Nerve damage due to systemic vasculitis
Introduction
Introduction to nerve damage caused by systemic vasculitis Vasculitis, also known as inflammatory disease, refers to an inflammatory disease of blood vessels, which can be infectious or non-infectious. If multiple organs are involved, it is systemic vasculitis. If vasculitis is limited to one organ, it is called solitary vasculitis. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: hemiplegia, ataxia, small chorea, mental disorder
Cause
Causes of neurological damage caused by systemic vasculitis
Cause:
Primary vasculitis often causes damage to the nervous system, and symptoms of nervous system damage often occur early in vasculitis.
Pathogenesis
Pathological changes in primary vasculitis are characterized by inflammation and necrosis of the vessel wall, which can affect blood vessels of any type, size or location, resulting in dysfunction of the affected organ.
Prevention
Prevention of neurological damage caused by systemic vasculitis
Primary vasculitic neurological damage: There are no good 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.
Secondary vasculitic neurological damage: Treatment of primary disease is the primary measure to prevent and reduce neurological damage.
Reasonable dietary habits: Maintain a light diet. Eat more fresh vegetables and aquatic products, such as vegetables, radishes, kelp, seaweed and so on. Should eat less meals. Fasting fat meat and animal offal, do not eat spicy onions, peppers, coffee, etc. Eat less refined sugar, honey, fruit candy, cakes, etc. Strengthen nutrition and use foods that are easy to digest, contain enough calories, protein and vitamins.
Complication
Complications of neurological damage caused by systemic vasculitis Complications, hemiplegia, ataxia, small chorea, mental disorder
The symptoms and signs of the clinical symptoms are diverse. With the development of the disease, the symptoms and signs that appear may be the manifestations of the disease, and can also be regarded as different types of complications of the disease.
Symptom
Symptoms of neurological damage caused by systemic vasculitis Common symptoms Demyelination and tinnitus weakness Systemic vasculitis Dementia Muscle atrophy Diplopia Weight loss Sleepiness ataxia
The location and extent of involvement of the nervous system in different primary vasculitis vary, and the clinical manifestations of damage in different sites are also different.
Peripheral nerve involvement
Vascular inflammatory peripheral neuropathy is a common clinical manifestation of primary vasculitis. Its clinical features are: often with severe limb burning pain and sensation as the first symptom, which may have muscle weakness, muscle atrophy, paresthesia and hypothermia. The lower extremities are often affected first, and often manifest as multiple mononeuritis. They can also be expressed as symmetrical distal "glove socks" motor sensation polyneuropathy and pure sensory neuropathy. In addition, there are also reported Guillain-Barre syndrome, nerve roots. Syndrome and plexus disease, in vasculitic neuropathy, often common in the common peroneal nerve, radial nerve, ulnar nerve and median nerve, vasculitis is mainly due to vascular obstruction caused by ischemic damage and nerve damage, resulting Demyelination and axonal degeneration, EMG shows typical features of peripheral nerve damage, slower conduction velocity of motor and sensory nerves, reduced amplitude of multifocal action potentials, common fibrillation and positive sharp waves.
2. Muscle system involvement
Angioinflammatory myopathy is also a clinical manifestation of primary vasculitis, which can manifest severe muscle weakness, muscle pain, swelling and activity difficulties. The proximal muscle weakness of the limb is obvious, and there may be muscle tenderness, often accompanied by increased muscle enzymes. EMG examination suggests myogenic damage, and muscle biopsy can be confirmed to be an inflammatory myopathy.
3. Central nervous system involvement
The exact incidence of primary vasculitis involving the central nervous system (CNS) is not clear, but it often occurs in the late stage of vasculitis. It is also a common symptom of primary vasculitis. Two manifestations of vasculitic central nervous system diseases are common. Form: Diffuse encephalopathy and focal or multifocal dysfunction of the brain or spinal cord due to stroke, which may be hemorrhagic stroke (subarachnoid hemorrhage or cerebral hemorrhage) or ischemic stroke, CNS is often manifested For headache, seizures, hemiplegia, aphasia, hemianopia, neck stiffness, ataxia, chorea, tremor paralysis, mental disorders, apathy, drowsiness, dementia and disturbance of consciousness.
