Pantothenate Kinase-Associated Neurodegeneration
"Pantothenate Kinase-Associated Neurodegeneration" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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)
| Descriptor ID |
D006211
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| MeSH Number(s) |
C10.228.140.079.800 C10.228.140.744.320 C10.228.662.575 C10.574.500.700 C16.320.400.650
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| Concept/Terms |
Pantothenate Kinase-Associated Neurodegeneration- Pantothenate Kinase-Associated Neurodegeneration
- 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
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Below are MeSH descriptors whose meaning is more general than "Pantothenate Kinase-Associated Neurodegeneration".
Below are MeSH descriptors whose meaning is more specific than "Pantothenate Kinase-Associated Neurodegeneration".
This graph shows the total number of publications written about "Pantothenate Kinase-Associated Neurodegeneration" by people in this website by year, and whether "Pantothenate Kinase-Associated Neurodegeneration" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 2004 | 1 | 0 | 1 |
| 2019 | 1 | 0 | 1 |
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Below are the most recent publications written about "Pantothenate Kinase-Associated Neurodegeneration" by people in Profiles.
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Incidence of PKAN determined by bioinformatic and population-based analysis of ~140,000 humans. Mol Genet Metab. 2019 12; 128(4):463-469.
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Susceptibility-weighted imaging in pantothenate kinase-associated neurodegeneration. J Pediatr. 2014 Jan; 164(1):212.
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Deep brain stimulation as a mode of treatment of early onset pantothenate kinase-associated neurodegeneration. Eur J Paediatr Neurol. 2009 Jan; 13(1):61-4.
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Clinical heterogeneity of neurodegeneration with brain iron accumulation (Hallervorden-Spatz syndrome) and pantothenate kinase-associated neurodegeneration. Mov Disord. 2004 Jan; 19(1):36-42.
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The eye-of-the-tiger sign. Radiology. 2000 Dec; 217(3):895-6.