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	panencephalitis, subacute sclerosing

Disease ID	1348
Disease	panencephalitis, subacute sclerosing
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)
Synonym	dawson's encephalitis dawson's inclusion body encephalitis dementia, decerebrate enceph inclusion body measles encephaltis, inclusion body, measles immunosuppressive measles encephalitis inclusion body enceph measles inclusion body encephalitis, measles leukoenceph subacute sclerosing leukoenceph van bogaerts leukoencephalitides, subacute sclerosing leukoencephalitis, subacute sclerosing leukoencephalitis, van bogaert leukoencephalitis, van bogaert's leukoencephalitis, van bogaerts measles inclusion body enceph measles inclusion body encephalitis measles inclusion body encephalitis (disorder) mibe - measles inclusion body encephalitis panenceph subacute sclerosing panencephalitides, subacute sclerosing sclerosing leukoenceph subacute sclerosing leukoencephalitides, subacute sclerosing leukoencephalitis, subacute sclerosing panenceph subacute sclerosing panencephalitides, subacute sclerosing panencephalitis, subacute sspe sspe - subacute sclerosing panencephalitis subac scleros panenceph subacute inclusion body encephalitis subacute sclerosing leukoencephalitides subacute sclerosing leukoencephalitis subacute sclerosing leukoencephalopathy subacute sclerosing panenceph subacute sclerosing panencephalitides subacute sclerosing panencephalitis subacute sclerosing panencephalitis (disorder) subacute sclerosing panencephalitis (disorder) [ambiguous] subacute sclerosing panencephalitis (sspe) subacute sclerosing panencephalitis [disease/finding] van bogaert leukoencephalitis van bogaert sclerosing leukoencephalitis van bogaert's leukoencephalitis van bogaert's sclerosing leucoencephalitis van bogaert's sclerosing leukoencephalitis van bogaert's sclerosing leukoencephalitis (disorder) van bogaerts leukoenceph van bogaerts leukoencephalitis
Orphanet	ORPHANET:2806
OMIM	OMIM:260470
DOID	DOID:8970
ICD10	ICD10CM:A81.1
UMLS	C0038522
MeSH	D013344
SNOMED-CT	SNOMEDCT_US_2016_09_01:359686005;SNOMEDCT_US_2016_09_01:192685000;SNOMEDCT_US_2016_09_01:416154000
Comorbidity	UMLS \| Disease \| Sentences' Count(Total Sentences:11) C0025007 \| measles \| 4 C0038522 \| subacute sclerosing panencephalitis \| 3 C0029134 \| optic neuritis \| 2 C0027813 \| neuritis \| 1 C0038522 \| subacute sclerosing panencephalitis (sspe) \| 1 C0155320 \| cortical blindness \| 1 C0042769 \| virus infection \| 1 C0085543 \| epilepsia partialis continua \| 1 C0014544 \| epilepsy \| 1 C0004134 \| ataxia \| 1 C0001175 \| acquired immunodeficiency syndrome \| 1
Curated Gene	(Waiting for update.)
Inferring Gene	Entrez_id \| Symbol \| Resource(Total Genes:18) 3458 \| IFNG \| CIPHER 3459 \| IFNGR1 \| CIPHER 3460 \| IFNGR2 \| CIPHER 3586 \| IL10 \| CIPHER 3594 \| IL12RB1 \| CIPHER 3565 \| IL4 \| CIPHER 3566 \| IL4R \| CIPHER 4599 \| MX1 \| CIPHER 151888 \| BTLA \| CIPHER 29126 \| CD274 \| CIPHER 941 \| CD80 \| CIPHER 942 \| CD86 \| CIPHER 1493 \| CTLA4 \| CIPHER 64135 \| IFIH1 \| CIPHER 5133 \| PDCD1 \| CIPHER 80380 \| PDCD1LG2 \| CIPHER 7098 \| TLR3 \| CIPHER 8764 \| TNFRSF14 \| CIPHER
Text Mined Gene	Entrez_id \| Symbol \| Score \| Resource(Total Genes:37) 60 \| ACTB \| 1.236 \| DISEASES 103 \| ADAR \| 1.332 \| DISEASES 3267 \| AGFG1 \| 1.487 \| DISEASES 57492 \| ARID1B \| 1.876 \| DISEASES 151888 \| BTLA \| 1.815 \| DISEASES 720 \| C4A \| 1.885 \| DISEASES 959 \| CD40LG \| 3.755 \| DISEASES 51428 \| DDX41 \| 1.563 \| DISEASES 1906 \| EDN1 \| 1.16 \| DISEASES 2214 \| FCGR3A \| 1.027 \| DISEASES 2304 \| FOXE1 \| 1.368 \| DISEASES 3033 \| HADH \| 1.505 \| DISEASES 3105 \| HLA-A \| 1.396 \| DISEASES 3329 \| HSPD1 \| 1.492 \| DISEASES 3440 \| IFNA2 \| 1.261 \| DISEASES 3456 \| IFNB1 \| 2.59 \| DISEASES 3586 \| IL10 \| 1.385 \| DISEASES 3594 \| IL12RB1 \| 2.547 \| DISEASES 56243 \| KIAA1217 \| 2.049 \| DISEASES 3821 \| KLRC1 \| 1.439 \| DISEASES 3822 \| KLRC2 \| 2.051 \| DISEASES 4099 \| MAG \| 2.438 \| DISEASES 4137 \| MAPT \| 2.091 \| DISEASES 4155 \| MBP \| 3.821 \| DISEASES 4340 \| MOG \| 1.346 \| DISEASES 4478 \| MSN \| 1.146 \| DISEASES 4599 \| MX1 \| 3.207 \| DISEASES 51199 \| NIN \| 1.972 \| DISEASES 56953 \| NT5M \| 1.644 \| DISEASES 4948 \| OCA2 \| 3.857 \| DISEASES 5053 \| PAH \| 1.306 \| DISEASES 5133 \| PDCD1 \| 1.687 \| DISEASES 80380 \| PDCD1LG2 \| 1.221 \| DISEASES 5238 \| PGM3 \| 2.214 \| DISEASES 7124 \| TNF \| 1.541 \| DISEASES 8764 \| TNFRSF14 \| 1.555 \| DISEASES 402682 \| UFSP1 \| 3.52 \| DISEASES
Locus	(Waiting for update.)

