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)
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
Concept/Terms
Pantothenate Kinase-Associated Neurodegeneration- Pantothenate Kinase-Associated Neurodegeneration
- Neurodegeneration, Pantothenate Kinase-Associated
- Pantothenate Kinase Associated Neurodegeneration
- Pigmentary Pallidal Atrophy
- Pallidal Atrophy, Pigmentary
- Hallervorden-Spatz Syndrome
- Hallervorden Spatz Syndrome
- Pigmentary Pallidal Degeneration
- Degeneration, Pigmentary Pallidal
- Hallervorden-Spatz Disease
- Hallervorden Spatz Disease
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 Harvard Catalyst Profiles by year, and whether "Pantothenate Kinase-Associated Neurodegeneration" was a major or minor topic of these publication.
To see the data from this visualization as text,
click here.
| Year | Major Topic | Minor Topic | Total |
|---|
| 1999 | 1 | 0 | 1 |
| 2001 | 1 | 0 | 1 |
| 2003 | 1 | 0 | 1 |
| 2011 | 1 | 0 | 1 |
| 2019 | 2 | 0 | 2 |
| 2020 | 1 | 0 | 1 |
| 2021 | 1 | 0 | 1 |
Below are the most recent publications written about "Pantothenate Kinase-Associated Neurodegeneration" by people in Profiles.
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Rational Design of Novel Therapies for Pantothenate Kinase-Associated Neurodegeneration. Mov Disord. 2021 09; 36(9):2005-2016.
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Fosmetpantotenate Randomized Controlled Trial in Pantothenate Kinase-Associated Neurodegeneration. Mov Disord. 2021 06; 36(6):1342-1352.
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4'-Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN. EMBO Mol Med. 2019 12; 11(12):e10489.
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Diagnostic and clinical experience of patients with pantothenate kinase-associated neurodegeneration. Orphanet J Rare Dis. 2019 07 12; 14(1):174.
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The FOsmetpantotenate Replacement Therapy (FORT) randomized, double-blind, Placebo-controlled pivotal trial: Study design and development methodology of a novel primary efficacy outcome in patients with pantothenate kinase-associated neurodegeneration. Clin Trials. 2019 08; 16(4):410-418.
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Pallidal neuronal apolipoprotein E in pantothenate kinase-associated neurodegeneration recapitulates ischemic injury to the globus pallidus. Mol Genet Metab. 2015 Dec; 116(4):289-97.
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Idiopathic basal ganglia calcifications: an atypical presentation of PKAN. Pediatr Neurol. 2013 Nov; 49(5):351-4.
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Metabolic consequences of mitochondrial coenzyme A deficiency in patients with PANK2 mutations. Mol Genet Metab. 2012 Mar; 105(3):463-71.
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Young-onset parkinsonism in a Hong Kong Chinese man with adult-onset Hallervorden-Spatz syndrome. Int J Neurosci. 2011 Apr; 121(4):224-7.
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Adult Hallervorden-Spatz syndrome simulating amyotrophic lateral sclerosis. Muscle Nerve. 2003 Jul; 28(1):118-22.