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

Abstract

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