Pantothenate Kinase-Associated Neurodegeneration [Hallervordenův-Spatzův syndrom]
- Terms
-
Hallervorden-Spatzova nemoc
Hallervordenova-Spatzova choroba
neuroaxonální degenerace s nástupem v mládí
neurodegenerace spojená s pantothenátkinasou
-
Hallervorden-Spatz Disease
Hallervorden-Spatz Syndrome
Neuroaxonal Dystrophy, Juvenile-Onset
Neurodegeneration With Brain Iron Accumulation 1
Neurodegeneration with Brain Iron Accumulation Type 1
Pigmentary Pallidal Atrophy
Pigmentary Pallidal Degeneration
PKAN Neuroaxonal Dystrophy, Juvenile-Onset
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)
- DUI
- D006211 MeSH Browser
- CUI
- M0009761
- History note
- 2008 (1965)
- Public note
- 2008; see HALLERVORDEN-SPATZ SYNDROME 1991-2007, see BASAL GANGLIA DISEASES 1979-1990, see BRAIN DISEASES 1975-1978
Allowable subheadings
- BL
- blood 0
- CF
- cerebrospinal fluid 0
- CI
- chemically induced 0
- CL
- classification 1
- CO
- complications 1
- CN
- congenital 0
- DI
- diagnosis 7
- DG
- diagnostic imaging 2
- DH
- diet therapy 0
- DT
- drug therapy 3
- EC
- economics 0
- EM
- embryology 0
- EN
- enzymology 0
- EP
- epidemiology 1
- EH
- ethnology 0
- ET
- etiology 3
- GE
- genetics 7
- HI
- history 0
- IM
- immunology 0
- ME
- metabolism 2
- MI
- microbiology 0
- MO
- mortality 0
- NU
- nursing 0
- PS
- parasitology 0
- PA
- pathology 2
- PP
- physiopathology 3
- PC
- prevention & control 0
- PX
- psychology 0
- RT
- radiotherapy 0
- RH
- rehabilitation 0
- SU
- surgery 0
- TH
- therapy 1
- UR
- urine 0
- VE
- veterinary 0
- VI
- virology 0