Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a movement disorder caused by damage to certain nerve cells in the brain. The cause of the damage is unknown. PSP might be genetic (passed down through families) or might be caused by a virus or exposure to toxins.

The symptoms of PSP are similar to those of many neurological and psychiatric disorders. PSP is difficult to diagnose. To help with diagnosis, a list of criteria (both symptoms and non-symptoms) was created.

A cure has yet to be found for PSP. At present, treatment can only help ease your symptoms. Doctors may use medications or techniques such as physical therapy to treat PSP. There is no complete recovery from PSP, and the condition is eventually fatal.

The exact cause of PSP is unknown, though several theories have been suggested.

Some cases appear to be inherited (passed down through families). This suggests that PSP may have a genetic cause. It might be due to a gene abnormality or mutation. Gene mutations are changes in the body’s DNA (the blueprint for all our physical and mental traits).

PSP might also be caused by exposure to toxins, such as pesticides.

The disease may also come from a virus. The theory is that a virus might enter the body unnoticed and replicate for years until it finally causes a genetic mutation that leads to PSP.

These theories are still being tested.

PSP is a progressive neurological disorder. Symptoms begin slowly and gradually become more obvious. The symptoms of PSP are also common in other neurological and psychiatric disorders.

Common clinical symptoms of PSP include:

• slow, unsteady walk
• loss of balance, making it easy to fall
• difficulty looking upwards (supranuclear vertical gaze)
• falls during the first year (during the first year of noticeable symptoms, patients will have unexpected falls)
• dementia (slowed thoughts, memory problems)
• difficulty swallowing and slurred speech

Psychiatric conditions that resemble PSP include:

• schizophrenia (a disorder in which patients may have altered thoughts or perceptions that sometimes cause them to hallucinate)
• bipolar disorder (a disorder in which a patient cycles between feeling elated and being deeply depressed)
• severe depression (a disorder in which patients may have no interest in life, feel deeply sad, and potentially become suicidal)

Neurological diseases that resemble PSP include:

• Alzheimer’s disease (AD)
• multi-infarct dementia (MID)
• Lewy body disease (buildup of abnormal structures in the brain)

PSP is underdiagnosed because of its resemblance to other neurologic and psychiatric disorders. Doctors have determined that the first year is the most important for making an accurate diagnosis. The best way to clinically differentiate PSP is by using these essential criteria.

For a clinical diagnosis of PSP, the following are necessary criteria:

• onset at age 40 or later
• gradual progression of the syndrome
• vertical supranuclear gaze (difficulty looking upwards)
• falls in the first year after symptoms present (falls occur for no reason)

The following criteria are not found in PSP and separate it from similar neurological disorders:

• response to treatment with levodopa medication (Parkinson’s disease)
• hallucinations or delusions (Lewy body disease)
• alien limb syndrome (a hand or other limb moves without conscious control – characteristic of corticobasal degeneration)

PSP has no cure. Neurologists can only focus on easing the symptoms. Antidepressants may be prescribed to help treat emotional issues, such as depression and apathy (lack of interest). Medications for this treatment include:

• duloxetine (Cymbalta)
• aripiprazole (Abilify)
• escitalopram (Lexapro)

Many of the symptoms of PSP cannot be treated with oral medication alone. These symptoms include eyelid spasms, which can be treated with botulinum toxin injections, and exposure keratitis (drying of the eye caused by inadequate closure), which is treated with eye drops.

PSP’s progressing symptoms will eventually require more than medications. Patients may need help walking and communicating. Supportive measures include:

• soft-wheeled walker or wheelchair
• physical or occupational therapy
• speech therapy for swallowing difficulties

Patients with PSP are facing a disease with no cure. Doctors will try to treat their patient’s symptoms and offer support as the disease progresses. The neurodegeneration will progress until it is severe enough to be fatal.

Prevention is difficult, as the exact cause of PSP is unknown. The best way to prevent any disease is by practicing a healthy lifestyle. Ways to maintain a healthy lifestyle include:

• refraining from smoking or using recreational drugs
• following a regular exercise routine
• eating a diet high in fiber and low in saturated fats and sugar