Modern Concepts on the Clinic and Diagnosis of Primary Vasculitis of the Central Nervous System

Primary vasculitis of the central nervous system (PACNS) is a rare form unknown cause that affects vessels brain, spinal cord and meninges without systemic damage. It was found average age onset disease 50 years. Clinical manifestations depend on caliber affected vessels. The most common initial symptoms are headache vascular cognitive impairment associated with small vessel involvement. development stroke focal interrelated manifests as lesion middle/large cerebral arteries. PACNS difficult to diagnose, since outside rare, serologic markers inflammation normal. analysis cerebrospinal fluid usually abnormal due moderate nonspecific increase in level total protein or number leukocytes. Deviations from norm (cerebral infarction, sometimes tumor-like lesions) detected according data magnetic resonance imaging brain 97 % patients PACNS. Angiography has low sensitivity specificity, it allows verify only middle large arteries, revealing segmental narrowing. To detect blood vessels, well exclude other diseases, necessary perform biopsy substance soft membranes brain.