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OLIVOPONTOCEREBELLAR ATROPHY, IDIOPATHIC
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DeCS
Descriptor
English
:
Olivopontocerebellar Atrophies
Descriptor
Spanish
:
Atrofias Olivopontocerebelosas
Descriptor
Portuguese
:
Atrofias Olivopontocerebelares
Synonyms
English
:
Dejerine-Thomas Syndrome
Olivopontocerebellar Atrophy, Idiopathic
Tree Number:
C10.228.140.079.612.600
C10.228.140.252.700.650
C10.228.662.550.600
C10.228.854.787.750
C10.574.500.825.650
C10.574.625.600
C10.574.750
C16.320.400.780.750
Definition
English
:
A group of inherited and sporadic disorders which share progressive
ataxia
in combination with
atrophy
of the
CEREBELLUM
;
PONS
; and inferior olivary nuclei. Additional clinical features may include
MUSCLE RIGIDITY
; NYSTAGMUS, PATHOLOGIC;
RETINAL DEGENERATION
;
MUSCLE SPASTICITY
;
DEMENTIA
;
URINARY INCONTINENCE
; and
OPHTHALMOPLEGIA
. The familial form has an earlier onset (second decade) and may feature
spinal cord
atrophy
. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of
MULTIPLE SYSTEM ATROPHY
. (From Adams et al., Principles of
Neurology
, 6th ed, p1085)
Indexing Annotation
English
:
DF: OPCA
See Related
English
:
Multiple System Atrophy
History Note
English
:
2000(1987)
Allowable Qualifiers
English
:
blood
cerebrospinal fluid
chemically induced
classification
congenital
complications
diet therapy
diagnosis
drug therapy
economics
ethnology
embryology
enzymology
epidemiology
etiology
genetics
history
immunology
metabolism
microbiology
mortality
nursing
pathology
prevention & control
physiopathology
parasitology
psychology
radiography
rehabilitation
radionuclide imaging
radiotherapy
surgery
therapy
urine
ultrasonography
veterinary
virology
Record Number:
19275
Unique Identifier:
D009849
Occurrence in VHL
:
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