Disease ID	1348
Disease	panencephalitis, subacute sclerosing
Integrated Phenotype	HPO \| Name(Total Integrated Phenotypes:1) HP:0002383 \| Encephalitis
Text Mined Phenotype	HPO \| Name \| Sentences' Count(Total Phenotypes:11) HP:0100653 \| Optic neuritis \| 2 HP:0002721 \| Immunodeficiency \| 2 HP:0001268 \| Mental deterioration \| 1 HP:0100033 \| Tic disorder \| 1 HP:0007401 \| Macular atrophy \| 1 HP:0001251 \| Ataxia \| 1 HP:0012847 \| Epilepsia partialis continua \| 1 HP:0100704 \| Cortical visual impairment \| 1 HP:0001907 \| Thromboembolic disease \| 1 HP:0003201 \| Rhabdomyolysis \| 1 HP:0000572 \| Visual loss \| 1

Disease ID	1348
Disease	panencephalitis, subacute sclerosing
Manually Symptom	(Waiting for update.)
Text Mined Symptom	UMLS \| Name \| Sentences' Count(Total Symptoms:2) C0025007 \| measles \| 2 C0014544 \| epilepsy \| 1

Manually Genotype(Total Text Mining Genotypes:0)
(Waiting for update.)

Text Mining Genotype(Total Genotypes:0)
(Waiting for update.)

All Snps(Total Genotypes:0)
(Waiting for update.)

GWASdb Annotation(Total Genotypes:0)
(Waiting for update.)

GWASdb Snp Trait(Total Genotypes:0)
(Waiting for update.)

Mapped by lexical matching(Total Items:0)
(Waiting for update.)

Mapped by homologous gene(Total Items:1)
HP ID	HP Name	MP ID	MP Name	Annotation
HP:0002383	Encephalitis	MP:0020215	impaired blood coagulation	impaired ability of the blood to clot

Disease ID	1348
Disease	panencephalitis, subacute sclerosing
Case	(Waiting for update.)

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