Preferred Label : olivopontocerebellar degeneration;
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];
UMLS CUI (RADLEX) : C0028968;
Origin ID : RID5162;
UMLS CUI : C0028968;
Currated CISMeF NLP mapping
- Semantic type(s)
- UMLS correspondences (same concept)
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]
