Hallervorden-Spatz syndrome (redirected from Pantothenate kinase-associated neurodegeneration)

Hal·ler·vor·den-Spatz syndrome (hllr-vôrdn-shpätz, hälr-) or Hallervorden syndrome

n.

An inherited syndrome in which the nerve fibers connecting the striatum and globus pallidus are completely demyelinated.

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Hallervorden-Spatz syndrome

[hol′ərfôr′dən shpots]

Etymology: Julius Hallervorden, German neurologist, 1882-1965; H. Spatz, German neurologist, 1888-1969

a progressive degenerative neurologic disease of children, with symptoms of parkinsonism. It is characterized by rigidity, athetosis, and dementia. The cause is an accumulation of iron pigments in the globus pallidus and substantia nigra. Treatment is similar to that of Parkinson's disease and Huntington's chorea.

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Hallervorden-Spatz syndrome [hal´er-for″den-spatz´]

a hereditary disorder involving marked reduction in the number of myelin sheaths of the globus pallidus and substantia nigra, with accumulations of iron pigment, progressive rigidity beginning in the legs, choreoathetoid movements, dysarthria, and progressive mental deterioration.

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Hallervorden-Spatz syndrome

Hallervorden-Spatz disease Neurology An AR condition first seen in childhood or adolescence Clinical Progressive neurologic degeneration with defects in muscle tone–rigidity, choreoathetosis, torsion spasm–dystonia, parkinsonism, cerebellar ataxia, speech impairment, mental deterioration, generalized rigidity Imaging Cerebral atrophy by CT DiffDx Dementia with extrapyramidal motor defects, Wilson's disease Management None Prognosis Poor; death within 10 yrs of onset