Abstract
We describe a case of neuro-Behçet disease diagnosed in a 12-year-old girl. This patient presented with recurrent oral ulcers, incontinence, spastic gait, blurry vision, and asymmetrical lower extremity hypertonia. Extensive testing revealed punctate lesions through the central nervous system, vitritis, papillitis, and uveitis. A thorough infectious and neoplastic workup was negative. She was treated with pulse steroids and azathioprine with gradual improvement in her gait and ophthalmologic findings. Although rare, primary neuro-Behçet should be considered in pediatric patients with neurologic abnormalities and recurrent aphthous ulcers without other explanation.