Familial infantile bilateral striatal necrosis (Q107470)
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Familial infantile bilateral striatal necrosis is the familial form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus and is characterised by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis.
| Language | Label | Description | Also known as |
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| default for all languages | ID_1873983370 |
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| English | Familial infantile bilateral striatal necrosis |
Familial infantile bilateral striatal necrosis is the familial form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus and is characterised by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. |
