| Descriptor English: | Pantothenate Kinase-Associated Neurodegeneration | ||||||
| Descriptor Spanish: |
Neurodegeneración Asociada a Pantotenato Quinasa
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| Descriptor Portuguese: | Neurodegeneração Associada a Pantotenato-Quinase | ||||||
| Descriptor French: | Neurodégénérescence associée à la pantothénate kinase | ||||||
| Entry term(s): |
Degeneration, Pigmentary Pallidal Dystrophies, Juvenile-Onset Neuroaxonal Dystrophy, Juvenile-Onset Neuroaxonal Hallervorden Spatz Disease Hallervorden Spatz Syndrome Hallervorden-Spatz Disease Hallervorden-Spatz Syndrome Juvenile-Onset Neuroaxonal Dystrophies Juvenile-Onset Neuroaxonal Dystrophy Neuroaxonal Dystrophies, Juvenile-Onset Neuroaxonal Dystrophy, Juvenile Onset Neuroaxonal Dystrophy, Juvenile-Onset Neurodegeneration With Brain Iron Accumulation 1 Neurodegeneration with Brain Iron Accumulation Type 1 Neurodegeneration, Pantothenate Kinase-Associated PKAN Neuroaxonal Dystrophy, Juvenile Onset PKAN Neuroaxonal Dystrophy, Juvenile-Onset Pallidal Atrophy, Pigmentary Pantothenate Kinase Associated Neurodegeneration Pigmentary Pallidal Atrophy Pigmentary Pallidal Degeneration |
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| 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 |
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| RDF Unique Identifier: | https://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) |
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| Allowable Qualifiers: |
BL blood CF cerebrospinal fluid CI chemically induced CL classification CN congenital CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PA pathology PC prevention & control PP physiopathology PS parasitology PX psychology RH rehabilitation RT radiotherapy SU surgery TH therapy UR urine VE veterinary VI virology |
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| Public MeSH Note: | 2008; see HALLERVORDEN-SPATZ SYNDROME 1991-2007, see BASAL GANGLIA DISEASES 1979-1990, see BRAIN DISEASES 1975-1978 |
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| History Note: | 2008 (1965) |
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| DeCS ID: | 6364 | ||||||
| Unique ID: | D006211 | ||||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||||
| Date Established: | 1991/01/01 | ||||||
| Date of Entry: | 1999/01/01 | ||||||
| Revision Date: | 2013/07/08 |
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Pantothenate Kinase-Associated Neurodegeneration
- Preferred
| Concept UI |
M0009761 |
| 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) |
| Preferred term | Pantothenate Kinase-Associated Neurodegeneration |
| Entry term(s) |
Degeneration, Pigmentary Pallidal Dystrophies, Juvenile-Onset Neuroaxonal Dystrophy, Juvenile-Onset Neuroaxonal Hallervorden Spatz Disease Hallervorden Spatz Syndrome Hallervorden-Spatz Disease Hallervorden-Spatz Syndrome Juvenile-Onset Neuroaxonal Dystrophies Juvenile-Onset Neuroaxonal Dystrophy Neuroaxonal Dystrophies, Juvenile-Onset Neuroaxonal Dystrophy, Juvenile Onset Neuroaxonal Dystrophy, Juvenile-Onset Neurodegeneration With Brain Iron Accumulation 1 Neurodegeneration with Brain Iron Accumulation Type 1 Neurodegeneration, Pantothenate Kinase-Associated PKAN Neuroaxonal Dystrophy, Juvenile Onset PKAN Neuroaxonal Dystrophy, Juvenile-Onset Pallidal Atrophy, Pigmentary Pantothenate Kinase Associated Neurodegeneration Pigmentary Pallidal Atrophy Pigmentary Pallidal Degeneration |
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