Behçet’s disease is a systemic vasculitis characterized by oral and genital ulcers, arthritis, uveitis and thrombophlebitis. Neuro-Behçet’s describes CNS manifestations of the disease.

A 35 year old male with Behçet’s syndrome presented with worsening bilateral lower extremity weakness. History was significant for aseptic meningitis. The patient was treated with cyclophosphamide and transitioned to maintenance azathioprine and colchicine. Nevertheless, he described 5 years of worsening leg weakness, associated with feeling his legs giving away as well as nighttime leg spasms. He currently uses a wheelchair.

Motor examination showed normal bulk, yet decreased power in the bilateral hip flexors and extensors and knee flexors and extensors. Lower extremities were flaccid, with hyperreflexia and extensor plantar responses. Non-sustained clonus was present in the ankles. Upper extremities were spastic with increased tone. Sensory examination revealed decreased proprioception at the ankles. Gait was spastic with scissoring.

Laboratory testing revealed low vitamin B12 levels. A brain CT showed mild volume loss. MRI showed a small, patchy, non-enhancing T2 hyperintense signal in the left ventral pons between the upper and middle cerebellar peduncles, representing demyelinating disease. MRI of the rest of the neuroaxis revealed atrophy of the cerebellum and longitudinal corticospinal tracts. Genetic testing was negative for spinocerebellar ataxia 1-3. A repeat MRI showed increased T2/FLAIR hyperintensity along the ventral medulla. This was attributed to colchicine toxicity, and the patient was switched to adalimumab, which also had intolerable side effects.