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  • Hallervorden-Spatz syndrome
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HSD Hallervorden-Spatz disease; honestly significant difference; hydroxysteroid dehydrogenase; hypertoni...
HSS Hallermann-Streiff syndrome; Hallervorden-Spatz syndrome; Henoch-Schonlein syndrome; high-speed supe...
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HSD Hallervorden Spatz disease
HSS Hallervorden Spatz syndrome
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    Hallervorden-Spatz disease
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Hallervorden syndrome <neurology, syndrome> 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)
Hallervorden, Julius <person> German neurologist, 1882-1965.
See: Hallervorden syndrome, Hallervorden-Spatz disease, Hallervorden-Spatz syndrome.
(05 Mar 2000)
Hallervorden-Spatz syndrome <neurology, syndrome> 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)
MeSH(Medical Subject Headings) ¸ÂÃã °Ë»ö (http://www.nlm.nih.gov) °á°ú : 1 ÆäÀÌÁö: 1
  • Hallervorden-Spatz Syndrome - »õâ A rare autosomal recessive degenerative disorder which usually presents in late childhood or adolescence. Clinical manifestations include progressive MUSCLE SPASTICITY; hyperreflexia; MUSCLE RIGIDITY; DYSTONIA; DYSARTHRIA; and intellectual deterioration which progresses to severe dementia over several years. Pathologic examination reveals neuronal atrophy in the globus pallidus and iron deposition in blood vessels and perivascular spaces. (From Adams et al., Principles of Neurology, 6th ed, p972; Davis & Robertson, Textbook of Neuropathology, 2nd ed, pp972-929)
    Synonyms : Hallervorden-Spatz Disease, Pigmentary Pallidal Atrophy, Atrophies, Pigmentary Pallidal, Atrophy, Pigmentary Pallidal, Degeneration, Pigmentary Pallidal, Degenerations, Pigmentary Pallidal, Hallervorden Spatz Disease, Hallervorden Spatz Syndrome
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Hallervorden-Spatz disease A slowly progressive hereditary disorder characterized by abnormal accumulations of iron pigments in certain regions of the basal ganglia (eg, globus pallidus and substantia nigra). This autosomal recessive disorder typically becomes apparent during late childhood or adolescence. ...
Ãâó: www.dbs-stn.org/glossary1.asp
Hallervorden-Spatz d. a hereditary disorder characterized by 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. Transmitted as an autosomal recessive trait, it usually begins in the first or second decade, with death usually occurring before the thirtieth year. Called also status dysmyelinatus, and status dysmyelinisatus.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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