Neuro-Behcet's syndrome: Case report and literature review

Abstract

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

• Neuro-Behcet disease
• Behcet syndrome
• Pons
• Ventral medulla
• Hyperintense punctate foci
• MRI