CSF examination is often helpful for diagnosis. The number of CSF cells is increased, the lymphocyte is mainly increased, the protein content is increased, and the blood CSF is seen occasionally. However, the CSF examination can also be normal. The brain CT can show the clinical evidence of brain damage or sub- Clinical lesions, more than 75% of patients reported to have abnormal CT findings, MRI examination is more sensitive to vascular inflammatory lesions, in short, cerebral angiography and combined with meningeal and brain biopsy can determine central nervous system damage caused by primary vasculitis .
4. Cranial nerve involvement
Vascular inflammatory neuropathy with single or multiple cranial nerve damage is not common, but it can be an early symptom of primary vasculitis. Common II-VIII cranial nerve involvement is manifested as loss of vision, diplopia, facial numbness, Bell's paralysis, sudden paralysis, dizziness and tinnitus.
Examine
Examination of neurological damage caused by systemic vasculitis
1. Routine examination should include erythrocyte sedimentation rate, C-reactive protein, hematuria routine, eosinophil count, liver and kidney function, muscle enzyme, hepatitis B, cold globulin, antinuclear antibody, serum complement, rheumatoid factor and blood biochemical examination Etc., secondary vasculitis exists in the primary laboratory corresponding clinical examination results.
2. Serological tests for syphilis, Lyme disease, herpes and HIV.
3. CSF examination of suspected CNS vasculitis should be performed by CSF examination to exclude other diseases, especially infections, which often contribute to the diagnosis of central nervous system involvement. The number of CSF cells is increased, with lymphocyte increase and protein increase. Even bloody CSF can be seen, but the CSF test can also be normal.
4. Serum anti-neutrophil cytoplasmic antibody (ANCA) titer determination is helpful for the diagnosis of primary vasculitis, and can be used as a monitoring indicator of recurrence.
Auxiliary inspection
1. X-ray pictures of the heart and chest, with differential diagnosis.
2. EEG, CT, MRI examination often shows abnormalities, but this is not unique to CNS vasculitis. Brain CT can show clinical evidence or subclinical lesions of brain damage. It is reported that more than 75% of patients have abnormal CT findings, MRI examination More sensitive to vascular inflammatory lesions.
3. Cerebral angiography can often determine the central damage caused by primary vasculitis.
4. EMG can show the characteristics of peripheral nerve damage or suggest myogenic damage.
5. Suspected peripheral nerve involvement or muscle involvement, may be considered for peripheral nerve / muscle biopsy, central nervous system damage diagnosis, biopsy of pia mater / brain tissue should be performed.
Diagnosis
Diagnosis and differentiation of nerve damage caused by systemic vasculitis
diagnosis
Patients with muscle weakness, peripheral neuropathy, cranial nerve spasm, headache, with or without stroke, and severe systemic non-specific clinical signs and symptoms (fever, weight loss, joint pain, etc.), doctors should be alert to primary blood vessels The possibility of inflammation, timely and accurate diagnosis of primary vasculitis is very important for the treatment of patients, careful medical history and detailed physical examination can provide clues to help diagnose.
ESR, C-reactive protein, routine hematuria, eosinophil count, liver and kidney function, muscle enzyme, hepatitis B virus antigen and antibody, HIV serology, cryoglobulin, serum complement, rheumatoid factor, antinuclear antibody and blood biochemistry Check, etc., to identify the cause of the disease, should also check the heart and chest X-rays, in recent years, found that the anti-neutrophil cytoplasmic antibody (ANCA) titer in serum by immunofluorescence technology, help some of the primary Diagnosis of vasculitis and as a monitoring indicator of disease recurrence.
If clinically suspected vasculitis of the CNS, CSF should be performed to rule out other diseases, especially infections, and serological tests for syphilis, Lyme disease, and herpes should be performed.
EEG, CT, MRI often show abnormalities, but this is not unique to CNS vasculitis. Cerebral angiography is necessary for diagnosis. Diagnosis of pia mater/brain tissue should be performed. Patients with systemic disease with ESR increase or When ANCA is positive, the invasive examination of the associated organ should be considered. Suspected peripheral nerve involvement or muscle involvement. In the case of abnormal electrophysiological examination, peripheral nerve/muscle biopsy may be considered.
Differential diagnosis
Vascular inflammatory neurological damage should be differentiated from primary peripheral neuropathy, primary central nervous system disease, and primary myopathy. Most of these diseases are not affected by other organs, and should be secondary to other diseases (such as diabetes). , sarcoidosis, poisoning, etc.) differentiated peripheral neuropathy.
