Longitudinally extensive transverse myelitis as the sole presentation of neuro-Behçet's disease responding to infliximab

Abstract

CONTEXT: Isolated involvement of the spinal cord is an uncommon presentation of neuro-Behçet's disease (NBD) and it is associated with a poor prognosis for functional recovery. METHOD: A case report of an 18-year-old Turkish man who presented with a progressive paraparesis and bladder dysfunction secondary to a longitudinally extensive transverse myelitis as the sole presentation of NBD. FINDINGS: Examination revealed a spastic paraparesis and a T7 sensory level. Magnetic resonance imaging revealed multiple enhancing lesions throughout the thoracic cord and cerebrospinal fluid showed intense neutrophilia. On further enquiry a family history of Behçet's disease was elicited. The patient subsequently reported a history of recurrent oral ulceration and intermittent occular inflammation. A diagnosis of NBD was made and intravenous high-dose steroids commenced with poor response. In view of the poor prognosis for functional recovery associated with spinal NBD the patient was treated with infliximab, an anti-tumour necrosis factor-alpha monoclonal antibody, leading to excellent recovery of function. CONCLUSION/CLINICAL RELEVANCE: Early treatment with infliximab may facilitate a favourable functional recovery and should be considered in cases of NBD with spinal cord involvement.