- 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)
- Terms
-
Pantothenate Kinase-Associated Neurodegeneration
Preferred Term
Term UI
T694229
Date03/22/2007
LexicalTag
NON
ThesaurusID
-
Hallervorden-Spatz Disease
Term UI
T018849
Date07/17/1995
LexicalTag
EPO
ThesaurusID
-
Hallervorden-Spatz Syndrome
Term UI
T018848
Date01/01/1999
LexicalTag
EPO
ThesaurusID
NLM (1965)
-
Neurodegeneration With Brain Iron Accumulation 1
Term UI
T811600
Date11/15/2011
LexicalTag
NON
ThesaurusID
OMIM (2013)
-
Pigmentary Pallidal Atrophy
Term UI
T364139
Date11/03/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
Pigmentary Pallidal Degeneration
Term UI
T364140
Date11/03/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
PKAN Neuroaxonal Dystrophy, Juvenile-Onset
Term UI
T825110
Date06/27/2012
LexicalTag
ACX
ThesaurusID
OMIM (2013)
-
Neuroaxonal Dystrophy, Juvenile-Onset
Term UI
T842243
Date04/18/2013
LexicalTag
NON
ThesaurusID
GHR (2014)
-
Neurodegeneration with Brain Iron Accumulation Type 1
Term UI
T842244
Date04/18/2013
LexicalTag
NON
ThesaurusID
GHR (2014)