Olivopontocerebellar Atrophies (definition)

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Olivopontocerebellar Atrophies

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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)

DF: OPCA
See Also Multiple System Atrophy

Other names Olivopontocerebellar Atrophy, Idiopathic; Dejerine-Thomas Syndrome; Syndrome, Dejerine-Thomas; Presenile Ataxias; Pontoolivocerebellar Atrophies; Olivopontocerebellar Degenerations; Olivopontocerebellar Atrophy, Nonfamilial; Olivopontocerebellar Atrophy, Inherited; Olivopontocerebellar Atrophy, Familial; Olivopontocerebellar Atrophies, Nonfamilial; Olivopontocerebellar Atrophies, Inherited; Olivopontocerebellar Atrophies, Idiopathic; Olivopontocerebellar Atrophies, Familial; Olivo-Ponto-Cerebellar Degenerations; Olivo Ponto Cerebellar Degeneration; Olivo Ponto Cerebellar Atrophy; Nonfamilial Olivopontocerebellar Atrophies; Inherited Olivopontocerebellar Atrophies; Idiopathic Olivopontocerebellar Atrophy; Idiopathic Olivopontocerebellar Atrophies

Substance	Categories	Source
Pontocerebellar Hypoplasia Type 6	*Olivopontocerebellar Atrophies.
Pontocerebellar Hypoplasia Type 3	*Olivopontocerebellar Atrophies.
Pontocerebellar Hypoplasia Type 2	*Olivopontocerebellar Atrophies.
Pontocerebellar Hypoplasia Type 1	*Olivopontocerebellar Atrophies.
Olivopontocerebellar atrophy 3	*Olivopontocerebellar Atrophies.
Olivopontocerebellar atrophy 2	*Olivopontocerebellar Atrophies.
Olivopontocerebellar atrophy 1	*Olivopontocerebellar Atrophies.
Olivopontocerebellar hypoplasia, fetal-onset	*Olivopontocerebellar Atrophies.
Microcephaly pontocerebellar hypoplasia dyskinesia	Microcephaly Olivopontocerebellar Atrophies *Dyskinesias.
Young McKeever Squier syndrome	*Olivopontocerebellar Atrophies/congenital.	J Med Genet. 1992 Oct;29(10):733-5

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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