Familial infantile bilateral striatal necrosis
Name: |
Familial infantile bilateral striatal necrosis
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Description: |
Familial infantile bilateral striatal necrosis is the familial form of infantile bilateral striatal necrosis (IBSN; see this term), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleaus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis.
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ORPHAcode: |
225154
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Synonyms: |
Familial IBSN
Familial infantile striatonigral degeneration
Familial infantile striatonigral necrosis
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XREF(s): | |
Analyte(s): | |
Created: |
13 May 2019 - 01:02
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Changed: |
22 Jun 2023 - 16:14
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