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striatonigral degeneration; nigrostriatal degeneration; multiple system atrophy with predominant parksinsonism (MSA-P)
Epidemiology: age of onset is mid 50's
Pathology:
1) striatal neuronal loss
2) striatal gliosis
3) putamen affected more than the caudate nucleus
Genetics:
- autosomal dominant
- associated with defects in PDE8B
Clinical manifestations:
1) similar to Parkinson's disease:
a) muscle rigidity
b) instability
c) dysarthria (impaired speech)
d) bradykinesia
2) distinguishing features from Parkinson's disease
a) symmetrical
b) early loss of postural reflexes
c) pyramidal tract signs
d) dementia is uncommon
e) no tremor
Differential diagnosis:
- other variants of multiple system atrophy
Management:
1) not responsive to L-dopa
2) dopamine & anticholinergic agents provide some benefit
3) re-evaluate as the disorder progresses
4) prognosis
a) slow progression
b) some patients have normal life expectancy
Interactions
disease interactions
Related
Parkinson's disease (paralysis agitans, PD)
General
multiple system atrophy (MSA)
Properties
PATHOLOGY: glial cytoplasmic inclusion
neuronal cytoplasmic inclusion
Database Correlations
OMIM 609161
References
- Bronstein J, UCLA Movement Disorders Clinic, 2001
- NINDS Striatonigral Degeneration Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Striatonigral-Degeneration-Information-Page