Clinical characteristics and course of spinal cord involvement in Behçet's disease

Eur J Neurol. 2007 Jul;14(7):729-37. doi: 10.1111/j.1468-1331.2007.01754.x.


Parenchymal neurological involvement in Behçet's disease (p-NBD) usually presents with a brainstem syndrome; occasionally spinal cord may also be involved. Files of patients with Behçet's disease and spinal cord involvement were reviewed retrospectively, in comparison with other types of p-NBD. Amongst 216 patients with p-NBD, 24 had spinal cord involvement (11%). Most commonly patients presented with sensory-motor symptoms, sphincter and/or sexual dysfunction evolving over days. Four of 10 patients showed single or multiple cervical and/or dorsal lesions on spinal MRI's and one showed dorsal atrophy. Although the clinical picture was variable, it tended to be severe; seven cases had primary progressive course, 11 cases had a secondary progressive course after initial attack(s), four had attacks with severe residual sequela and two had improvement after attacks. After a median follow-up period of 67 months, eight were independent and 14 were dead or dependent, whereas amongst the remaining patients with p-NBD, 113 patients were independent and 56 patients were dead or dependent (P < 0.05). Our study suggests that spinal cord involvement has even worse prognosis compared with other types of p-NBD. Therefore, recognition of spinal cord involvement in Behçet's patients should prompt early vigorous treatment.

MeSH terms

  • Adolescent
  • Adult
  • Atrophy
  • Behcet Syndrome / complications
  • Behcet Syndrome / drug therapy
  • Behcet Syndrome / pathology*
  • Child
  • Cognition Disorders / etiology
  • Cohort Studies
  • Disease Progression
  • Evoked Potentials, Somatosensory
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Paraparesis / etiology
  • Sensation Disorders / etiology
  • Sexual Dysfunction, Physiological / etiology
  • Spinal Cord / pathology*
  • Urination Disorders / etiology