ORIGINAL ARTICLE Vascular neurobehcet disease:correlation with current disease activity forum and systemic vascular involvement Reem H. A. Mohammed & Amr Nasef & Hanady H. Kewan & Mohammed Al Shaar Received: 16 July 2011 / Revised: 18 December 2011 / Accepted: 28 January 2012 / Published online: 14 March 2012 # Clinical Rheumatology 2012 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 vas- cular disease. The findings were studied in relation to dis- ease activity and features of systemic vascular involvement. Forty patients fulfilling the diagnostic criteria of the Inter- national 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 cere- bral blood vessels’ flow pattern abnormalities by transcra- nial 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 cere- brovascular disease (p 0 0.01). Patients with NBS had sig- nificantly higher disease activity index score (r 0 0.69, p 0 0.0001). Radiographic findings and flow abnormalities were significantly less in patients on immune suppressants and antiplatelet drugs (p 0 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 pro- gression and flow abnormalities, respectively. Keywords Behcet’ s Current Disease Activity Form . Behcet’ s syndrome . Magnetic resonance imaging . Neuro- Behcet disease . Transcranial Doppler ultrasound . Vascular involvement Introduction Behcet’ s disease is a chronic idiopathic multisystem, relaps- ing, vasculitic disorder [1]. The disease encompasses a variety of clinical presentations for which reason the term Behcet’ s syndrome (BS) is the term commonly used. BS typically presents with prominent mucosal inflammation which remains a universal finding in almost all patients with BS. Multisystem vascular inflammatory changes with ocular involvement and recurrent thrombosis are among the emi- nent clinical presentations of the disease. BS generally presents in the third decade of life, with a slightly increased tendency to affect men more than women, 1.4:1, and with a R. H. A. Mohammed (*) Department of Rheumatology and Rehabilitation, Cairo University, Cairo, Egypt e-mail: rmhamdy@yahoo.com A. Nasef Department of Radio-diagnosis, Faculty of Medicine, Cairo University, Cairo, Egypt H. H. Kewan Department of Radio-diagnosis, Faculty of Medicine, Mansoura University, Mansoura, Egypt M. Al Shaar Department of Neurology, Al Azhar University, Cairo, Egypt Clin Rheumatol (2012) 31:1033–1040 DOI 10.1007/s10067-012-1953-0