Search on: OLIVOPONTOCEREBELLAR ATROPHIES 
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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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