Progressive Supranuclear Palsy - Overview, Symptoms, Causes, Diagnosis, Treatments, and Prognosis

Progressive Supranuclear Palsy - Overview, Symptoms, Causes, Diagnosis, Treatments, and Prognosis

00:00 - What is progressive supranuclear palsy?
00:50 - What are the symptoms?
02:50 - What causes PSP?
03:51 - How is PSP diagnosed?
04:25 - Treatment
06:07 - What is the prognosis?

--SCRIPT--

Progressive supranuclear palsy (PSP) is a rare brain disorder that causes problems with movement, walking and balance, and eye movement. It results from damage to nerve cells in the brain that control thinking and body movement. The disorder’s long name indicates that the disease worsens (progressive) and causes weakness (palsy) by damaging certain parts of the brain above nerve cell clusters called nuclei (supranuclear) that control eye movements.

PSP is different than Parkinson’s disease—another movement disorder—although they share some symptoms (see section, “How is PSP different from Parkinson’s Disease?”). Currently there is no effective treatment for PSP, but some symptoms can be managed with medication or other interventions.

top
What are the symptoms?

PSP affects movement, control of walking (gait) and balance, speech, swallowing, eye movements and vision, mood and behavior, and thinking. The pattern of signs and symptoms can be quite different from person to person. The most frequent first symptom of PSP is a loss of balance while walking. Individuals may have abrupt and unexplained falls without loss of consciousness, a stiff and awkward gait, or slow movement.

As the disease progresses, most people will begin to develop a blurring of vision and problems controlling eye movement. These symptoms may include:

Slow eye movements.
Trouble voluntarily shifting gaze vertically (i.e., downward and/or upward).
Trouble controlling eyelids.
Tendency to move the head to look in different directions.
Involuntary closing of the eyes.
Prolonged or infrequent blinking.
Difficulty in opening the eyes.
Inability to maintain eye contact during a conversation.

People with PSP often show alterations of mood and behavior. These symptoms may include:

Depression.
Apathy.
Changes in judgment, insight, and problem solving.
Difficulty finding words.
Loss of interest in ordinary pleasurable activities.
Increased irritability and forgetfulness.
Sudden laughing or crying or displaying angry outbursts for no apparent reason.
Personality changes.

Other symptoms may include:

Slowness of thought.
Memory problems.
Slowed, slurred, or monotone speech.
Difficulty swallowing solid foods or liquids.
Mask-like facial expressions.

What causes PSP?
The exact cause of PSP is unknown, but research suggests that it involves a gradual deterioration of brain cells in a few specific areas in the brain, mainly in brain stem. The death of brain cells in one of these areas, the substantia nigra, accounts in part for the motor symptoms that PSP and Parkinson's have in common.

The hallmark of PSP is the accumulation of abnormal deposits of the protein tau in nerve cells in the brain. These deposits cause the cells to malfunction and die, which stops the flow of information to other nerve cells. The accumulation of tau puts PSP in the group of disorders called the tauopathies, which includes Alzheimer’s disease, corticobasal degeneration, and some forms of frontotemporal degeneration.

PSP is usually sporadic, meaning that it occurs infrequently and without a known cause. In very few cases, the disease results from mutations in the MAPT gene. This mutation provides faulty instructions for making tau to the nerve cell. Genetic factors have not been implicated in most individuals.

How is PSP diagnosed?
Currently there are no tests or brain imaging techniques to definitively diagnose PSP. An initial diagnosis is based on the person’s medical history and a physical and neurological exam. Identifying early gait problems, problems moving the eyes, speech and swallowing abnormalities, as well as ruling out other similar disorders is important. Diagnostic imaging may show shrinkage at the top of the brain stem and look at brain activity in known areas of degeneration.

Is there any treatment?

There is currently no effective treatment for PSP and symptoms usually do not respond to medications.

Parkinson’s disease medications, such as ropinirole, rarely provide additional benefit. In some individuals, other antiparkinsonian medications, such as levodopa, can treat the slowness, stiffness, and balance problems associated with PSP, but the effect is usually minimal and short-lasting.
Botulinum toxin, which can be injected into muscles around the eyes, can treat excessive eye closing.
Some antidepressant drugs may offer some benefits beyond treating depression, such as pain relief and decreasing drooling.

Видео Progressive Supranuclear Palsy - Overview, Symptoms, Causes, Diagnosis, Treatments, and Prognosis канала Rehealthify