Behcet's disease (BD) is a multisystem, autoimmune vasculitis disorder affecting small, medium, and large blood vessels, with poorly understood pathogenesis. It commonly presents with recurrent aphthous ulcers, genital ulcers, skin lesions, and bilateral uveitis. Neurological symptoms are present in less than 10% of cases and develop, on average, 5-6 years after the first non-neurological symptoms. This presentation, known as Neuro-Behcet's disease (NBD), is associated with a worse prognosis of BD. Treatment for NBD is dependent on the severity of symptoms and the presence of other systemic manifestations but often initially involves glucocorticoids and a disease-modifying agent. This case report presents a 44-year-old female patient, previously diagnosed with BD, who presented with neurological symptoms and MRI findings consistent with NBD.
Keywords: ANA, antinuclear antibodies; BD, Behcet's disease; Behcet syndrome; CNS, central nervous system; CSF, cerebrospinal fluid; CT, computed tomography; CTA, computed tomography angiography; ED, emergency department; HLA, human leukocyte antigens; Hyperintense punctate foci; IV, intravenous; LE, lower extremity; LP, lumbar puncture; MRI; MRI, magnetic resonance imaging; MS, multiple sclerosis; NBD, Neuro-Behcet's disease; NIH, National Institutes of Health; Neuro-Behcet disease; Pons; RCVS, reversible cerebral vasoconstriction syndrome; UE, upper extremity; Ventral medulla; tPA, tissue plasminogen activator.
© 2022 The Authors. Published by Elsevier Inc. on behalf of University of Washington.