striatonigral degeneration; nigrostriatal degeneration; multiple system atrophy with predominant parksinsonism (MSA-P)

Epidemiology

age of onset is mid 50's

Pathology

• striatal neuronal loss
• striatal gliosis
• putamen affected more than the caudate nucleus

Genetics

• autosomal dominant
• associated with defects in PDE8B

Clinical manifestations

• similar to Parkinson's disease:
  • muscle rigidity
  • instability
  • dysarthria (impaired speech)
  • bradykinesia
• distinguishing features from Parkinson's disease
  • symmetrical
  • early loss of postural reflexes
  • pyramidal tract signs
  • dementia is uncommon
  • no tremor

Differential diagnosis

• other variants of multiple system atrophy

Management

• not responsive to L-dopa
• dopamine & anticholinergic agents provide some benefit
• re-evaluate as the disorder progresses
• prognosis
  • slow progression
  • some patients have normal life expectancy

More general terms

• multiple system atrophy (MSA)

Additional terms

• Parkinson's disease (paralysis agitans, PD)

Patient information

striatonigral degeneration patient information

Database

• OMIM: https://mirror.omim.org/entry/609161