Preferred Label : olivopontocerebellar atrophies;

MeSH definition : 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);

MeSH synonym : atrophy, pontoolivocerebellar; olivo ponto cerebellar degeneration; olivopontocerebellar atrophy; degeneration, olivopontocerebellar; olivopontocerebellar degeneration; presenile ataxias; pontoolivocerebellar atrophies; degeneration, olivo-ponto-cerebellar; olivo-ponto-cerebellar degenerations; pontoolivocerebellar atrophy; olivopontocerebellar degenerations; olivo-ponto-cerebellar degeneration; syndrome, dejerine-thomas; presenile ataxia; ataxia, presenile; olivo ponto cerebellar atrophy; dejerine-thomas syndrome; dejerine thomas syndrome; atrophy, olivo-ponto-cerebellar; atrophy, olivopontocerebellar; olivo-ponto-cerebellar atrophy;

CISMeF synonym : degenerations, olivo-ponto-cerebellar; degenerations, olivopontocerebellar; ataxias, presenile;

MeSH hyponym : olivopontocerebellar atrophy, idiopathic; nonfamilial olivopontocerebellar atrophy; familial olivopontocerebellar atrophy; Atrophy, Idiopathic Olivopontocerebellar; Idiopathic Olivopontocerebellar Atrophies; Idiopathic Olivopontocerebellar Atrophy; Olivopontocerebellar Atrophies, Idiopathic; Atrophy, Familial Olivopontocerebellar; Familial Olivopontocerebellar Atrophies; Olivopontocerebellar Atrophies, Familial; Olivopontocerebellar Atrophy, Familial; Inherited Olivopontocerebellar Atrophy; Atrophy, Inherited Olivopontocerebellar; Inherited Olivopontocerebellar Atrophies; Olivopontocerebellar Atrophies, Inherited; Olivopontocerebellar Atrophy, Inherited; Atrophy, Nonfamilial Olivopontocerebellar; Nonfamilial Olivopontocerebellar Atrophies; Olivopontocerebellar Atrophies, Nonfamilial; Olivopontocerebellar Atrophy, Nonfamilial;

Related MeSH term : Hypoplasia, Olivopontocerebellar; Olivopontocerebellar Hypoplasias;

MeSH annotation : DF: OPCA;

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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)

http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=227510
2011
France
scientific and technical information
multiple system atrophy (MSA) with orthostatic hypotension
olivopontocerebellar atrophies
multiple system atrophy

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http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=2732
2007
France
French
resource guides
rare diseases
olivopontocerebellar atrophies
deafness
scientific and technical information

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03/05/2025


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