Longitudinally extensive spinal cord lesion in a case of Neuro-Behçet disease]

A 56-year-old right-handed man with recurrent orogenital aphtoid ulcers and bilateral uveitis had presented with memory disturbance, dressing apraxia and constructional apraxia at age 53. Neuro-Behçet disease was diagnosed based on pathergy test results and positivity for HLA-B51. Four months after azathioprine was introduced, he presented with subacute spastic paraparesis and urinary retention at age 56. Neurological examination demonstrated hyperreflexia in the lower limbs without pathological reflexes. He also showed memory disturbance, dressing apraxia and constructional apraxia. Spinal cord MRI showed a longitudinally extensive spinal cord lesion (LESCL) from C1 to Th3 with partial gadolinium enhancement from C6 to C8. Brain MRI showed moderate atrophy of the right temporal and parietal lobes without contrast enhanced lesion. There were hyperintense lesions in the pons, bilateral periventricular white matter and right parietal subcortical white matter. Cerebrospinal fluid analysis showed mild lymphocytic pleocytosis. After intravenous methylprednisolone treatment, clinical symptoms largely resolved and the abnormal intensities with contrast enhancement of the cord disappeared. However, higher cortical dysfunctions were not changed. LESCL may reflect inflammatory venous vasculitis with edema extending along the neural fibers since the lesion shows excellent responses to steroid without neurological sequelae. Differential diagnosis of neurological diseases demonstrating LESCL should include Neuro-Behçet disease.