An 18-year-old man with recurrent oral and genital ulcers presented with uveitis, encephalopathy, headache, spastic paraparesis, and urinary retention. MRI demonstrated periventricular and hypothalamic lesions, sinus thrombosis, and longitudinally extensive spinal cord lesions (figure). Treatment with high-dose steroid medication resulted in dramatic neurologic and radiographic improvement.
Figure Imaging features of neuro-Behçet disease
(A) Coronal fluid-attenuated inversion recovery sequence shows periventricular, chiasmatic, and hypothalamic edema. (B) Magnetic resonance venography reveals left transverse sinus thrombosis. (C) Axial T2-weighted sequence shows high signal primarily within the spinal cord gray matter. (D) Sagittal T2-weighted sequence demonstrates high signal intensity longitudinally throughout the cervical and thoracic spinal cord.
Behçet disease is a multisystemic inflammatory disease characterized by recurrent oral aphthae, uveitis, and genital ulcers. Two patterns of CNS involvement have been described, including direct parenchymal involvement and cerebrovascular involvement.1 Simultaneous symptomatic parenchymal, vascular, and spinal cord lesions characteristic of neuro-Behçet disease in a single patient are highly unusual.
Disclosure: The authors report no disclosures.
Address correspondence and reprint requests to Dr. Albert Y. Jin, Department of Medicine (Neurology), Queen’s University c/o Connell 7, Division of Neurology, Kingston General Hospital, Kingston, Ontario, Canada K7L 2V7; ayj@queensu.ca
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