Symptoms of progressive multifocal leukoencephalopathy?
summary
Progressive multifocal leukoencephalopathy (PME) is a rare subacute demyelinating disease. It is mainly seen in patients with low autoimmune function and is caused by opportunistic infection. It is easy to be seen in Hodgkin's disease, lymphoma, chronic lymphocytic leukemia and other reticuloendothelial system diseases, occasionally in other visceral cancer, sarcoidosis and patients treated with immunosuppressive drugs.
Symptoms of progressive multifocal leukoencephalopathy?
PML always occurs in patients with defective cellular immune response. The inclusion bodies composed of a large number of papillomavirus particles were found in the oligodendrocytes of PML patients. Most of the viruses isolated from brain tissue of PML patients were JC virus.
Primary infection in childhood, the virus latent in the kidney or peripheral blood mononuclear cells, under certain conditions through the bloodstream infection of the central nervous system (CNS), there are also reports in PML and non PML patients with brain tissue by PCR can detect jcv-dna, indicating the existence of CNS latent infection, but how to promote the occurrence of PML, remains to be studied.
At the beginning, there were no obvious symptoms, and the onset age was mostly 50-70 years old. It usually occurs in some serious diseases, such as cancer, leukemia, lymphoma, other chronic diseases, or patients receiving immunosuppressive therapy for a long time and AIDS patients. The incidence of PML in AIDS patients is more than 4%.
matters needing attention
Brain biopsy is the only way to make a definite diagnosis. Typical histopathological changes, inclusion bodies in oligodendrocytes, electron microscopic examination showed the presence of lactulovirus particles, and immunohistochemistry confirmed the presence of lactulovirus antigen. It has been reported that 75% of JC sequences can be detected in CSF and 60% can be detected by cross reaction with SV40.