Background/Purpose: Spinal cord involvement may occur in the course Behcet’s Disease (BD). It may present with distinct manifestations such as sphincter and/or sexual dysfunction. We aimed to investigate the frequency and clinical features of spinal cord involvement of BD in our Hacettepe University Vasculitis Center (HUVAC) cohort.

Methods: Patients enrolled in prospective HUVAC database were searched in terms of spinal cord involvement of BD. Of 1585 patients recorded since October 2014, 419 patients were BD patients (329 complete and 90 incomplete for ISG Criteria), and 77 (18.4%) of them had NBD (61 definite, 16 possible) according to International Consensus Recommendations (ICR). Spinal cord involvement was diagnosed with neurological examination and spinal magnetic resonance imaging  (MRI). Demographics, clinical features and treatment characteristics of patients were evaluated.

Results: Fifty two (12.4%) patients had parenchymal central nervous system involvement (CNS) whereas 12 (2.8%) had spinal cord involvement. The mean age at diagnosis was not different for spinal with  pNBD and spinal without  pNBD (24.9 ± 5.4 vs. 29.2 ± 9.24, p=0.22). Median time period between diagnosis of BD and NBD is 5.29 years (IQR=9.95).

Detailed clinical features of BD patients with spinal cord involvement were summarized in Table. Three patients had total atrophy of spinal cord. Servical and/or thoracic segments of spinal cord was predominantly involved. Twenty five percent of spinal with pNBD had only one attack of NBD. Corticosteroids (IV pulse = 37/49, 75.5% and oral maintenance =45/50, 90%), interferon (40/52 79%) and cyclophosphamide (28/49, 57.1%) were the most frequently preferred treatment regimens for parenchymal NBD. Over half of them had more than one attack. Almost all had sphincter dysfunction.  One third of patients had deceased.

Conclusion: Spinal cord involvement is very rare in BD. However, it has a big impact on morbidity and mortality of BD patients. Awareness, early diagnosis and recent effective biologic agents might decrease this dramatic scenario.