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Concept information

Preferred term

Pantothenate Kinase-Associated Neurodegeneration  

Type

  • mesh:Descriptor

Definition

  • 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)

Entry terms

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

In other languages

  • French

  • Dégénérescence pallidale pigmentaire
  • Dystrophie neuro-axonale tardive
  • Dystrophie neuroaxonale tardive
  • Maladie de Hallervorden-Spatz
  • Neurodégénerescence avec accumulation de fer dans le cerveau
  • Neuroferritinopathie
  • Syndrome de Hallervorden-Spatz

URI

http://data.loterre.fr/ark:/67375/JVR-VNH65KKH-P

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