Progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

2021-08-28 08:54 AM

Signs and symptoms of progressive supranuclear palsy vary from person to person and can mimic those of Parkinson's disease and dementia.

Define

Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a brain disorder that causes severe problems with walking, balance, and eyes. Although the cause is unknown, PSP results from the deterioration of cells in areas of the brain that control movement.

The brain degeneration is a rare disorder that slowly gets worse over time. It usually affects adults over 60, although some are diagnosed as young as 40. While PSP itself is not life-threatening, it can lead to life-threatening complications such as pneumonia and problems with swallowing.

Because there is no cure for PSP, treatment focuses on managing and improving the signs and symptoms of this disorder.

Symptoms

Characteristic signs and symptoms of PSP include:

Loss of balance while walking. This can happen very soon.

Inability to close the eyes properly. This is especially true when looking down, or can happen as blurring or double vision. Eyes have difficulty focusing.

Signs and symptoms of progressive supranuclear palsy vary from person to person and can mimic those of Parkinson's disease and dementia. These may include:

Muscle stiffness.

Clumsy.

Fall.

Problems with speech and swallowing.

Uncomfortable.

Loss of interest in activities (apathy).

Worried.

Laugh or cry for no reason.

Negligent.

As the name implies, the signs and symptoms of progressive supranuclear palsy gradually tend to get worse.

Make an appointment with your doctor if you experience signs and symptoms of progressive supranuclear palsy.

Causes

The cause of progressive supranuclear palsy is not known. However, the signs and symptoms of the disease result from the deterioration of nerve cells in the brain, cerebral cortex, and basal ganglia. Areas of the brain that help control body movements. This explains why the decline leads to coordination and movement problems of the disease.

Although no known cause exists, scientists have several theories about what could be progressive supranuclear palsy. These include:

Viruses. Some speculate that a virulent virus enters the body, years before it becomes activated and begins to cause signs and symptoms.

Gene Mutation. It is possible that random changes (mutations), which can develop in everyone's genes, occur in specific genes that damage cells that cause progressive supranuclear palsy.

Exposure to the environment. Some scientists think that an unknown chemical in the environment may be the cause of the disease. This chemical can be something eaten, drunk, or breathed in. For example, certain tropical fruits have been linked to progressive supranuclear palsy in people on certain Caribbean islands.

Damage caused by free radicals. When food is processed, substances called free radicals are produced. Free radicals are thought to contribute to aging and certain diseases. May damage brain cells associated with signs and symptoms of advanced degenerative brain disease.

Risk factors

Age is the most important known risk factor for progressive supranuclear palsy. This disease usually affects people over 60 years of age. In addition, men are affected somewhat more often than women.

Complications

Complications of progressive supranuclear palsy mainly from impediments to muscle movement. Complications may include:

Frequent falls, which can lead to head injuries, fractures, and other injuries.

Eyes have difficulty focusing, which can also lead to injury.

Problems reading, or with other tasks that require hand-eye coordination.

Wheelchair required.

Dependent on others for care.

Difficulty sleeping.

Difficulty seeing in the light.

Swallowing problems.

Difficulty swallowing can lead to choking or aspiration of food or liquids into the airways. Can develop into pneumonia - the most common cause of death in people with advanced brain degeneration.

Tests and diagnostics

Doctors diagnose progressive supranuclear palsy by identifying the vital signs of the disorder - problems with balance and walking and difficulty with eye movement, especially reducing - and ruling out disorders other similar.

progressive supranuclear palsy can be difficult to diagnose because some of the signs and symptoms, such as muscle stiffness, mobility difficulties, and mood or personality changes, are similar to those of Parkinson's disease and dementia. . In fact, because of its similarity to Parkinson's disease, it is considered a "Parkinson-plus syndrome."

Signs of more progressive supranuclear palsy than Parkinson's disease include lack of vibration (concussion) and poor response to Parkinson's medications.

Although a diagnosis can be made through an evaluation process, currently the only definitive way to determine if a person has advanced brain degeneration is through an autopsy. Research is underway to develop medical tests that can help diagnose advanced brain degeneration.

Treatments and medicine

Radical treatment does not exist for advanced brain degeneration. However, certain medications and other measures can help improve some of the symptoms of this disorder.

Medicine

Medicines for Parkinson's patients, including carbidopa-levodopa, a dopamine agonist, and Amantadine, which increase levels of dopamine, a chemical that transmits signals between regions of the brain. These medications can help improve slow motion, muscle stiffness, and balance problems in about 30 percent of people. However, the effectiveness of the drugs is limited and usually temporary, lasting about two years.

Some antidepressants. Antidepressants, including fluoxetine (Prozac), imipramine (Tofranil), and amitriptyline, may improve symptoms. The mechanism of action is unknown; The benefit did not appear to be related to its ability to treat depression.

Treatments

Botulinum toxin (Botox). Botulinum can be injected into the muscle or tissue around the eye. When injected in small doses into specific muscles, Botox blocks the chemical signals that cause muscle contraction, which can increase eyelid spasms, although not eye movement.

Eyeglasses with bifocal lenses. May help alleviate problems when looking down.

Evaluation of speaking and swallowing. Performing speech therapy, this assessment can help specialists advise on safe swallowing techniques.

Physiotherapy and occupational therapy. This can help improve balance to avoid falls and to help with daily living.

Lifestyle and remedies

There are several things you can do at home to minimize the effects of progressive supranuclear palsy. It may include:

Eye drops. Eye drops can help relieve dry eyes.

Avoid falling. Install hallway and bathroom bars or use an exercise frame to help prevent and help avoid falls. Making changes, such as removing carpets or other hard-to-see objects, can also help with visibility. When possible, avoid climbing stairs.

Coping and supporting

Living with any chronic illness can be difficult, feeling angry, depressed, and frustrated. Degeneration is particularly problematic because it can cause changes in the brain that make you feel anxious or laugh or cry for no reason. progressive supranuclear palsy can also become extremely uncomfortable as walking, talking, and even eating become more difficult.

The good news is that there are steps you can take to manage the stress of living with advanced brain degeneration. Consider these suggestions:

Learn all you can about the condition. Find out how the disease is progressing, the prognosis, and the options and treatment of side effects. The more you know, the more activities you can do in self-care. In addition to talking to your health care team, look for books and information on the Internet, including progressive supranuclear palsy websites.

Be proactive. Although it is often possible to feel anxious or depressed, do not let others - including family and doctors - make important decisions for you. Take an active role in treatment.

Maintain a strong support system. Strong relationships are crucial in dealing with chronic illness. While friends and family can be your best allies, understanding those who know what they're going through can be especially helpful. Support groups aren't for everyone, but for many, they can be a good resource for factual information. Support groups may also exist for families of people with advanced brain degeneration. To learn about support groups in your community, talk to your doctor, social worker, or a public health nurse.

Maintain good communication with partners and support system. Emotional openness is important, especially when it comes to living with advanced brain degeneration. Mood and personality changes or inappropriate behavior, such as sudden laughter or crying, can make communication difficult. If it is possible to talk honestly is best about these changes. The amount of time a caregiver spends with progressive supranuclear palsy is also often an issue. Because it can take a long time to do common tasks with the help of family and partners. But stay as independent as possible. Need to let them know when help is needed and when not.

Most importantly, try to talk candidly about your feelings and concerns. Repressed emotions can be harmful to immediate and long-term health and well-being. If necessary, discuss issues with your doctor or counselor.

Related articles:

- Progressive supranuclear palsy (Pdf) 

- Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy (Pdf)