Familial infantile bilateral striatal necrosis (Q107470): Difference between revisions
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A necrose estriatal bilateral infantil familiar é a forma familiar de necrose estriado bilateral infantil (NEBI), uma síndrome de degeneração esponjosa simétrica bilateral do núcleo caudado, putâmen e globo pálido e é caracterizada por regressão do desenvolvimento, coreoatetose e distonia que progride para quadriparesia espástica. | |||
| description / en | description / en | ||
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. | |||
Revision as of 14:27, 17 August 2026
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. |
