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

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.

Effectiveness and tuberculosis-related safety profile of interleukin-1 blocking agents in the management of Behçet's disease

“…Indeed, the central nervous system is more frequently involved than the peripheral one [54], and the typical manifestations include focal necrotic cerebral lesions, mostly of the brainstem and basal ganglia, vascular thrombosis, arterial vasculitis, and aseptic meningo-encephalitis. On the other hand, nonparenchymal neurological BD is mostly secondary to cerebral venous sinus thrombosis [55,56]. Finally, the peripheral nervous system may rarely be involved in BD, causing peripheral neuropathies or multiplex mononeuritis [57].…”

Headache Attributed to Cranial or Cervical Vascular Disorders

“…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 [28]. Migraine without aura did prove to be the most frequent type of headache in BS patients (with and without neurological involvement) and there were no differences in the frequency of the different pattern of headache between BS patients and controls [29].…”

Posterior Reversible Encephalopathy Syndrome in a Woman With Behcet’s Disease: Case Report

“…Neuro-Behcet is the main consideration in the differential diagnosis in this patient. The clinical presentation with a reversible course and the neuroimaging findings would be highly atypical for a small venous inflammatory disease of the brain typical of Behcet's disease [6,7], supporting the diagnosis of PRES. This was the reason why an invasive conventional angiography was not performed.…”