Progressive supranuclear palsy is a neurodegenerative disorder caused by destruction of brain cells, primarily in the brain stem, and accumulation of tau protein. Average age of onset is 60-65 years, and it is more common in men. Symptoms include parkinsonism (bradykinesia, rigidity, with or without tremor), axial rigidity, gait / postural instability and early falls, eye movement abnormalities, blurry vision, blepharospasm, eyelid opening apraxia, dysarthria, dysphagia, retrocollis, cognitive decline, depression, anxiety, apathy, behavioral changes, and pseudobulbar palsy.
Lack of response to levodopa is common. Prognosis is poor; most patients are significantly disabled within 5 years of diagnosis and die within 10 years of diagnosis.
Progressive supranuclear palsy
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Synopsis

Codes
ICD10CM:
G23.1 – Progressive supranuclear ophthalmoplegia [Steele-Richardson-Olszewski]
SNOMEDCT:
192976002 – Progressive supranuclear palsy
G23.1 – Progressive supranuclear ophthalmoplegia [Steele-Richardson-Olszewski]
SNOMEDCT:
192976002 – Progressive supranuclear palsy
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Differential Diagnosis & Pitfalls
- Parkinson disease
- Corticobasal syndrome
- Dementia with Lewy bodies
- Frontotemporal dementia
- Alzheimer disease
- Vascular dementia and/or parkinsonism
- Multiple system atrophy
- Normal pressure hydrocephalus
- Creutzfeldt-Jakob disease
- Paraneoplastic encephalopathy
- Huntington disease
- Drug-induced parkinsonism
- Neurodegeneration with brain iron accumulation
- Whipple disease
- Wilson disease
- Neuroacanthocytosis
- Neurosyphilis
- Vitamin B12 deficiency
- Human immunodeficiency virus / AIDS dementia
- Wernicke-Korsakoff syndrome
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Last Reviewed:10/18/2018
Last Updated:01/20/2022
Last Updated:01/20/2022