- Late-onset degenerative disease involving the gradual deterioration and death of specific volumes of the brain
- Course
- Rapidly progressive or early dementia
- Rapidly progressive course
- The condition leads to symptoms including
- Upper Motor Neuron signs
- Axial rigidity is greater than extremity rigidity
- Cerebellar signs (dysmetria, Ataxia) with impaired gait
- Impaired speech
- Urinary Incontinence
- Early symptomatic Postural Hypotension
- Distinguishing features
- Resting Tremor absent
- Refractory to Carbidopa/Levodopa
- may be mistaken for other neurodegenerative diseases such as Parkinson’s, frontotemporal dementia and Alzheimer’s.
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