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Pantothenate Kinase-Associated Neurodegeneration

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 and Robertson, Textbook of Neuropathology, 2nd ed, pp972-929)

Year introduced: 2008 (1965)

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Subheadings:

Tree Number(s): C10.228.140.079.800, C10.228.140.744.320, C10.228.662.575, C10.574.500.700, C16.320.400.650

MeSH Unique ID: D006211

Entry Terms:

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

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