Acute neuro-Behçet’s disease with longitudinal extensive transverse myelitis in a patient receiving long-term anti-TNF-α therapy

Abstract

Anti-tumor necrosis factor (TNF)-α therapy has high efficacy even for severe or refractory Behçet’s disease (BD). Here, we present the first report of a BD patient who developed neuro-Behçet’s disease (NBD) with isolated longitudinal extensive transverse myelitis (LETM) during long-term anti-TNF-α therapy. A 67-year-old woman receiving infliximab (IFX; 6 mg/kg, every 8 weeks) for 5 years presented with acute right-side-dominant paraplegia, superficial sensory disturbance and bladder dysfunction. Spinal magnetic resonance imaging (MRI) revealed spinal cord swelling with abnormal high-intensity lesions from the medulla oblongata to second thoracic vertebra (T2) on T2-weighted imaging. Analysis of cerebrospinal fluid (CSF) revealed polymorphonuclear pleocytosis (205 cells/mm3 and 94% polymorphs) and a high IL-6 concentration (13,064 pg/mL) without IgG oligoclonal bands and serum anti-aquaporin 4 antibody was negative. Neutralising antibodies against IFX were negative, and free TNF-α in serum was in a normal range 8 weeks after administration of IFX. Treatment with high-dose corticosteroid therapy including an intravenous methylprednisolone pulse and methotrexate improved limb weakness, CSF abnormalities and longitudinal spinal lesions by MRI. However, the patient retained a neurological disability for 10 months after rehabilitation. This case suggests that NBD with LETM may occur despite inhibition of TNF-α, and that anti-TNF-α therapy does not necessarily prevent the onset of acute NBD.