infantile bilateral striatal necrosis
Several syndromes of bilateral symmetric spongy degeneration of the caudate nucleaus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. IBSN can be familial or sporadic.
MONDO:0015983
obsolete rare genetic syndromic intellectual disability (obsolete MONDO:0015983)
excluded_subClassOf
MONDO:0017641
obsolete miscellaneous movement disorder due to neurodegenerative disease (obsolete MONDO:0017641)
excluded_subClassOf
MONDO:0017662
obsolete miscellaneous movement disorder due to genetic neurodegenerative disease (obsolete MONDO:0017662)
excluded_subClassOf
MONDO:0005559
neurodegenerative disease
is_a
IBSN
exact
SNDI
related
infantile bilateral striatal necrosis
exact
infantile striatonigral degeneration
exact
infantile striatonigral necrosis
exact
striatal degeneration familial
related
striatonigral degeneration infantile
related
SPAC26A3.15c SPMIT.07