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SNOMED CT
Last uploaded:
January 16, 2025
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| Id | http://purl.bioontology.org/ontology/SNOMEDCT/1208478005
http://purl.bioontology.org/ontology/SNOMEDCT/1208478005
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|---|---|
| Preferred Name | Familial infantile bilateral striatal necrosis |
| Definitions |
The familial form of infantile bilateral striatal necrosis, a syndrome of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus with characteristics of developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. Prognosis is usually poor with patients progressing to spastic quadriparesis followed by death, usually due to infection.
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| Synonyms |
Familial IBSN (infantile bilateral striatal necrosis)
Familial infantile bilateral striatal necrosis (disorder)
Familial infantile striatonigral degeneration
Familial infantile striatonigral necrosis
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| Type | http://www.w3.org/2002/07/owl#Class |
All Properties
| definition | The familial form of infantile bilateral striatal necrosis, a syndrome of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus with characteristics of developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. Prognosis is usually poor with patients progressing to spastic quadriparesis followed by death, usually due to infection. |
|---|---|
| altLabel |
Familial IBSN (infantile bilateral striatal necrosis)
Familial infantile bilateral striatal necrosis (disorder)
Familial infantile striatonigral degeneration
Familial infantile striatonigral necrosis
|
| prefLabel | Familial infantile bilateral striatal necrosis
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| Type ID |
900000000000003001
900000000000013009
|
| CASE SIGNIFICANCE ID |
900000000000020002
900000000000448009
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| notation | 1208478005
|
| Effective time | 20220331
|
| Active | 1
|
| Has finding site | |
| Has clinical course | |
| interprets | |
| subClassOf | |
| Semantic type UMLS property | |
| Has interpretation | |
| type | |
| Subset member | 447562003~MAPRULE~TRUE
447562003~MAPTARGET~G23.2
6011000124106~MAPGROUP~1
900000000000497000~MAPTARGET~XVHtq
900000000000508004~ACCEPTABILITYID~900000000000549004
447562003~MAPGROUP~1
447562003~CORRELATIONID~447561005
6011000124106~MAPCATEGORYID~447637006
900000000000509007~ACCEPTABILITYID~900000000000548007
900000000000508004~ACCEPTABILITYID~900000000000548007
447562003~MAPPRIORITY~1
447562003~MAPCATEGORYID~447637006
6011000124106~MAPADVICE~ALWAYS G23.2
6011000124106~MAPTARGET~G23.2
6011000124106~MAPPRIORITY~1
6011000124106~CORRELATIONID~447561005
447562003~MAPADVICE~ALWAYS G23.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
6011000124106~MAPRULE~TRUE
900000000000509007~ACCEPTABILITYID~900000000000549004
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| DEFINITION STATUS ID | 900000000000074008
|
| tui | T047
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| CTV3ID | XVHtq
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| Occurs in | |
| cui | C4087174
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| Has associated morphology |
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