Progressive supranuclear palsy

Progressive supranuclear palsy
Other names	Steele–Richardson–Olszewski syndrome, frontotemporal dementia with parkinsonism
A person with progressive dementia, ataxia, and incontinence. A clinical diagnosis of normal-pressure hydrocephalus was entertained. Imaging did not support this, however, and on formal testing, abnormal nystagmus and eye movements were detected. A sagittal view of the CT/MRI scan shows atrophy of the midbrain, with preservation of the volume of the pons. This appearance has been called the "hummingbird sign" or "penguin sign". Also, atrophy of the tectum is seen, particularly the superior colliculi. These findings suggest the diagnosis of progressive supranuclear palsy.[1]
Specialty	Neurology
Symptoms	Impaired balance, slowed movements, difficulty moving eyes, dementia
Usual onset	60–70 years
Causes	Unknown
Differential diagnosis	Parkinson's disease, corticobasal degeneration, FTDP-17, Alzheimer's disease
Treatment	Medication, physical therapy, occupational therapy
Medication	Levodopa, amantadine
Prognosis	Fatal (usually 7–10 years after diagnosis)
Frequency	6 per 100,000

Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain.^[2][3] The condition leads to symptoms including loss of balance, slowing of movement, difficulty moving the eyes, and cognitive impairment.^[2] PSP may be mistaken for other types of neurodegeneration such as Parkinson's disease, frontotemporal dementia and Alzheimer's disease. The cause of the condition is uncertain, but involves the accumulation of tau protein within the brain. Medications such as levodopa and amantadine may be useful in some cases.^[2]

PSP affects about six people per 100,000.^[2] The first symptoms typically occur at 60–70 years of age. Males are slightly more likely to be affected than females.^[2] No association has been found between PSP and any particular race, location, or occupation.^[2]