What Is Progressive Supranuclear Palsy (PSP)?
PSP is a type of frontotemporal degeneration that affects approximately 20,000 Americans. It results in problems with motor functioning (such as walking and balance) and brain functioning (such as memory and thought processing). PSP was identified in 1964 as a specific disease and is sometimes referred to as Steele-Richardson-Olszewski Syndrome, named after the Canadian scientists who identified it.
The brains of people with PSP show a similar buildup of the tau protein that occurs with Alzheimer's disease.
Who Gets PSP?
More men than women get PSP, and the average age of onset is in the early 60s. There is not a strong component of heredity in PSP and how it develops is not well understood. PSP does not affect one ethnic group more than another.
Symptoms
PSP's initial symptoms include poor balance, unsteady gait in walking and unexplained falls. Memory problems, emotional outbursts, depression, apathy and irritability are other symptoms, as well as complaints of double vision or difficulty moving the eyes.
Eye movement problems are one of the hallmark symptoms of PSP. Because of the lesions that develop in the brain, it becomes difficult to aim the eyes correctly. For example, a person may not be able to maintain eye contact during a conversation.
As PSP continues to progress, most people require 24-hour assistance with their care needs.
Diagnosis
There is no test to definitively diagnose PSP, so physicians focus on ruling out other conditions. An accurate diagnosis of PSP often is not reached until it progresses to the stage when the eyes begin to function poorly. Until then, it is often misdiagnosed as Parkinson’s.
How Is PSP Different from Parkinson's?
PSP is different from Parkinson’s in several ways. One significant difference is that individuals with PSP often stand straight up or even lean back (causing the risk for falling backwards), while those with Parkinson’s often lean forward and bend over. As noted, eye movements are impaired in PSP, but remain fairly normal in Parkinson's. PSP also typically affects speech and motor functioning earlier and more significantly than Parkinson's.
How Is PSP Different from Alzheimer's Disease?
Although both PSP and Alzheimer's cause dementia, PSP's earlier symptoms may be falls or balance problems, while the early symptoms of Alzheimer's are usually memory problems and difficulty with learning new information. Alzheimer's does diminish the ability to walk and physically function, but usually this does not occur until the disease has significantly progressed into the later stages.Treatment
Treatment options for PSP are limited. Some patients experience temporary improvement in their symptoms with the use of medications like Sinemet, a prescription drug traditionally used to treat Parkinson’s. There also has been some improvement in motor and movement symptoms with Elavil, an antidepressant, though some patients report decreased balance when taking this drug.
Prognosis
People with PSP generally decline in functioning over time. Life expectancy may be up to 12 years after initial symptoms; some people live well into their 70s with PSP. PSP by itself is not fatal, but death usually results from a complication of PSP, such as choking or pneumonia.
Sources:
The Association for Frontotemporal Degeneration. http://www.theaftd.org/
Golbe, Lawrence I., MD. Cure PSP. PSP: Some Information. April 2011. http://www.psp.org/about/
National Center for Biotechnology Information. U.S. National Library of Medicine. Pub Med Health. Pick’s Disease. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001752/
National Institute of Neurological Disorders and Stroke. National Institutes of Health. Progressive Supranuclear Palsy Fact Sheet. Accessed December 11, 2011.http://www.ninds.nih.gov/disorders/psp/detail_psp.htm
Northwestern Memorial Hospital. Progressive Supranuclear Palsy. Accessed January 26, 2012. http://www.nmh.org/nm/progressive-supranuclear-palsy
University of California, San Francisco. Forms of Frontotemporal Dementia. Accessed December 11, 2011. http://memory.ucsf.edu/ftd/overview/ftd/forms/multiple
U.S. National Institutes of Health. National Institute on Aging. Types of Frontotemporal Disorders. Accessed December 12, 2011. http://www.nia.nih.gov/Alzheimers/Publications/FTLD/02.htm
