Cerebral vaskulit — the disease caused by inflammatory process in a wall of brain vessels. Arises generally again. Manifestations are variable: encephalopathy, paresis, mental violations, epileptic attacks, faints, visual frustration, relative deafness, ataxy. Diagnostics is guided by the clinical information supplied the neurologic status, results of MRT, cerebral angiography, a research of a likvor, blood biochemistry. Treatment is carried out differentially according to an etiology and clinical features. Can include corticosteroids, cytostatics, vascular medicines, nootropa, symptomatic means.
Cerebral Vaskulit (CV) arises mainly in structure of system vaskulit or against the background of infections, rheumatic diseases, an oncopathology, intoxications. The term "vaskulit" means inflammatory defeat of a vascular wall. Isolated vaskulit brain vessels treats rare forms. Exact incidence is not established as there are no specific kliniko-tool diagnostic criteria of TsV. A number of authors indicate prevalence of pathology — 2-3 cases 100 thousand people. Persons are subject from 7 to 71 years to a disease, it is the most frequent – representatives of age category from 30 to 60 years. Gender distinctions in incidence are not traced.
Reasons of a cerebral vaskulit
The etiology of the idiopathic (primary) isolated defeat of cerebral vessels is unknown. The role of traumatic damages, stresses, overcoolings as the triggers provoking a debut of a vaskulit is not excluded. In literature on neurology cases of development of a disease after a craniocereberal trauma are described. Can act as the reasons of secondary TsV:
- System defeat of vessels of nonspecific inflammatory genesis. Takayasu's disease, a syndrome of Cherdzha-Stross, microscopic poliangiit, nodular periarteriit, hemorrhagic vaskulit proceed with involvement of the vascular course of TsNS.
- Rheumatic diseases: Hard currency, rheumatoid arthritis, system sklerodermiya, Shegren's syndrome. More rare is noted damages of cerebral arteries in comparison with visceral that is caused by work of a hematoencephalic barrier.
- Infectious diseases: syphilis, tuberculosis, sypny typhus, herpetic infection, trichinosis, listeriosis. The vascular inflammation is provoked by infectious agents and their toxins.
- Intoxications. Cerebral is described vaskulit at abuse of amphetamine, a kokainomaniye, "pharmaceutical" drug addiction.
- Oncological diseases. Difficulties of diagnostics cause rare detection of TsV at neoplastic processes.
The mechanism of development of the isolated TsV is not installed. Morphologically in a vascular wall infiltrates (a congestion of one-nuclear cages) come to light, formation of granulomas is observed. Secondary cerebral vaskulit at system vascular and rheumatic diseases has autoimmune pathogenesis: the vascular wall is damaged by the antibodies which are developed to its elements owing to inadequate reaction of immune system. In other cases the inflammatory mechanism is started by direct influence of an etiofaktor (toxins, bacteria, viruses). The inflammation of a vascular wall leads to its thinning, narrowing of a vascular gleam, the increased permeability. Haemo dynamic frustration develop, blood supply of certain sites of a brain worsens, there are episodes of cerebral ischemia, lacunary heart attacks, melkoochagovy hemorrhages. Usually cerebral process has widespread multiple character.
Essential distinctions during idiopathic and the TsV secondary forms are observed. Therefore division of a disease according to an etiology has the clinical importance on:
- Primary cerebral vaskulit — idiopathic inflammatory changes of exclusively brain arteries. System vascular defeat, background diseases are absent.
- Secondary forms — inflammatory process in a wall of arteries results from the main disease. Make the vast majority of cases of TsV.
Like system vaskulita, cerebral process proceeds with primary involvement of arteries of a certain caliber. Depending on diameter allocate:
- TsV with defeat of large vascular trunks. It is observed at Takayasu's disease, temporal arteritis.
- TsV with defeat of vessels of small and average caliber. It is characteristic of a microscopic poliangiit, a system volchanka.
Symptoms of a cerebral vaskulit
Primary TsV has a sharp demonstration from an intensive headache, an epileptic paroxysm or sudden emergence of focal neurologic deficiency. Certain researchers point to a possibility of the long subclinical period preceding a disease debut. In the subsequent one of the following options of symptomatology is implemented: sharp encephalopathy with mental disorders, the multifocal manifestations similar to clinic of multiple sclerosis, the all-brain and focal symptoms typical for volume formation of a brain. Pyramidal insufficiency in the form of paresis of one is most characteristic, is more often than two, extremities with increase in a tone of muscles and reflexes. A number of cases is followed by stovolovo-cerebellar simptomokompleks: (twitching of eyeballs), cerebellar ataxy (unsteadiness of gait, diskoordination, disproportion of movements), disorder of glazodvigatelny function. Violations of the speech (aphasia), loss of a part of visual fields are possible (gemianopsiya), a convulsive syndrome (symptomatic epilepsy).
