Vascular neurobehcet disease: correlation with current disease activity forum and systemic vascular involvement

Clin Rheumatol. 2012 Jul;31(7):1033-40. doi: 10.1007/s10067-012-1953-0. Epub 2012 Mar 14.

Abstract

Behcet's syndrome (BS) is a chronic relapsing vascular inflammatory disease of unknown etiology with high morbidity and mortality. This research aims to study the clinical patterns of CNS disease in a group of patients with BS as well as the frequency and type of the associated radiographic findings suggestive of structural cerebral vascular disease. The findings were studied in relation to disease activity and features of systemic vascular involvement. Forty patients fulfilling the diagnostic criteria of the International Study Group for Behcet's Disease, mean age of 33.56 ± 9.7 years, were enrolled. Patients were subjected to magnetic resonance imaging with conjugate survey of cerebral blood vessels' flow pattern abnormalities by transcranial Doppler study. Thirty healthy controls were included. Behcet's Disease Current Activity Form Score was used. Neuro-Behcet's syndrome (NBS) was diagnosed in 37.5% with headache being the most common (86.6% of cases), pyramidal affection (signs of upper motor neuron lesions/hemiplegia) was reported in 33.3%, attacks of disturbed conscious level in 26.6%, and cranial nerve affection in 6.5%. Of the patients, 66.6% with clinical features of NBS had statistically significant radiographic evidences of cerebrovascular disease (p = 0.01). Patients with NBS had significantly higher disease activity index score (r = 0.69, p = 0.0001). Radiographic findings and flow abnormalities were significantly less in patients on immune suppressants and antiplatelet drugs (p = 0.003, 0.04). BS patients with clinical neurologic disease were found to have radiographic findings suggestive of cerebral vascular disease with high disease activity index score. Drugs like immunosuppressants and oral antiplatelets might retard cerebral vascular disease progression and flow abnormalities, respectively.

MeSH terms

  • Adult
  • Behcet Syndrome / diagnostic imaging
  • Behcet Syndrome / pathology
  • Behcet Syndrome / physiopathology*
  • Cerebrovascular Circulation / physiology*
  • Cross-Sectional Studies
  • Disease Progression
  • Female
  • Headache / diagnostic imaging
  • Headache / pathology
  • Headache / physiopathology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Regional Blood Flow / physiology*
  • Severity of Illness Index
  • Stroke / diagnostic imaging
  • Stroke / pathology
  • Stroke / physiopathology*
  • Ultrasonography