Granulomatous vasculitis of the central nervous system
Introduction
Introduction to central nervous system granulomatous vasculitis The central nervous system granulomatous angiitis of the central vascular vasculitis (granulomatousangiitis of the central nervvous system) is also known as the primary central nervous system of the central vasculitis (PACNS), or isolated central nervous system of the vascular vasculitis (isolated angiitis of the central nervous system). This vasculitis does not have vasculitis and other inflammatory manifestations in other parts of the body, and vasculitis changes are limited to the blood vessels of the central nervous system. Before cerebral angiography and brain biopsy were used in clinical practice, due to the lack of clinical features of the disease and people's neglect, the clinical diagnosis and reporting of central nervous system granulomatous vasculitis before death is rare. In fact, PACNS is It is not uncommon in clinical practice. In recent years, with the application of cerebral angiography and stereotactic brain tissue biopsy techniques, as well as the clinician's understanding of central nervous system granulomatous vasculitis, reports of central nervous system granulomatous vasculitis have gradually increased. basic knowledge The proportion of illness: 0.0035% Susceptible people: no specific population Mode of infection: non-infectious Complications: lymphoma leukemia consciousness disorder intelligent disorder dementia
Cause
Causes of central nervous system granulomatous vasculitis
Virus infection (30%):
The etiology of central nervous system granulomatous vasculitis is still unclear, presumably related to viral infection. It is reported in the literature that in patients with central nervous system granulomatous vasculitis, it has been found with hepatitis B virus, chickenpox - Serological evidence of herpes zoster virus, HIV and other related viral infections. This evidence supports the possible involvement of the virus in the pathogenesis of primary central nervous system vasculitis.
Pathogen effects (30%):
However, the exact mechanism of vascular damage in the central nervous system granulomatous vasculitis is still unclear, and there is no direct evidence to support the mediation of immune complexes. It is speculated that the pathogen may directly affect vascular tissue, or more likely it is a virus-vascular cell. The interaction between the two makes the structure change, which leads to secondary immune destruction. Clinical observation of immunosuppressive therapy can improve the symptoms and course of the patients with central nervous system granulomatous vasculitis, and also support the central nervous system granulomatous vasculitis. The onset may be an immune stimulating mechanism.
Major pathological changes:
The pathological changes in patients with different central nervous system granulomatous vasculitis vary to a large extent.
The most common pathological changes in the brain are multiple infarcts or hemorrhagic foci. Occasionally, large infarcts or hemorrhagic foci are seen, and the formation of cerebral palsy (hookback or cerebellar tonsil) is rare.
The blood vessels of the brain and spinal cord include large intracranial blood vessels, and the internal carotid artery and vertebral artery can be involved. However, the small blood vessels on the pia mater are most susceptible, the arterial involvement is more obvious, and the affected blood vessels have segmental necrosis or granulation. Swollen vasculitis, the blood vessels have obvious inflammatory reaction, mainly involving the middle and outer membrane of blood vessels. Commonly, lymphocytes, monocytes, tissue cells and plasma cells are infiltrated, and occasionally giant cells (Langhans giant cells) are mainly seen. Infiltration, inflammatory reactions and fibrous necrosis around the arteries are rarely seen, and infiltration of multinucleated giant cells is generally not observed.
Pathogenesis
It has been reported that pathogens may occur in three different ways in the pathogenesis of central nervous system granulomatous vasculitis:
1 affecting or infecting vascular tissue through its direct toxicity;
2 through the immune mechanism;
3 through the combined effect of the above two aspects.
Prevention
Central nervous system granulomatous vasculitis prevention
There are no better preventive measures, early diagnosis and early treatment. In the remission period, the hormone maintenance dose should be taken for several months, which can improve the prognosis.
Complication
Central nervous system granulomatous vasculitis complications Complications lymphoma leukemia consciousness disorder intelligent disorder dementia
Often associated with immunosuppressive diseases, such as lymphoma, leukemia, etc., patients may also have symptoms of consciousness, mental state changes, mental disorders, and even dementia.
