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Pantothenate Kinase-Associated Neurodegeneration MeSH Descriptor Data 2021


MeSH Heading
Pantothenate Kinase-Associated Neurodegeneration
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
Unique ID
D006211
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D006211
Scope Note
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 Term(s)
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
Pigmentary Pallidal Degeneration
Public MeSH Note
2008; see HALLERVORDEN-SPATZ SYNDROME 1991-2007, see BASAL GANGLIA DISEASES 1979-1990, see BRAIN DISEASES 1975-1978
History Note
2008 (1965)
Date Established
1991/01/01
Date of Entry
1999/01/01
Revision Date
2013/07/08
Pantothenate Kinase-Associated Neurodegeneration Preferred
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