Medical Definition of Hallervorden-Spatz syndrome
1.
A progressive hereditary disorder with marked reduction in the number of myelin sheaths of the globus pallidus and substantia nigra with accumulation of iron pigment.
Manifestations include dysarthria, progressive mental deterioration, and corticospinal and extrapyramidal motor signs.
CT findings: high-signal lesions in globus pallidus bilaterally.
MRI findings: (T2-weighted spin-echo), hypointensity in globus pallidus (iron accumulation), small area of hyperintensity in internal segment (so-called eye of the tiger sign) corresponding to area of gliosis and vacuolation.
Inheritance: autosomal recessive.
(20 Mar 2000)
Lexicographical Neighbors of Hallervorden-Spatz Syndrome
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