Symptom
Central nervous system granulomatous vasculitis symptoms common symptoms hemianopia consciousness disorder sensory disorder paraplegia personality change coma convulsion
1. Can occur at any age, the previous body is more healthy, the average age of onset is 40 years old (3 to 74 years old), both men and women can be affected, slightly more males, about 60% of patients with acute or subacute onset, but Some patients may experience a slow progression of mental decline, as well as a course of focal neurological damage, which can resolve itself in the early stages of the disease.
2. The most common symptom is headache. According to the literature, the incidence rate is about 58%. About 80% of patients have symptoms and signs of central nervous system damage, which may be hemiplegia, lower limb paraplegia, quadriplegia, and partial sensation. Symptoms, hemianopia, convulsions and other symptoms of focal brain damage; can also be manifested as psychiatric symptoms, personality changes, varying degrees of disturbance of consciousness, even coma and other multifocal, diffuse lesions, about one-third of patients with brain Symptoms of nerve damage, common facial nerve and nerve damage, manifested as peripheral facial paralysis, eye movement disorders and so on.
3. A small number of patients may have spinal cord damage, generally no fever, weight loss, joint pain and other systemic symptoms, and does not affect the performance of other organs.
Examine
Examination of central nervous system granulomatous vasculitis
1. Blood, urine, and routine examinations are more normal.
2. ESR is mostly normal, a small number can increase, no evidence of other autoimmune diseases.
3. Lumbar puncture CSF examination: 80% of patients have abnormalities, CSF pressure is normal, colorless and transparent, the number of cells can be increased or normal, mainly lymphocyte growth, normal sugar and chloride content, slightly increased protein.
4. EEG examination: About 81% of patients have abnormalities, mostly non-specific diffuse slow waves.
5. Brain CT or MRI examination showed great differences in the performance of different patients. Most of them showed low-density ischemic lesions or infarcts with different sizes, which could be asymmetry-enhanced.
6. Cerebral angiography: There is a certain diagnostic value. More than 71% of patients have typical vascular multifocal lesions, uneven blood vessels, segmental stenosis or dilatation, vascular occlusion and delayed vascular emptying. Etc., like "sausage"-like changes, suggesting the presence of vasculitis in the brain, but it is not clear that it is vasculitis of the disease. Other brain vasculitis may have similar manifestations, some may be due to the affected blood vessels too Small, cerebral angiography can be normal.
7. Brain biopsy: Stereotactic biopsy of brain lesions is decisive for the diagnosis of PACNS. The pathology of biopsy tissue can be seen in segmental necrosis of small blood vessels, or granulomatous vasculitis, and the vessel wall is obvious. Inflammatory manifestations, often infiltration of lymphocytes, monocytes, tissue cells and plasma cells, in order to improve the accuracy of its diagnosis, it is recommended to take the cortex and pia mater at the same time.
Diagnosis
Diagnosis and diagnosis of central nervous system granulomatous vasculitis
Central nervous system granulomatous vasculitis lacks characteristic clinical manifestations, clinical diagnosis is difficult, but for patients with headache, dementia, mental state changes, symptoms and signs of focal nerve damage, myelopathy and cranial neuropathy, When it is impossible to explain with other diseases, the diagnosis of central nervous system granulomatous vasculitis should be highly suspected.
Cerebral angiography and combined with stereotactic brain tissue biopsy have decisive value for diagnosis.
In the highly suspected central nervous system granulomatous vasculitis, it should be differentiated from the following diseases, such as systemic vasculitis (nodular polyarteritis, allergic vasculitis, temporal arteritis, Behcet's disease, connective tissue diseased blood vessels) Inflammation, etc., infection (viral infection, bacterial infection, fungal infection, rickettsial infection, etc.), tumor (Hodgkin's lymphoma, leukemia, small cell lung cancer, atrial myxoma, non-Hodgkin's lymph Tumors, etc., other vascular diseases (thrombocytopenic purpura, sarcoidosis, cerebrovascular atherosclerosis, sickle-like cell anemia, etc.) and others (necrotic sarcomatoid granuloma, allergic granulomatosis, lymph Tumor-like granulomatosis, etc.), because it affects the blood vessels of the CNS, the clinical performance is similar to PACNS.
