Cerebral vasculitis is a disease caused by an inflammatory process in the wall of cerebral vessels. Occurs mostly secondarily. Manifestations are variable: encephalopathy, paresis, mental disorders, epileptic seizures, fainting, visual disorders, hearing loss, ataxia. Diagnosis is based on clinical data, neurological status data, MRI results, cerebral angiography, cerebrospinal fluid examination, blood biochemistry. Treatment is carried out differentially in accordance with the etiology and clinical features. It may include corticosteroids, cytostatics, vascular drugs, nootropics, symptomatic agents.
ICD 10
I67.7 Cerebral arteritis, not classified elsewhere
General information
Cerebral vasculitis (CV) occurs mainly in the structure of systemic vasculitis or against the background of infections, rheumatic diseases, oncopathology, intoxication. The term “vasculitis” means an inflammatory lesion of the vascular wall. Isolated vasculitis of cerebral vessels refers to rare forms. The exact incidence has not been established, since there are no specific clinical and instrumental diagnostic criteria for CV. A number of authors indicate the prevalence of pathology — 2-3 cases per 100 thousand people. People from 7 to 71 years old are susceptible to the disease, most often representatives of the age category from 30 to 60 years old. There are no gender differences in morbidity.
Causes
The etiology of idiopathic (primary) isolated cerebral vascular lesion is unknown. The role of traumatic injuries, stress, hypothermia as triggers provoking the onset of vasculitis is not excluded. In the literature on neurology, cases of the development of the disease after a traumatic brain injury are described. The causes of secondary CV can be:
- Systemic vascular lesion of nonspecific inflammatory genesis. Takayasu’s disease, Churge-Strauss syndrome, microscopic polyangiitis, nodular periarteritis, hemorrhagic vasculitis occur with the involvement of the vascular bed of the central nervous system.
- Rheumatic diseases: SLE, rheumatoid arthritis, systemic scleroderma, Sjogren’s syndrome. There is a more rare lesion of cerebral arteries in comparison with visceral ones, which is caused by the work of the blood-brain barrier.
- Infectious diseases: syphilis, tuberculosis, typhus, herpes infection, trichinosis, listeriosis. Vascular inflammation is provoked by the infectious agents themselves and their toxins.
- Intoxication. Cerebral vasculitis is described in the case of amphetamine abuse, cocaine addiction, “pharmacy” addiction.
- Oncological diseases. Diagnostic difficulties cause rare detection of CV in neoplastic processes.
Pathogenesis
The mechanism of development of isolated CV has not been established. Morphologically, infiltrates (clusters of mononuclear cells) are detected in the vascular wall, the formation of granulomas is observed. Secondary cerebral vasculitis in systemic vascular and rheumatic diseases has an autoimmune pathogenesis: the vascular wall is damaged by antibodies produced to its elements due to an inadequate response of the immune system. In other cases, the inflammatory mechanism is triggered by the direct action of an etiofactor (toxins, bacteria, viruses).
Inflammation of the vascular wall leads to its thinning, narrowing of the vascular lumen, increased permeability. Hemodynamic disorders develop, blood supply to certain parts of the brain deteriorates, episodes of cerebral ischemia, lacunar infarcts, small focal hemorrhages occur. Usually the cerebral process has a widespread multiple character.
Classification
There are significant differences in the course of idiopathic and secondary forms of CV. Therefore, the division of the disease according to etiology into:
- Primary cerebral vasculitis is idiopathic inflammatory changes exclusively in the cerebral arteries. Systemic vascular lesion, no background diseases.
- Secondary forms — an inflammatory process in the artery wall occurs as a result of the underlying disease. They make up the vast majority of cases of CV.
Like systemic vasculitis, the cerebral process proceeds with the predominant involvement of arteries of a certain caliber. Depending on the diameter, there are:
- CV with lesions of large vascular trunks. It is observed in Takayasu’s disease, temporal arteritis.
- CV with lesions of small and medium-sized vessels. It is characteristic of microscopic polyangiitis, systemic lupus.
Symptoms
Primary cerebral vasculitis
It has an acute manifestation with intense headache, epileptic paroxysm or sudden appearance of focal neurological deficit. Some researchers point to the possibility of a prolonged subclinical period preceding the onset of the disease. Subsequently, one of the following variants of symptoms is realized: acute encephalopathy with mental disorders, multi-focal manifestations similar to the clinic of multiple sclerosis, general cerebral and focal symptoms typical of volumetric brain formation.
The most characteristic is pyramidal insufficiency in the form of paresis of one, more often two, limbs with increased muscle tone and reflexes. A number of cases are accompanied by a brain-cerebellar symptom complex: nystagmus (twitching of the eyeballs), cerebellar ataxia (unsteadiness of gait, discoordination, disproportionality of movements), oculomotor function disorder. Speech disorders (aphasia), loss of part of the visual fields (hemianopsia), convulsive syndrome (symptomatic epilepsy) are possible.
