PSP Disease

1 What is Progressive Supranuclear Palsy?

Progressive supranuclear palsy, or Steele-Richardson-Olszewski syndrome, is a rare brain disorder that affects the body’s motor activities, such as balance, walking, and eye movements.

It is a result of brain cells deterioration, particularly in the part of the brain where body movement and thinking are controlled. The condition worsens over time and fatal complications are common.

Unfortunately, Progressive supranuclear palsy has no cure and treatment usually focuses only on coping with the signs and symptoms.

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2 Symptoms

Progressive supranuclear palsy is characterized by the following signs and symptoms:

  • Balance loss, especially when walking. In early stages of the disease, you may notice a loss of balance. Falling backward as you walk can occur.
  • Compromised vision. A person with Progressive supranuclear palsy is more likely to experience double vision, blurring, or difficulty looking downward.

Other signs and symptoms for this condition are very similar to the signs and symptoms of Dementia and Parkinson’s disease. These may include:

  • Stiffness of the body
  • Awkward movements
  • Speech and swallowing problems
  • Sensitivity to light
  • Impulsive behavior
  • Memory loss 
  • Difficulties with reasoning and problem-solving
  • Sleep disturbances
  • Depression 
  • Anxiety
  • Rigid facial muscles that can make facial expression to appear frightened or surprised

3 Causes

The cause of progressive supranuclear palsy is still unknown.

The symptoms of the condition are a result of cell deterioration in the parts of the brain where body movements and thinking are controlled. However, researches state that abnormal amounts of tau, a type of protein, are found in the deteriorating brain cells of progressive supranuclear palsy. This characteristic is also present in other neurodegenerative disorders like Alzheimer’s disease.

While genetic link is not yet proved, the disorder runs in the family in rare cases. Most of the time, the patient has not inherited the disorder.

4 Making a Diagnosis

Your general physician may refer you to a neurologist, or a specialist in brain and nervous system disorders. Before going to the appointment, it would be advisable to write down important things about your condition, such as:

  • Symptoms that you are experiencing, and how long have you got them
  • Key personal information, such as medical background, major stresses, and if there are some major changes that happened lately
  • List of all the medications, supplements, or vitamins that you are taking

It would also help if you bring along a family member or a trusted friend with you to help you remember anything that you missed. Since your time with your doctor is limited, it’ll be ideal to write down the questions you would like to ask.

Here are some indications that you have progressive supranuclear palsy rather than Parkinson's disease:

  • Lack of tremors or shaking
  • You don’t respond to Parkinson’s treatment
  • Difficulty looking downward

An MRI scan might be requested to determine if specific brain regions have shrunk. The scan can also help distinguish progressive supranuclear palsy from other disorders that mimic its symptoms. In case the MRI did not show any signs of abnormalities, which is possible with the early stages of progressive supranuclear palsy, a PET or positron emission topography may be recommended. 

5 Treatment

 There is still no available cure for progressive supranuclear palsy, but there are treatments you can use to alleviate its symptoms. Treatment options include:

  • Medications for Parkinson’s disease. Drugs to treat Parkinson’s disease increase the levels of dopamine, a brain chemical responsible to maintain control in muscle movements. The effectiveness of these drugs is only limited and temporary.
  • Botox. Botulinum toxin type A or widely known as Botox blocks the chemical signals that result in muscle contraction, thus improving eyelid spasms. Botox is injected into the muscles around the eye area.
  • Bifocal eyeglasses. Using bifocals or glasses with prism lenses may help with eye movement, especially when looking downward. 
  • Speech and swallowing therapies. These will help with your slurring speech as well as learn how to swallow safely.
  • Physical and occupational therapies. These are done to improve balance.

6 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with progressive supranuclear palsy.

To delay the effects of the disease, people with progressive supranuclear palsy can do a few things. These include: 

  • Using eyedrops or artificial tears to ease dry eyes that usually come with the uncontrolled blinking,
  • Installing grab bars around the house, particularly in bathrooms and hallways, to help prevent falls,
  • Using a weighted walker to help avoid falls,
  • Removing rugs and other floor clutters that can’t be seen without looking downward,
  • If possible, avoid climbing stairs.

Living with the disorder can be stressful. To help you cope with stress, you may need your family’s support, keep a circle of loyal friends close, too. A support group where you can talk with others with similar conditions will also be ideal.

7 Risks and Complications

There are several risks and complications associated with progressive supranuclear palsy.

Progressive supranuclear palsy affects those who are around 60 years of age, and there’s virtually no case of the disease on ages 40 and below.

Complications of this disease are usually a result of unstable muscle movements. These may include falls, sleep-related problems, problems in swallowing and speaking, and difficulty focusing the eyes. 

The issue of swallowing may cause aspiration or ingesting food or liquid to the airway, which can possibly develop into pneumonia

8 Related Clinical Trials