What Is Progressive Multifocal Leukoencephalopathy?
Progressive multifocal leukoencephalopathy (PML) is a rare subacute demyelinating disease, and its pathogen is mostly papillary polyoma vacuole virus (JCV). It is mainly found in patients with low autoimmune function, which is caused by opportunistic infection. Before the 1980s, the disease was relatively rare, but with the epidemic of human immunodeficiency syndrome (AIDS), the number of reported cases of the disease has increased sharply. In addition, the disease is easy to see in malignant hematological diseases such as: Huo Patients with organ transplants such as chidkin disease, lymphoma, and chronic lymphocytic leukemia, and patients with autoimmune diseases.
Basic Information
- English name
- progressive multifocal leukoencephalopathy
- Visiting department
- Neurology
- Multiple groups
- Patients with defective cellular immune response
- Common symptoms
- Hemiplegia, aphasia, visual field loss, ataxia, dysarthria
Causes of progressive multifocal leukoencephalopathy
- PML always occurs in patients with defective cellular immune responses. There are inclusion bodies consisting of a large number of papilloma vacuole virus particles in glial cells in the brain tissue of patients with PML.
Clinical manifestations of progressive multifocal leukoencephalopathy
- The disease is a rare disease, with peak age between 40 and 60 years. There are very few individuals with the disease. Most of them are at the time of low immune function, such as chronic lymphocytic leukemia, lymphosarcoma, tuberculosis, malignant tumor, immunosuppressive agents for kidney transplantation or other diseases, and AIDS patients are most susceptible. Subacute onset, neurological symptoms occur within a few days to weeks, progressively worsening, with the corresponding central nervous system appearing hemiplegia, aphasia, visual field defects, cortical blindness, ataxia, dysarthria, intelligent progress Sexual decline to dementia, mental withdrawal showed a state of mental disorder, eventually coma, and complications died. Seizures may occur during the course of some patients. After proper symptomatic support and antiviral treatment, the one-year survival rate of AIDS patients with this disease has risen from less than 10% to more than 50%, but overall the disease has a poor prognosis and a high mortality rate.
Progressive multifocal leukoencephalopathy
- General blood, urine, stool, and blood biochemical tests are within the normal range. Cerebrospinal fluid is usually not specifically seen. EEG often presents non-specific diffuse or focal slow waves. A CT scan of the skull showed that most of the low-density foci in the subcortical white matter had no space effect and were not enhanced. MRI also showed most lesions with long T 1 and long T 2 signals in white matter.
Diagnosis of progressive multifocal leukoencephalopathy
- The diagnosis of progressive multifocal leukoencephalopathy depends on histopathological confirmation. For those who cannot perform brain tissue biopsy, the following points must be clearly defined for the diagnosis of progressive multifocal autoencephalopathy: typical clinical symptoms of progressive multifocal autoencephalopathy that continue to exist; CSF JC virus DNA test positive; Typical imaging findings of multifocal autonomic encephalopathy. A positive diagnosis of JC virus in blood or urine is of little value.
Progressive multifocal leukoencephalopathy treatment
- There are currently no specific antiviral drugs or treatments for JC virus. There are still many controversies about the efficacy of cidofovir and cytarabine. Mefluoxamine also has antiviral ability in vitro, and can penetrate the blood-brain barrier, which is effective in some cases. For patients with HIV infection, highly effective antiretroviral therapy is the best choice, which can stabilize the condition of 50% to 60% of patients.