olivopontocerebellar atrophy (OPCA) or degeneration (OPCD); multiple system atrophy, cerebellar subtype

Introduction

Parkinsonism plus cerebellar signs & symptoms.

Epidemiology

age of onset 50-60 years, range 20-79 years

Pathology

• atrophy & cell loss in pons, cerebellum & inferior olive
• depigmentation of substantia nigra

Genetics

both sporadic & inherited forms

Clinical manifestations

(sporadic form)

• early ataxia
• loss of postural reflexes
• pyramidal tract signs
• dystonia
• dysarthria
• dysphagia
• oculomotor disturbances
• peripheral neuropathy
• dementia is uncommon

Differential diagnosis

• other variants of multiple system atrophy

Management

• Sinemet (not as responsive as Parkinson's disease)
• see Parkinson's disease

More general terms

• multiple system atrophy (MSA)
• genetic disease of the central nervous system

More specific terms

• olivopontocerebellar atrophy type 1 (Menzel type); spinocerebellar ataxia type 1 (SCA-1)
• olivopontocerebellar atrophy type 2 (Fickler-Winkler type)
• 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)
• olivopontocerebellar atrophy type 3 (retinal degeneration) or spinocerebellar ataxia type 7 (SCA-7)

Additional terms

• spinocerebellar ataxia (SCA)

Patient information

olivopontocerebellar atrophy patient information