Manifestations of Neuro-Behçet’s disease: Report of two cases and review of the literature

Behçet’s disease (BD) is a rare condition, more commonly seen in Turkey, the Middle East, the Mediterranean, and the Far Eastern countries. It is a multifaced disease involving the central nervous system (CNS), skin, joints, intestines and blood vessels. CNS involvement occurs in 10–25% of the patients with BD. We report two cases with Neuro-Behçet’s disease. Both patients were females and they were in their second decade of life (early forties) when they suffered from relapsing retinitis and uveitis which led to blindness, despite treatment with steroids. They presented with clinical signs of brainstem syndrome, meningoencephalitis and the initial radiological investigations of our patients revealed encephalitic lesions in the brainstem. In the first case, an intracerebral abscess developed 4 years later, and it was evacuated under stereotactic guidance. The most common neuropathologic findings in Neuro-Behçet’s disease are focal necrotic lesions in the brain. However, the association of Behçet’s disease (BD) with cerebral abscess is very rare. There were only three reported cases of such. Ten years after the initial diagnosis of BD, our second patient suffered from subarachnoid hemorrhage due to a ruptured aneurysm of the superior cerebellar artery, which was then resected successfully. Intracranial aneurysm in patients with Behçet’s disease has previously been documented in eight patients. To our knowledge, this is the only case of Neuro-Behçet’s disease associated with an aneurysm of the superior cerebellar artery.