A 25-year-old man presented with 2 days of left arm weakness and unsteadiness. Four years prior, he presented with three separate episodes of oral and scrotal ulcers. Two years later he developed pericarditis. The same year he presented with hematemesis from an antral ulcer. Six months later he was admitted for seizure and papilledema was noted to have cerebral venous sinus thrombosis. A year later, he presented with headache, hearing impairment and left sixth nerve palsy and MRI disclosed an irregular, peripheral rim-enhancing area within the pons and upper medulla suggestive of an inflammatory process. He was successfully treated with 4 weeks of steroids. A month later he was treated again for pericarditis with colchicine.
Examination revealed an alert, oriented male with left arm weakness and unsteady gait. MRI showed multiple enhancing lesions within the midbrain, cerebral peduncle and middle cerebral peduncle. Cerebrospinal fluid analysis (CSF) showed slightly elevated protein 95 mg/dl, low glucose 35 mg/dl and pleocytosis (2000/microliter) with neutrophil predominance. Bacterial meningitis was suspected, but CSF gram stain and culture remained negative. Further CSF analysis for encephalitis, flow cytometry, para neoplastic panel and oligo clonal bands were negative. He was briefly treated with broad spectrum antibiotics for suspected meningitis and steroids for presumed vasculitis
Eventually, brain biopsy of right mesial temporal lobe was obtainined, showing acute and chronic inflammation with non-granulomatous vasculitis. Prior history recurrent oral/genital ulcers, pericarditis, venous thrombosis in the context of vasculitis in brain biopsy supported a diagnosis of NBD.
He was treated with six monthly cyclophosphamide infusions while continuing prednisone. A month later his neurological exam had significantly improved with continued immunosuppressive treatment.
Discussion:
Behcet’s disease (BD), a rare multi system vasculitis, affects blood vessels of all sizes. It is more common in countries along the silk road. Estimated prevalence in United States is 1 in 170 000 individuals. Neuro Behcet’s disease (NBD) occurs in less than 10% of patients with BD. NBD is one of the most devastating manifestations of the disease and it can be parenchymal or non – parenchymal (eg: cerebral venous thrombosis).
BD is a clinical diagnosis based on recurrent oral ulcers with characteristic systemic manifestations. The diagnosis is often delayed as it may take months to years for common symptoms to appear. Early diagnosis and aggressive immunosuppressive treatment can hasten the neurological recovery of a potentially disabling disease.
Conclusions:
BD should be considered in young patients who present with neurological symptoms and recurrent oral/genital ulcers. Hence a detailed history and thorough medical record review are crucial in diagnosing BD.