Neuro-Behçet’s is rare, more frequent in people from the Middle East. Vasculitis of brainstem and diencephalon and venous sinus thrombosis are typical manifestations.Case report
A previously well 51-year-old white British lady was admitted for a 2 day history of blurred vision, with impaired up-gaze and convergence retraction nystagmus. The symptoms resolved rapidly. MRI demonstrated hyperintensities in the diencephalon, CSF showed 10 mononuclear cells, normal otherwise. She was re-admitted eighteen months later with sudden imbalance, headache, slurred speech and right facial weakness. She reported mouth ulcers appearing in crops continuously over the past 2 years, no genital ulcers. MRI showed T2 hyperintensities in the thalami extending into the mid-brain, pons and superior and middle cerebellar peduncles. CSF showed 54 mononucleate cells, proteins 0.67 g, and raised CSF IL-6. The symptoms resolved spontaneously. Multimodal MRI one week later showed near resolution of the lesions. After excluding neoplastic, vascular and infective causes, probable neuro- Behçet’s was diagnosed according to the International Consensus Recommendations criteria (2014). The patient remains on steroids and azathioprine, and is asymptomatic at one year.Conclusions
Neuro-Behçet’s can present with relapsing brainstem symptoms. The diagnosis is challenging when not all the criteria for the systemic condition are met.