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Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Heredodegenerative Disorders, Nervous System > Pantothenate Kinase-Associated Neurodegeneration
Nervous System Diseases > Neurodegenerative Diseases > Heredodegenerative Disorders, Nervous System > Pantothenate Kinase-Associated Neurodegeneration
Nervous System Diseases > Central Nervous System Diseases > Movement Disorders > Pantothenate Kinase-Associated Neurodegeneration
Nervous System Diseases > Central Nervous System Diseases > Brain Diseases > Basal Ganglia Diseases > Pantothenate Kinase-Associated Neurodegeneration
Nervous System Diseases > Central Nervous System Diseases > Brain Diseases > Neuroaxonal Dystrophies > Pantothenate Kinase-Associated Neurodegeneration

Preferred term

Pantothenate Kinase-Associated Neurodegeneration  

Type

  • Topical Descriptor

Broader concept

Entry terms

  • Degeneration, Pigmentary Pallidal
  • Dystrophies, Juvenile-Onset Neuroaxonal
  • Dystrophy, Juvenile-Onset Neuroaxonal
  • Hallervorden Spatz Disease
  • Hallervorden-Spatz Disease
  • Hallervorden Spatz Syndrome
  • Hallervorden-Spatz Syndrome
  • Juvenile-Onset Neuroaxonal Dystrophies
  • Juvenile-Onset Neuroaxonal Dystrophy
  • Neuroaxonal Dystrophies, Juvenile-Onset
  • Neuroaxonal Dystrophy, Juvenile Onset
  • Neuroaxonal Dystrophy, Juvenile-Onset
  • Neurodegeneration With Brain Iron Accumulation 1
  • Neurodegeneration with Brain Iron Accumulation Type 1
  • Pallidal Atrophy, Pigmentary
  • Pigmentary Pallidal Atrophy
  • Pigmentary Pallidal Degeneration
  • PKAN Neuroaxonal Dystrophy, Juvenile Onset
  • PKAN Neuroaxonal Dystrophy, Juvenile-Onset

Scope note

  • 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. (From Adams et al., Principles of Neurology, 6th ed, p972; Davis & Robertson, Textbook of Neuropathology, 2nd ed, pp972-929)

History note

  • 2008 (1965)

In other languages

  • pigmentoitunut pallidusrappeuma

    Finnish

  • degeneratio globi pallidi pigmentata
  • Hallervorden
  • Hallervorden-Spatz
  • Hallervorden-Spatzin oireyhtymä
  • Hallervorden-Spatzin syndrooma
  • Hallervorden-Spatzin tauti
  • Hallervorden-Spatzs sjukdom
  • morbus Hallervorden-Spatz
  • pantotenaattikinaasiin liittyvä neurodegenraatio
  • pigmentär pallidusdegeneration
  • Spatz
  • syndroma Hallervorden-Spatz
  • Pantotenatkinas-associerad neurodegeneration

    Swedish

  • Hallervorden-Spatz syndrom

URI

http://www.yso.fi/onto/mesh/D006211

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