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Descriptor English: Olivopontocerebellar Atrophies
Descriptor Spanish: Atrofias Olivopontocerebelosas
Descriptor atrofias olivopontocerebelosas
Entry term(s) atrofia olivopontocerebelosa idiopática
síndrome de Dejerine-Thomas
Scope note: Grupo de trastornos esporádicos y hereditarios que comparten ataxia progresiva en combinación con atrofia del CEREBELO, PROTUBERANCIA y de los nucleos olivares inferiores. Otras características clínicas pueden ser RIGIDEZ MUSCULAR, NISTAGMO, DEGENERACIÓN RETINIANA, ESPASTICIDAD MUSCULAR, DEMENCIA, INCONTINENCIA URINARIA y OFTALMOPLEJÍA. La forma familiar tiene un inicio precoz (segunda década) y puede haber atrofia de la médula espinal. La forma esporádica tiende a presentarse en la quinta a sexta década de la vida y se considera un subtipo clínico de ATROFIA DE MÚLTIPLES SISTEMAS. (Adams et al., Principles of Neurology, 6th ed, p1085)
Descriptor Portuguese: Atrofias Olivopontocerebelares
Descriptor French: Atrophies olivo-ponto-cérébelleuses
Entry term(s): Ataxia, Presenile
Ataxias, Presenile
Atrophy, Familial Olivopontocerebellar
Atrophy, Idiopathic Olivopontocerebellar
Atrophy, Inherited Olivopontocerebellar
Atrophy, Nonfamilial Olivopontocerebellar
Atrophy, Olivo-Ponto-Cerebellar
Atrophy, Olivopontocerebellar
Atrophy, Pontoolivocerebellar
Degeneration, Olivo-Ponto-Cerebellar
Degeneration, Olivopontocerebellar
Degenerations, Olivo-Ponto-Cerebellar
Degenerations, Olivopontocerebellar
Dejerine Thomas Syndrome
Dejerine-Thomas Syndrome
Familial Olivopontocerebellar Atrophies
Familial Olivopontocerebellar Atrophy
Idiopathic Olivopontocerebellar Atrophies
Idiopathic Olivopontocerebellar Atrophy
Inherited Olivopontocerebellar Atrophies
Inherited Olivopontocerebellar Atrophy
Nonfamilial Olivopontocerebellar Atrophies
Nonfamilial Olivopontocerebellar Atrophy
Olivo Ponto Cerebellar Atrophy
Olivo Ponto Cerebellar Degeneration
Olivo-Ponto-Cerebellar Atrophy
Olivo-Ponto-Cerebellar Degeneration
Olivo-Ponto-Cerebellar Degenerations
Olivopontocerebellar Atrophies, Familial
Olivopontocerebellar Atrophies, Idiopathic
Olivopontocerebellar Atrophies, Inherited
Olivopontocerebellar Atrophies, Nonfamilial
Olivopontocerebellar Atrophy
Olivopontocerebellar Atrophy, Familial
Olivopontocerebellar Atrophy, Idiopathic
Olivopontocerebellar Atrophy, Inherited
Olivopontocerebellar Atrophy, Nonfamilial
Olivopontocerebellar Degeneration
Olivopontocerebellar Degenerations
Pontoolivocerebellar Atrophies
Pontoolivocerebellar Atrophy
Presenile Ataxia
Presenile Ataxias
Syndrome, Dejerine-Thomas
Tree number(s): C10.177.575.550.375
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
C16.320.400.780.750
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D009849
Scope note: 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)
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CN congenital
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Atrophy (1966-1986)
Brain Diseases (1966-1986)
Cerebellar Ataxia (1968-1986)
Cerebellar Diseases (1966-1986)
Olivary Nucleus (1966-1986)
Pons (1966-1986)
Public MeSH Note: 2000; see OLIVOPONTOCEREBELLAR ATROPHY 1991-1999, see SPINOCEREBELLAR DEGENERATION 1987-1990
History Note: 2000(1987)
Entry Version: OPCA
Related: Multiple System Atrophy MeSH
DeCS ID: 19275
Unique ID: D009849
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1986/03/31
Revision Date: 2015/06/25
Olivopontocerebellar Atrophies - Preferred
Concept UI M0015287
Scope note 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)
Preferred term Olivopontocerebellar Atrophies
Entry term(s) Ataxia, Presenile
Ataxias, Presenile
Atrophy, Olivo-Ponto-Cerebellar
Atrophy, Olivopontocerebellar
Atrophy, Pontoolivocerebellar
Degeneration, Olivo-Ponto-Cerebellar
Degeneration, Olivopontocerebellar
Degenerations, Olivo-Ponto-Cerebellar
Degenerations, Olivopontocerebellar
Dejerine Thomas Syndrome
Dejerine-Thomas Syndrome
Olivo Ponto Cerebellar Atrophy
Olivo Ponto Cerebellar Degeneration
Olivo-Ponto-Cerebellar Atrophy
Olivo-Ponto-Cerebellar Degeneration
Olivo-Ponto-Cerebellar Degenerations
Olivopontocerebellar Atrophy
Olivopontocerebellar Degeneration
Olivopontocerebellar Degenerations
Pontoolivocerebellar Atrophies
Pontoolivocerebellar Atrophy
Presenile Ataxia
Presenile Ataxias
Syndrome, Dejerine-Thomas
Olivopontocerebellar Atrophy, Idiopathic - Narrower
Concept UI M0332843
Preferred term Olivopontocerebellar Atrophy, Idiopathic
Entry term(s) Atrophy, Idiopathic Olivopontocerebellar
Idiopathic Olivopontocerebellar Atrophies
Idiopathic Olivopontocerebellar Atrophy
Olivopontocerebellar Atrophies, Idiopathic
Familial Olivopontocerebellar Atrophy - Narrower
Concept UI M0332844
Preferred term Familial Olivopontocerebellar Atrophy
Entry term(s) Atrophy, Familial Olivopontocerebellar
Atrophy, Inherited Olivopontocerebellar
Familial Olivopontocerebellar Atrophies
Inherited Olivopontocerebellar Atrophies
Inherited Olivopontocerebellar Atrophy
Olivopontocerebellar Atrophies, Familial
Olivopontocerebellar Atrophies, Inherited
Olivopontocerebellar Atrophy, Familial
Olivopontocerebellar Atrophy, Inherited
Nonfamilial Olivopontocerebellar Atrophy - Narrower
Concept UI M0332845
Preferred term Nonfamilial Olivopontocerebellar Atrophy
Entry term(s) Atrophy, Nonfamilial Olivopontocerebellar
Nonfamilial Olivopontocerebellar Atrophies
Olivopontocerebellar Atrophies, Nonfamilial
Olivopontocerebellar Atrophy, Nonfamilial



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