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olivopontocerebellar atrophy (OPCA) or degeneration (OPCD); multiple system atrophy, cerebellar subtype
Parkinsonism plus cerebellar signs & symptoms.
Epidemiology: age of onset 50-60 years, range 20-79 years
Genetics: both sporadic & inherited forms
Pathology:
1) atrophy & cell loss in pons, cerebellum & inferior olive
2) depigmentation of substantia nigra
Clinical manifestations: (sporadic form)
1) early ataxia
2) loss of postural reflexes
3) pyramidal tract signs
4) dystonia
5) dysarthria
6) dysphagia
7) oculomotor disturbances
8) peripheral neuropathy
9) dementia is uncommon
Differential diagnosis:
- other variants of multiple system atrophy
Management:
1) Sinemet (not as responsive as Parkinson's disease)
2) see Parkinson's disease
Interactions
disease interactions
Related
spinocerebellar ataxia (SCA)
Specific
olivopontocerebellar atrophy type 1 (Menzel type); spinocerebellar ataxia type 1 (SCA-1)
olivopontocerebellar atrophy type 2 (Fickler-Winkler type)
olivopontocerebellar atrophy type 3 (retinal degeneration) or spinocerebellar ataxia type 7 (SCA-7)
olivopontocerebellar atrophy type 4 (Schut-Haymaker type)
olivopontocerebellar atrophy type 5 (parkinsonism, ophthalmoplegia and dementia)
olivopontocerebellar atrophy, Holguin type or spinocerebellar ataxia type 2 (SCA-2)
General
genetic disease of the central nervous system
multiple system atrophy (MSA)
References
- Bronstein J, UCLA Movement Disorders Clinic, 2001
- NINDS Olivopontocerebellar Atrophy Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Olivopontocerebellar-Atrophy-Information-Page