Structured Summary
Abstract
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)
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Synonyms
21 entry terms
- 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
- 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
MeSH Record
Aspects Covered
35 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, congenital, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
History Note
2008 (1965)
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AMA Style
References
- National Library of Medicine. Pantothenate Kinase-Associated Neurodegeneration. Medical Subject Headings (MeSH). 2026. Unique ID D006211. http://id.nlm.nih.gov/mesh/2026/D006211
- Pantothenate Kinase-Associated Neurodegeneration. In: Wikipedia. https://en.wikipedia.org/wiki/Pantothenate_kinase-associated_neurodegeneration
- Pantothenate Kinase-Associated Neurodegeneration. In: Wikidata. https://www.wikidata.org/wiki/Q1436162