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

Also Known As:

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

Nervous System Diseases: 14178
- Neurodegenerative Diseases: 42472
  - Nervous System Heredodegenerative Disorders: 63
    - Pantothenate Kinase-Associated Neurodegeneration: 301
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Basal Ganglia Diseases: 346
      - Pantothenate Kinase-Associated Neurodegeneration: 301
    - Neuroaxonal Dystrophies: 166
      - Pantothenate Kinase-Associated Neurodegeneration: 301
  - Movement Disorders: 4634
    - Pantothenate Kinase-Associated Neurodegeneration: 301
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Nervous System Heredodegenerative Disorders: 63
    - Pantothenate Kinase-Associated Neurodegeneration: 301

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Drug/Important Bio-Agent (IBA)