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.
Department of Neurology, University of Kentucky College of Medicine, Kentucky Clinic L445, Lexington, KY 40536-0284, USA.
Abdullah
A
Al Sawaf
Department of Neurology, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce St., Gates W, Philadelphia, PA 19104, USA.
Joseph R
JR
Berger
D000328:Adult; D001528:Behcet Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009188:Myelitis, Transverse / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology
Basilar meningitis; Behçet's disease; HLA B51; LETM; Transverse myelitis; Uveitis