Secondary TsV differs in gradual increase of manifestations. In an initial stage patients complain of deterioration in hearing, sight easing, a headache, preunconscious episodes, omission of an upper eyelid. The developed period depends on the main pathology. Involvement of brain vessels within a system vaskulit is shown by giperkineza (involuntary motive acts), faints, episodes of a cataplexy and a narcolepsy, convulsive attacks. Cerebral vaskulit a rheumatic etiology it is characterized by clinic passing small trochees with pristupoobrazny emergence of a giperkinez. Vaskulit a brain at hard currency in 60% of cases proceeds with tranzitorny mental deviations (concern, disorder of behavior, psychoses). As frequent manifestations of TsV of tubercular genesis paresis, , a dizartriya, orientation violations acts. At rickettsial diseases comas, convulsive paroxysms are observed.
Sharp disorder of brain blood supply in a zone of the brain artery affected vaskulity leads to developing of a stroke. The melkoochagovy ischemic strokes having repeated character are more often observed. Thinning of pathologically changed vascular wall can be complicated by a gap and a hemorrhagic stroke. The chronic ischemia of a brain arising owing to a vaskulit leads to decrease in cognitive functions (memory, attention, thinking), to formation of dementia. A complication of a convulsive syndrome is the epileptic status. In rare instances the course of a disease can lead to development of a coma.
Heterogeneity of mechanisms of emergence, current, clinical picture TsV significantly complicate diagnosis, demand participation of several experts: neurologist, rheumatologist, infectiologist, psychiatrist. The identification/exception of a basic disease is important. The main stages of a diagnostic algorithm are:
- Neurologic survey. Reveals pyramidal disorders, pathological reflexes, symptoms of dysfunction of a cerebellum and a trunk of a brain, symptoms of intra cranial hypertensia.
- Consultation of the ophthalmologist. Includes visual acuity check, an oftalmoskopiya, perimetry. Defines decrease in sight, puffiness of disks of optic nerves, a gemianopsiya.
- Brain MRT. In a debut of a disease can not fix pathological changes. In the subsequent pathology on MRT is diagnosed for 50-65% of patients. Mainly multiple small centers in brain substance, puffiness of brain substance, a zone of the postponed lacunary heart attacks, sharp ischemic episodes are observed.
- Cerebral angiography. It can be carried out radiological and by means of MRT of vessels. According to various data, it is possible to reveal vascular changes at 40-90% of patients. On angiograms the smazannost of a vascular contour, narrowing, sites of a dilyatation, interruption, occlusion, existence multiple kollateraly is noted.
- UZDG and duplex scanning of a cerebral blood-groove reveal nonspecific changes of haemo dynamics which can be result of other vascular diseases. It is sometimes used in dynamics assessment against the background of the carried-out therapy.
- Research of tserebrospinalny liquid. Can not reveal deviations. At rheumatic genesis of a vaskulit moderately increased concentration of protein is observed . Definition of an infectious etiology is promoted by PTsR, RIF with a likvor.
- Biochemical blood test. Allows to find existence of markers of rheumatic and autoimmune diseases. Includes the analysis to the Russian Federation, SRB, antibodies to Sm and Scl-70, volchanochny anticoagulant, a complement of C3 and C4, anti-nuclear antibodies.
- Biopsy of a cerebral parenchyma. The research of bioptat allows to reveal inflammatory changes of arteries of small caliber. However the site with the changed vessels can not get to biopsiyny material. Damage of large arteries which biopsy is not carried out is possible.
To differentiate cerebral vaskulit follows with multifocal encephalitis, cerebral atherosclerosis, demiyeliniziruyushchy pathology (multiple sclerosis, optikomiyelity, Balo's sclerosis). At young patients it is necessary to exclude an anti-phospholipidic syndrome.
Treatment of a cerebral vaskulit
Therapy of the isolated forms, secondary cerebral defeat at system and rheumatic vaskulita is carried out by glucocorticosteroids. Consists of 2 stages: the shock and supporting treatment. In hard cases steroids combine with cytostatics (azatiopriny, tsiklofosfamidy). Basic therapy of other options of a secondary vaskulit depends on the main pathology. The infectious etiology demands the corresponding antibacterial or antiviral treatment, toxic — desintoxication.
For the purpose of improvement of a brain blood-groove vazoaktivny medicines, means the improving rheological properties of blood are applied. Maintenance of metabolism of nervous cages, stimulation of cognitive functions is carried out by purpose of nootrop. Complex treatment includes symptomatic therapy, physiotherapy exercises and massage of paretichny extremities, occupations with the logopedist (at speech disturbance) etc.
Forecast and prevention
In general cerebral vaskulit gives in to treatment and has the favorable forecast. Some neurologists point to the best effect of therapy at patients with the MR-centers of Difficulty of diagnostics which are well accumulating contrast in some cases lead to overdue diagnosis and the late beginning of therapy that causes progressing of symptomatology to a deep invalidization, a lethal outcome. Specific prevention is absent. The prevention of secondary TsV comes down to an exception of intoxications, timely treatment of infections and system diseases.