Progressive supranuclear Palsy, or PSP, is a rare neurodegenerative disease sometimes referred to as the Steele-Richardson-Olszewski Syndrome for the physicians who first described it in 1964. Approximately five to six individuals per 100,000 will develop the disease, which is much less common than Parkinson's disease but more common than the well-known Lou Gehrig's disease, according to the Foundation for PSP-CBD and Related Brain Diseases.
Identification
Progressive supranuclear palsy is a degenerative brain disease for which there is no cure. It is characterized by the loss of nerve cells which control balance and gait, eye movements, and speech and swallowing. There is currently no cure for PSP and treatment options have not been very effective. The disease is often misdiagnosed as the symptoms resemble Parkinson's disease and other forms of dementia.
Symptoms
On average, symptoms of PSP generally develop in individuals in their early 60s, but can be found in those in their 40s. Typically, the first noticeable symptoms are problems with balance and eye movements. Individuals notice changes in gait and report falling down more frequently. Blurring vision is reported as the disease results in the inability to properly aim the eyes due to muscle paralysis, or palsy. Symptoms progressively get worse over time, causing increased walking impairments, difficulties in speech and swallowing, slowed mental thoughts and continued eyesight impairments. Personality changes are also present in PSP, with increased irritability and loss of interest in things normally considered pleasurable.
Causes
PSP and its associated symptoms are the result of the general deterioration of nerve cells in specific parts of the brain stem. One important structure affected is the substantia nigra which plays a role in movement as well as reward and addiction. However, the reason for this deterioration is still unknown, but researchers have proposed several possible reasons. A viral infection that enters the body and slowly attacks the body leading to the symptoms of PSP is one possible cause; another is the possibility of random genetic mutations. Some clues have led scientists to suspect environmental and dietary toxins as a possible cause, along with damage to nerve cells by free radicals, according to the Mayo Clinic.
Diagnosis
There is no specific test for the diagnosis of PSP, so physicians must rely on symptom identification for diagnosis. However, since the symptoms closely resemble Parkinson's disease and other forms of dementia, especially in the early stages, the disease is often misdiagnosed. Two important hallmarks of PSP which help in identifying the disease are gait instabilities and difficulties with eye movements. A person with PSP will generally slouch backwards slightly--as opposed to forwards in Parkinson's--and have trouble looking downward and making eye contact. Tremors are also generally not present in individuals with PSP, unlike in those with Parkinson's.
Treatment
Treatment for PSP is focused on managing symptoms, yet no specific treatment as been shown to be very effective in alleviating symptoms. Some of the medications used for Parkinson's disease therapy are prescribed for PSP. The most common include dopamine agonists and other combination drugs aimed at increasing levels of the neurotransmitter dopamine in the brain. However, they provide few benefits; at best, the benefits are also only temporary, lasting a few years, according to the Foundation for PSP-CBD and Related Brain Diseases. One therapy often used to treat eyelid spasms is Botox injections. These injections contain botulinum toxin, a neurotoxin produced by a specific bacteria that interferes with the chemical signals needed for muscle contraction. A small amount is carefully injected into the muscle or tissue of the eyelid to relieve symptoms. Bifocals and special eyeglasses called "prisms" have also been used to help with difficulties in looking downward.