Secondary cerebral vasculitis
It is characterized by a gradual increase in manifestations. In the initial period, patients complain of hearing impairment, impaired vision, headache, pre-fainting episodes, drooping of the upper eyelid. The extended period depends on the underlying pathology. Involvement of cerebral vessels within the framework of systemic vasculitis is manifested by hyperkinesis (involuntary motor acts), fainting, episodes of cataplexy and narcolepsy, convulsive seizures.
Cerebral vasculitis of rheumatic etiology is characterized by a clinic of transient minor chorea with paroxysmal hyperkinesis. Brain vasculitis in SLE in 60% of cases occurs with transient mental abnormalities (anxiety, behavior disorder, psychoses). Paresis, choreoathetosis, dysarthria, and disorientation are frequent manifestations of central nervous system of tuberculous genesis. With recketsiosis, comatose states, convulsive paroxysms are observed.
Complications
Acute disorder of cerebral blood supply in the area of the cerebral artery affected by vasculitis leads to a stroke. More often, small-focal ischemic strokes are observed, which are repeated in nature. Thinning of the pathologically altered vascular wall can be complicated by rupture and hemorrhagic stroke. Chronic cerebral ischemia resulting from vasculitis leads to a decrease in cognitive functions (memory, attention, thinking), the formation of dementia. A complication of convulsive syndrome is an epileptic status. In rare cases, the course of the disease can lead to the development of coma.
Diagnostics
Heterogeneity of the mechanisms of occurrence, course, clinical picture of CV significantly complicate the diagnosis, require the participation of several specialists: neurologist, rheumatologist, infectious disease specialist, psychiatrist. It is important to identify/exclude the underlying disease. The main stages of the diagnostic algorithm are:
- Neurological examination. It reveals pyramidal disorders, pathological reflexes, signs of cerebellar and brainstem dysfunction, symptoms of intracranial hypertension.
- Consultation of an ophthalmologist. Includes visual acuity testing, ophthalmoscopy, perimetry. It determines a decrease in vision, swelling of the optic nerve discs, hemianopia.
- MRI of the brain. At the onset of the disease, it may not record pathological changes. Subsequently, pathology on MRI is diagnosed in 50-65% of patients. There are mainly multiple small foci in the substance of the brain, swelling of the medulla, areas of lacunar infarcts, acute ischemic episodes.
- Cerebral angiography. It can be carried out radiologically and with the help of vascular MRI. According to various data, it is possible to detect vascular changes in 40-90% of patients. Angiograms show blurring of the vascular contour, narrowing, areas of dilatation, interruption, occlusion, and the presence of multiple collaterals.
- Ultrasound and duplex scanning of cerebral blood flow reveal nonspecific changes in hemodynamics that may be the result of other vascular diseases. It is sometimes used in assessing dynamics against the background of ongoing therapy.
- Examination of cerebrospinal fluid. It may not detect deviations. With rheumatic genesis of vasculitis, lymphocytosis is observed, moderately increased protein concentration. The determination of infectious etiology is facilitated by PCR, RIF with cerebrospinal fluid.
- Biochemical blood testing. Allows you to detect the presence of markers of rheumatic and autoimmune diseases. It includes analysis for RF, CRP, antibodies to Sm and Scl-70, lupus anticoagulant, complement C3 and C4, antinuclear antibodies.
- Cerebral parenchyma biopsy. Examination of biopsies reveals inflammatory changes in small-caliber arteries. However, the area with altered vessels may not get into the biopsy material. It is possible to damage large arteries, the biopsy of which is not carried out.
Cerebral vasculitis should be differentiated with multi-focal encephalitis, cerebral atherosclerosis, demyelinating pathology (multiple sclerosis, opticomyelitis, Balo sclerosis). Antiphospholipid syndrome should be excluded in young patients.
Treatment
Therapy of isolated forms, secondary cerebral lesions in systemic and rheumatic vasculitis is carried out with glucocorticosteroids. It consists of 2 stages: shock and supportive treatment. In severe cases, steroids are combined with cytostatics (azathioprine, cyclophosphamide). The basic therapy of other variants of secondary vasculitis depends on the underlying pathology. Infectious etiology requires appropriate antibacterial or antiviral treatment, toxic — detoxification.
In order to improve cerebral blood flow, vasoactive drugs are used, which improve the rheological properties of blood. Maintenance of the metabolism of nerve cells, stimulation of cognitive functions is carried out by the appointment of nootropics. The complex treatment includes symptomatic therapy, physical therapy and massage of paretic limbs, classes with a speech therapist (for speech disorders), etc.
Prognosis and prevention
In general, cerebral vasculitis is treatable and has a favorable prognosis. Some neurologists point to the best effect of therapy in patients with well-accumulating contrast MR foci, Diagnostic difficulties in some cases lead to delayed diagnosis and late initiation of therapy, which causes the progression of symptoms to deep disability, death. There is no specific prevention. Prevention of secondary CV is reduced to the exclusion of intoxication, timely treatment of infections and systemic diseases.