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Leukoencephalopathy, Progressive Multifocal (CUI C0023524) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0023524

NCI Thesaurus Code: C26815  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: A progressive demyelination within the central nervous system associated with reactivation of a latent JC virus infection.

GARD Definition: Progressive multifocal leukoencephalopathy (PML) is a neurological disorder that damages the myelin that covers and protects nerves in the white matter of the brain. It is caused by the JC virus (JCV). By age 10, most people have been infected with this virus, but it rarely causes symptoms unless the immune system becomes severely weakened.  The disease occurs, rarely, in organ transplant patients; people undergoing chronic corticosteroid or immunosuppressive therapy; and individuals with cancer, such as Hodgkin's disease, lymphoma, and sarcoidosis.  PML is most common among individuals with acquired immune deficiency syndrome (AIDS). - this information is from GARD/ORDR/NCATS.

NICHD Definition: A demyelinating central nervous system disease caused by reactivation of the polyomavirus JC, which results in a lytic infection of oligodendrocytes.

CSP Definition: demyelination in various part of the central nervous system, usually secondary to neoplastic disease; JC virus has been recovered from brain tissue.

MSH Definition: An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7)

Synonyms & Abbreviations: (see Synonym Details)
ENCEPH JC POLYOMAVIRUS
Encephalitis, JC Polyomavirus
Encephalopathies, JC Polyomavirus
Encephalopathy, JC Polyomavirus
JC Polyomavirus Encephalitis
JC Polyomavirus Encephalopathy
JC POLYOMAVIRUS ENCEPH
LEUKOENCEPH PROGRESSIVE MULTIFOCAL
Leukoencephalopathies, Progressive Multifocal
Leukoencephalopathy, Progressive Multifocal [Disease/Finding]
LEUKOENCEPHALOPATHY, PROGRESSIVE MULTIFOCAL
Multifocal leucoencephalopathy
Multifocal Leukoencephalopathies, Progressive
Multifocal leukoencephalopathy NOS
Multifocal Leukoencephalopathy, Progressive
Multifocal leukoencephalopathy
PML - Progressive multifocal leucoencephalopathy
PML - Progressive multifocal leukoencephalopathy
PMLE - Progressive multifocal leucoencephalopathy
PMLE - Progressive multifocal leukoencephalopathy
PML
Prog multifoc leukoencep
Progressive multifocal leucoencephalopathy
Progressive Multifocal Leukoencephalopathies
Progressive multifocal leukoencephalopathy (disorder)
Progressive Multifocal Leukoencephalopathy
PROGRESSIVE MULTIFOCAL LEUKOENCEPH

External Source Codes: 
NCI Thesaurus Code C26815 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH TM UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000020002 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
Contributing_Source MedDRA NCI
Contributing_Source NICHD NCI
CTV3ID A413. SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000073002 SNOMEDCT_US
DID 2042-2309 CSP
DISEASE_IDENTIFIER_ID 5491 GARD
DISEASE_IDENTIFIER_ID 5492 GARD
DX 19730101 MSH
EFFECTIVE_TIME 20020731 SNOMEDCT_US
HAS_GARD_PAGE true GARD
HN 1973(1971) MSH
IAN DEFAULT ICD10
ICE Multifocal leukoencephalopathy NOS ICD9CM
IDENTIFIER_TYPE_ID 1 GARD
INCLUSION_TERM Multifocal leukoencephalopathy NOS ICD10
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_DATE 2009-01-23T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7) NDFRT
MESH_DUI D007968 NDFRT
MESH_NAME Leukoencephalopathy, Progressive Multifocal NDFRT
MESH_UI M0012419 NDFRT
MMR 20150702 MSH
MN C02.182.525.500 MSH
MN C02.256.721.500 MSH
MN C02.290.575 MSH
MN C02.839.550 MSH
MN C10.228.140.430.520.750.500 MSH
MN C10.228.140.695.750 MSH
MN C10.228.228.245.340.500 MSH
MN C10.228.228.399.750.500 MSH
MN C10.314.450 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
NDFRT_KIND DISEASE_KIND NDFRT
NICHD_Hierarchy_Term Progressive Multifocal Leukoencephalopathy NCI
NUI N0000001836 NDFRT
ORDER_NO 00607 ICD10CM
PM 1973 MSH
PRIMARY_PATH 10036807$10057200$10047438$10021881$Progressive multifocal leukoencephalopathy$Polyomavirus infections$Viral infectious disorders$Infections and infestations MDR
PRIMARY_SOC 10021881 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/7468/progressive-multifocal-leukoencephalopathy/resources/1 GARD
RXAUI 3153712 RXNORM
RXAUI 3183444 RXNORM
RXAUI 3189246 RXNORM
RXAUI 3189247 RXNORM
RXAUI 3223941 RXNORM
RXCUI 1024146 RXNORM
SMQ_TERM_ADDVERSION 11.1 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 11.1 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 22255007 NDFRT
SNOMEDID DE-32A51 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS A81.2 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~A81.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS A81.2 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY ASSOCIATED WITH ACQUIRED IMMUNODEFICIENCY SYNDROME CHOOSE A81.2 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY ASSOCIATED WITH ACQUIRED IMMUNODEFICIENCY SYNDROME CHOOSE B20 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY CO-OCCURRENT WITH HUMAN IMMUNODEFICIENCY VIRUS INFECTION CHOOSE B20 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~MAP SOURCE CONCEPT CANNOT BE CLASSIFIED WITH AVAILABLE DATA SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447638001 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447639009 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~3 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 420554003 | Progressive multifocal leukoencephalopathy associated with acquired immunodeficiency syndrome (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 713487008 | Progressive multifocal leukoencephalopathy co-occurrent with human immunodeficiency virus infection (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~OTHERWISE TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~A81.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~B20 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~ SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~A413. SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DE-32A51 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T023751 MSH
TERMUI T368989 MSH
TERMUI T368990 MSH
TERMUI T368991 MSH
TH NLM (1971) MSH
TH NLM (2000) MSH
TH ORD (2010) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0023524

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