Overview.
Isolated central nervous system vasculitis, formerly known as primary central nervous system vasculitis (PACNS), was first reported in 1959 and changed to its current name, granulomatous vasculitis, in 1983. The name PACNS has been commonly adopted in recent years. It is an inflammatory disease of the blood vessels confined to the central nervous system, i.e., the brain and spinal cord. The incidence of the disease is low, accounting for only 1% of systemic vasculitides. the cause of PACNS is unknown and clinical diagnosis is difficult.
Etiology
The cause of PACNS remains unclear.
Symptoms
PACNS can develop at any age, and is more common in young and middle-aged people, with a median age of onset of about 50 years old and a male-to-female ratio of 2:1, and rarely develops in children. The disease has an acute or insidious onset, and tends to have a chronic course, alternating between remission and relapse. As a result of diffuse cortical impairment, patients commonly experience headache and a wide range of encephalopathic symptoms, including chronic progressive headache, insidious cognitive decline, stroke or persistent neurologic deficits, transient ischemic attacks, aphasia, visual disturbances, and seizures. In the absence of headache and encephalopathy, stroke or focal symptoms occur in less than 20%, systemic symptoms (e.g., fever, rash, joint pain, weight loss) in less than 20%, and increased erythrocyte sedimentation rate in less than 25%. Subarachnoid hemorrhage, chronic meningoencephalitis or transverse myelitis, and acute impaired consciousness or coma are rare.
Examination
1. MRI examination
PACNS mainly involves small blood vessels, and the morphology of the lesion does not show a typical wedge shape, nor does it conform to the typical distribution of a certain blood vessel. Conventional MRI has high sensitivity to the lesions, but lacks specificity.
2. Cerebrospinal fluid (CSF) examination
CSF examination accounts for 80% to 90% of patients with pathologically confirmed abnormalities. The common manifestations are slightly elevated protein levels and increased lymphocyte counts.
3. Angiography
The diagnosis of PACNS can be assisted by angiography, which may show staged stenosis, dilatation and obstruction, as well as changes in the circulation time of the affected vascular areas, and may also show arterial bead-like changes and aneurysm formation.
4. Brain biopsy and pathologic examination
Due to the focal and staged distribution of the lesions, brain biopsy has a positive rate of only 50% to 75%. Brain biopsy should be performed when there are focal signs, elevated CSF protein levels and increased lymphocyte counts, and suspicious signs of vasculitis on cranial MRA or angiography.
Diagnosis
Diagnosis can be made based on the symptoms of headache, dementia, hemiparesis, hemianopsia, convulsions, etc., combined with the results of cerebrospinal fluid routine, magnetic resonance imaging, angiography and pathological histology examination, and after excluding systemic vasculitis, infection, tumors, or other vasculopathies.
1. The patient presents with headache, dementia, hemiplegia, mild paraplegia of the lower limbs, quadriplegia, hemiplegia, hemianopsia, hemianopsia, convulsions, cranial nerve damage, psychiatric symptoms, and personality changes.
2. Magnetic resonance imaging shows low-density ischemic foci or infarct foci of varying sizes.
3. Cerebral angiography shows typical manifestations of multifocal damage to blood vessels.
4. Histopathologic examination shows obvious inflammation in the vessel wall with infiltration of lymphocytes, monocytes, histiocytes and plasma cells.
5. Exclude systemic vasculitis, infection, tumor, other vascular diseases.
Treatment
The recommended standard treatment regimen is a combination of glucocorticoids and cyclophosphamide (CYC). The most common treatment regimen is intravenous methylprednisolone followed by oral prednisone and CYC, or intravenous CYC, which may be combined with antibiotics, aspirin, and other drugs.
Prognosis
The prognosis for patients with this disease is poor. If not actively treated, the disease will progressively worsen and eventually die.
Dietary management
1. Eat a balanced diet, eat more fruits, vegetables and other high-fiber foods, eat more eggs, soybeans and other high-protein foods, pay attention to a light diet, you can do a moderate amount of exercise.
2. Avoid smoking, alcohol, spicy, coffee and other stimulating foods.