E-Pearls of the Week: Neuro-Behcet disease
June 11, 2013
Neurologic complications of Behcet's disease were first described in 1993. The frequency of neurologic involvement ranges from 5-10%. Sequelae can include subacute meningoencephalitis, vascular thromboses, and intra- as well as extra-cranial aneurysms. MRI is critical in diagnosis and establishes evidence for parenchymal damage. A recent case series of pediatric patients with Neuro-Behcet's disease revealed 89% had cerebral venous sinus thrombosis and 11% had parenchymal damage. The most common symptoms were headache, seizure, ophthalmoparesis, and hemiplegia. The acute treatment of Neuro-Behcet's disease includes immunomodulation with high-dose corticosteroids with good outcome in the majority of cases. Long-term treatment is often guided by the systemic symptoms and includes azathioprine, cyclophosphamide, and TNF-alpha inhibitors.
1. Adnan Al-Araji, and Desmond P Kidd Neuro-Behçet's disease: epidemiology, clinical characteristics, and management. Lancet Neurology 2009; 8: 192-204.
2. Uluduz D, Kürtüncü M, Yapıcı Z et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology 2011; 77: 1900-1905.
Submitted by Adam Numis, M.D. Resident Physician, University of California, San Francisco
Disclosure: Dr. Numis serves on the editorial team for the Neurology Resident and Fellow Section.