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DeCS
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Descriptor English:
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Pantothenate Kinase-Associated Neurodegeneration
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Descriptor Spanish:
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Neurodegeneración Asociada a Pantotenato Quinasa
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Descriptor Portuguese:
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Neurodegeneração Associada a Pantotenato-Quinase
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Synonyms English:
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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:
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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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Definition English:
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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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History Note English:
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2008 (1965)
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Allowable Qualifiers English:
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Record Number:
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6364
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Unique Identifier:
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D006211
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Occurrence in VHL:
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Similar:
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DeCS
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