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PIGMENTARY PALLIDAL DEGENERATION
Descriptors Found:
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DeCS
Descriptor
English
:
Pantothenate Kinase-Associated Neurodegeneration
Descriptor
Spanish
:
Neurodegeneración Asociada a Pantotenato Quinasa
Descriptor
Portuguese
:
Neurodegeneração Associada a Pantotenato-Quinase
Synonyms
English
:
Pigmentary Pallidal Degeneration
Tree Number:
C10.228.140.079.800
C10.228.140.744.320
C10.228.662.575
C10.574.500.700
C16.320.400.650
Definition
English
:
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)
History Note
English
:
2008 (1965)
Allowable Qualifiers
English
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
CN
congenital
DI
diagnosis
DH
diet therapy
DT
drug therapy
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology
GE
genetics
HI
history
IM
immunology
ME
metabolism
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology
PP
physiopathology
PC
prevention & control
PX
psychology
RA
radiography
RI
radionuclide imaging
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
US
ultrasonography
UR
urine
VE
veterinary
VI
virology
Record Number:
6364
Unique Identifier:
D006211
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS