KEGG   DISEASE: Progressive multifocal leukoencephalopathy
Entry
H00370                      Disease                                
Name
Progressive multifocal leukoencephalopathy
Description
Progressive multifocal leukoencephalopathy (PML) is a fatal neurological disorder characterized by destruction of oligodendrocytes by the JC polyomavirus that occurs in immunosuppressed individuals such as patients with AIDS. Patients with PML show white matter lesions on head computed tomography scan or magnetic resonance imaging.
Category
Viral infectious disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 08 Diseases of the nervous system
  Multiple sclerosis or other white matter disorders
   8A45  Secondary white matter disorders
    H00370  Progressive multifocal leukoencephalopathy
Genome-based classification of infectious diseases [BR:br08401]
 Viral infections
  Infections caused by dsDNA viruses
   H00370  Progressive multifocal leukoencephalopathy
Pathogen
JC polyomavirus [GN:T40072]
Comment
Combination therapy
AIDS-associated: Highly active antiretroviral therapy (HAART) + Cidofovir
Immunodepressed patient: Discontinuation of immunosuppressive agent + Cidofovir [DR:D00273]
Other DBs
ICD-11: 8A45.02
ICD-10: A81.2
MeSH: D007968
MedlinePlus: 000674
Reference
  Authors
Koralnik IJ
  Title
New insights into progressive multifocal leukoencephalopathy.
  Journal
Curr Opin Neurol 17:365-70 (2004)
DOI:10.1097/00019052-200406000-00019
Reference
PMID:7551117
  Authors
Major EO, Ault GS
  Title
Progressive multifocal leukoencephalopathy: clinical and laboratory observations on a viral induced demyelinating disease in the immunodeficient patient.
  Journal
Curr Opin Neurol 8:184-90 (1995)
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