Interferon-α treatment for acute myelitis and intestinal involvement in severe Behçet’s disease

A 40-year-old HIV-negative Greek woman was admitted to the Department of Neurology at the University Medical Center of Patras with a 4-day history of progressive muscle weakness in her legs, accompanied by fever (39.5°C), diarrhea and urine retention and a 6-year history of oral and genital lesions that had relapsed 20 days prior to admission. Physical examination revealed multiple large aphthous lesions in the oral mucosa, extensive and deep anogenital ulcers, pseudofolliculitis and pathergy signs but no evidence of arthritis. Initial neurological examination showed muscle power grade 3/5 [Medical Research Council (MRC) scale] in the proximal and grade 4/5 in the distal muscles], brisk symmetrical tendon reflexes, extensor plantar response bilaterally, severe suppression of vibration sense below the knees (particularly on the right extremity) and bilateral reduction of pain appreciation below the T5 level. Neurological disability score in the expanded disability status scale (EDSS) was 6.5, indicating that bilateral assistance was required to walk 20 m. Cranial nerves and motor and sensory function of upper limbs were normal. Magnetic resonance imaging (MRI) of thoracic spinal cord revealed a long intramedullar lesion extending from C7 to T7 vertebrae with gadolinium-enhancement at T3–T4 level, whereas that of the brain showed three unenhanced small lesions at the brainstem (Figure 1.). Cerebrospinal fluid analysis revealed 60 lymphocytes/ml, normal protein and glucose content and negative oligoclonal bands. Figure 1. Magnetic resonance scan of the thoracic cord (sagittal T1-weighted images with gadolinium) showing the intramedullary enhanced lesion before (left image) and 1 month after treatment (right image). Ophthalmologic investigation disclosed posterior uveitis and spotty hemorrhagic foci on both eyes, whereas colonoscopy showed multiple deep ulcers along the large bowel. Erythrocyte sedimentation rate was 70 mm/h, HLA B51 was positive, whereas serum protein electrophoresis revealed a decrease …