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 Syndrome
- Hallervorden Spatz Syndrome
- Neurodegeneration With Brain Iron Accumulation 1
- Neurodegeneration with Brain Iron Accumulation Type 1
- 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
- Hallervorden-Spatz Disease
- Hallervorden Spatz Disease
- Pigmentary Pallidal Atrophy
- Pallidal Atrophy, Pigmentary