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RGD DISEASE ONTOLOGY - ANNOTATIONS

RGD uses the Human Disease Ontology (DO, https://disease-ontology.org/) for disease curation across species. RGD automatically downloads each new release of the ontology on a monthly basis. Some additional terms which are required for RGD's curation purposes but are not currently covered in the official version of DO have been added. As corresponding terms are added to DO, these custom terms are retired and the DO terms substituted in existing annotations and subsequently used for curation.

Term:subacute sclerosing panencephalitis
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Accession:DOID:8970 term browser browse the term
Definition:A rare, slowly progressive encephalitis caused by chronic infection with the MEASLES VIRUS. The condition occurs primarily in children and young adults, approximately 2-8 years after the initial infection. A gradual decline in intellectual abilities and behavioral alterations are followed by progressive MYOCLONUS; MUSCLE SPASTICITY; SEIZURES; DEMENTIA; autonomic dysfunction; and ATAXIA. DEATH usually occurs 1-3 years after disease onset. Pathologic features include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. It is caused by the SSPE virus, which is a defective variant of MEASLES VIRUS. (From Adams et al., Principles of Neurology, 6th ed, pp767-8)
Synonyms:exact_synonym: SSPE;   Subacute Sclerosing Leukoencephalitis;   Van Bogaert leukoencephalitis;   Van Bogaert's leukoencephalitis;   Van Bogaert's sclerosing leukoencephalitis;   Van Bogaerts leukoencephalitis;   immunosuppressive measles encephalitis;   inclusion body encephalitis, measles;   subacute sclerosing leukoencephalitides;   subacute sclerosing leukoencephalopathy;   subacute sclerosing panencephalitides
 primary_id: MESH:D013344
 alt_id: OMIM:260470
 xref: GARD:7708;   ICD10CM:A81.1;   ICD9CM:046.2;   NCI:C85171
For additional species annotation, visit the Alliance of Genome Resources.


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Nasu-Hakola disease term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Trem2 triggering receptor expressed on myeloid cells 2 ISO ClinVar Annotator: match by term: Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy ClinVar PMID:12080485, PMID:12754369, PMID:12925681, PMID:15883308, PMID:18546367, PMID:21834902, PMID:23399524, PMID:25615530, PMID:25741868, PMID:27995897, PMID:28492532, PMID:28768830, PMID:29142083 NCBI chrNW_004955437:7,780,886...7,785,597
Ensembl chrNW_004955437:7,780,679...7,785,697
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G Tyrobp transmembrane immune signaling adaptor TYROBP ISO OMIM NCBI chrNW_004955468:2,673,657...2,677,633
Ensembl chrNW_004955468:2,673,980...2,677,321
JBrowse link

Term paths to the root
Path 1
Term Annotations click to browse term
  disease 11834
    disease by infectious agent 1549
      viral infectious disease 1291
        subacute sclerosing panencephalitis 2
          Nasu-Hakola disease 2
Path 2
Term Annotations click to browse term
  disease 11834
    disease by infectious agent 1549
      viral infectious disease 1291
        RNA Virus Infections 1162
          Mononegavirales Infections 76
            Paramyxoviridae Infections 58
              Morbillivirus Infections 5
                measles 5
                  subacute sclerosing panencephalitis 2
                    Nasu-Hakola disease 2
paths to the root

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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.