Sporadic infantile bilateral striatal necrosis

Orpha code: 225147OMIM code:

Definition

Sporadic infantile bilateral necrosis is the sporadic 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.

Disease data
Classification

Disease

Synonyms
ABSN
Sporadyczna dziecięca martwica nigrostriatialna
Sporadyczna IBSN
Sporadyczne zwyrodnienie striatonigralne
Acute bilateral striatal necrosis
Sporadic IBSN
Sporadic infantile striatonigral degeneration
Sporadic infantile striatonigral necrosis
ORPHA code
225147
OMIM code
-
ICD10 code
G23.2
ICD11 code
LD90.Y

No additional description.

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