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

Multiple Sclerosis and Related Disorders
Abdullah Al Sawaf, Joseph R Berger

Abstract

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.

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Citations

Jul 4, 2017·Therapeutic Advances in Neurological Disorders·Sung-Min KimKazuo Fujihara
Jul 2, 2017·Clinical Neuroradiology·Stefan WeidauerMichael Nichtweiß
Jan 5, 2021·Current Neurology and Neuroscience Reports·Gina S Perez Giraldo, Jorge G Ortiz Garcia
Mar 13, 2021·Case Reports in Neurology·Shunya FujiwaraKoji Abe

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