Longitudinally extensive transverse myelitis suspected for isolated Neuro-Behçet: A diagnostic conundrum

Behçet disease (BD) is a chronic relapsing autoimmune disease. Involvement of the nervous system occurs in 5-50% and is referred to as Neuro-Behçet's (NBD). The clinical diagnosis of NBD can be challenging, particularly when the history is atypical and the systemic manifestations of the disorder are absent or scant. We report a young Caucasian man who presented with a non-specific systemic illness evolving rapidly to a basilar meningitis with a neutrophilic pleocytosis. Shortly afterwards, he developed a cervical myelopathy and ultimately a longitudinally extensive transverse myelitis and brainstem involvement with an active uveitis. There was no history of recurrent oral aphthous ulcers or genital ulcers, other skin lesions, or thrombophlebitis. The diagnosis was supported by the clinical, radiographic and laboratory findings including heterozygosity for the HLA-B51 allele on genetic testing. NBD must be included in the differential diagnosis of longitudinally extensive transverse myelitis, especially when it is associated with uveitis.