Progressive supranuclear palsy
Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain. The condition leads to symptoms including loss of balance, slowing of movement, difficulty moving the eyes, and cognitive impairment. 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.
| 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. | |
| 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 |
PSP affects about six people per 100,000. The first symptoms typically occur at 60–70 years of age. Males are slightly more likely to be affected than females. No association has been found between PSP and any particular race, location, or occupation.