clinical, imaging, pathological, and prognostic features: a central nervous system inflammatory parenchymal disease and a less common nonparenchymal form that involves large extraparenchymal vascular structures, mainly venous dural sinuses. In contrast, dural venous sinus thrombosis is not uncommon in most cases of pediatric NBS. Peripheral nervous system involvement may be seen but is extremely rare.

 

• NBS is a great mimicker of various central nervous system disorders. A history of oral or genital ulcers should be sought in any patient with brainstem and white matter lesions, increased intracranial pressure, venous thromboses, or brainstem and cerebellar atrophy unexplained otherwise.

 

• New anti-inflammatory agents, in particular TNF-alpha inhibitors, are likely to provide a more efficient therapeutic alternative to conventional immunosuppressive drugs in this syndrome in which therapeutic options are limited.

Historical note and terminology

The earliest known observations of oral and genital ulcerations associated with uveitis are in Hippocrates' notes (Feigenbaum 1956). The disease was named after Dr. Hulusi Behcet (Behcet 1937).

Image: Dr. Hulusi Behcet
Since then, the manifestations of the disease have become more widely known and have been extended to include multiple systems.

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