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

J Spinal Cord Med. 2012 Mar;35(2):122-4. doi: 10.1179/2045772311Y.0000000050. Epub 2012 Feb 8.

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Anti-Inflammatory Agents / therapeutic use*
  • Antibodies, Monoclonal / therapeutic use*
  • Behcet Syndrome / complications
  • Behcet Syndrome / drug therapy*
  • Humans
  • Infliximab
  • Magnetic Resonance Imaging
  • Male
  • Myelitis, Transverse / complications
  • Myelitis, Transverse / drug therapy*
  • Thoracic Vertebrae / pathology

Substances

  • Anti-Inflammatory Agents
  • Antibodies, Monoclonal
  • Infliximab