Progressive Supranuclear Palsy (PSP) – Symptoms, Causes & Treatment

progressive supranuclear palsy

A complex and uncommon progressive condition that affects the brain, progressive supranuclear palsy (PSP) is a disorder that causes weakness of various muscles.

Supranuclear is the section of the brain above 2 small areas known as nuclei.

This is the region that the disorder primarily affects.

What is Progressive Supranuclear Palsy

PSP is a rare disorder that has varying estimates.

Research shows that about 3-6 people in every 100,000 people globally or about 20,000 Americans have the condition.

The disorder is also believed to be more common in males than in females.

In most cases, it mostly affects people who are in their 60s but it can occur earlier or later. In short, it is very unpredictable.

PSP was first described as a distinctive disorder in 1964 after three scientists published a paper that highlighted the differences of the condition compared to Parkinson’s disease.

For some time, it was known as the Steele-Richardson-Olszewski syndrome the combined names of the scientists who defined the condition.

How PSP is Different from Parkinson’s Disease

how PSP is different from parkinsons disease
Both Parkinson’s disease and progressive supranuclear palsy cause movement difficulties, clumsiness and stiffness.

However, PSP progresses more rapidly when compared to Parkinson’s disease.

Persons with PSP normally stand exceptionally straight or at times title their head backward causing them to fall backward.

It is different for persons with Parkinson’s disease because most people with this disease will bend forward.

Difficulties with swallowing and speech tend to be more pronounced and common in PSP than in Parkinson’s disease.

Causes of Progressive Supranuclear Palsy

causes of PSP
Experts have figured out how PSP happens, but they are yet to pinpoint why it happens.

The disorder develops when brain cells in a section of the brain stem become damaged.

It is still not clear how and why it damages the cells.

NHS reports that PSP happens when brain cells in certain sections of the brain become infected as a result of excessive accumulation of a protein referred to as tau.

Tau naturally occurs in the brain but in this situation, due to the build-up, the body cannot break it down quickly enough.

In persons with PSP, tau reaches high levels forming harmful clumps in the brain cells.

The levels of abnormal tau in the brain, as well as the location of these clumps, usually vary in persons with this progressive condition.

This implies that the disorder may have a broad range of symptoms.

Several areas of the brain are thought to be affected by PSP such as:

  • Brainstem: this is especially the area of the midbrain where “supranuclear” eye movement resides
  • Basal ganglia
  • Cerebral Cortex
  • Spinal cord
  • Dentate nucleus of the cerebellum

Research has also linked the disorder to changes in various genes.

The genetic faults are, however, not inherited and the risk to relatives including siblings or children of a person with PSP is quite low.

Symptoms of Progressive Supranuclear Palsy

symptoms of progressive supranuclear palsy
It is easy to mistake PSP for Parkinson’s disease because the two conditions share plenty of symptoms.

Some of the warning signs for this progressive disorder include:

  • Problems with Movement: The first sign of PSP is usually a problem with balance while a person is walking. Affected persons may start to fall a lot or feel uncomfortable or rigid when walking.
  • Challenges Moving Eyes: Many people with PSP will have a hard time directing their eyes where they want them to go. This is especially for a person who wants to look downwards. Some people will also experience blurred vision while others may have no control over their eyelids where they go through unwanted blinking or inability to open eyes. It is also common for persons with this disorder to hold another person’s gaze.
  • Speech Difficulties
  • Problems with Swallowing
  • Becoming more cranky and forgetful
  • Tremors in the hands
  • Muscle stiffness especially around the neck region
  • Having unusual emotional outbursts like laughing or crying at unexpected times
  • Depression
  • Extreme tiredness
  • Becoming angry for no reason
  • Dementia
  • Changes in behavior like poor judgment and recklessness
  • Slowness of thought and some memory issues
  • Changes in personality such as mood swings, apathy, and irritability
  • Photophobia: a dislike of bright lights
  • Disturbed sleep
  • Loss of interest in pleasurable activities
  • A frightened or surprised facial expression caused by rigid facial muscles
  • Headaches, joint pain, back or neck pain
  • Bladder problems, constipation, and incontinence
  • Face or jaw spasms or jerks

Because PSP is a progressive disease, the symptoms start out subtle and become worse over time.

Diagnosis Process

diagnosis process
A thorough evaluation of symptoms can help with the diagnosis of progressive supranuclear palsy.

PSP is, however, difficult to diagnose especially in its early stages.

Most of the time, this disorder will mimic Parkinson’s disease. PSP can also be misdiagnosed for an inner ear infection because balance is usually affected.

The diagnosis process normally includes ruling out other medical conditions.

Changes in gait and balance issues are two of the clearest symptoms that can identify the brain disorder especially when it is combined with the inability to move or close eyes.

However, for some affected individuals, problems with eye movement may develop in the later stages of the disease.

Doctors conduct several tests and examinations to help with the diagnosis such as:

Neuropsychological Testing

A person suspected to have PSP may also be referred to a neurologist and possibly a psychologist to help with diagnosis. It usually involves going through several tests designed to evaluate the extent of a person’s symptoms and the impact they have on their mental abilities.

The tests look into abilities like processing of visual information like pictures and words, concentration, memory, and understanding language.

Brain Scans

If a person’s PSP symptoms suggest that there is something wrong with the brain, medics will likely request brain scans like:

  • MRI Scan: Radio waves and a strong magnetic field are used to offer detailed images of the inside of the brain. These can also detect abnormal changes to the brain like shrinkage of certain parts of the brain consistent with PSP diagnosis. Experts are coming up with scans that will identify the build-up of tau protein associated with PSP in the brain.
  • DaTscan: this is where a doctor uses a gamma camera to take pictures of the brain.
    PET Scan: it detects radiation given off by a substance which is normally injected beforehand.

The scans are also helpful when it comes to ruling out other possible conditions like strokes or brain tumors.

Treatment Options

treatment options for psp
Currently, there are no procedures or medications that can cure progressive supranuclear palsy.

Nonetheless, there are methods and strategies that can help manage the symptoms and offer a better quality of life.

Parkinson’s Disease Medication

Drugs that are used to treat Parkinson’s disease can be effective in enhancing flexibility and balance. These include levodopa which can be used in conjunction with other drugs where necessary.

Physicians may recommend antidepressants for persons who are suffering from irritability or depression.

Assistive Aids

Individuals who have PSP may benefit from the use of certain aids designed to make their lives easier. These include special glasses that feature prisms that can help to enhance vision.

Weighted tools can also help people with walking problems so that they do not keep falling over. At times, all a person needs is specially designed footwear that reduces the risk of slipping and falling.

When symptoms are advanced, a person may need a feeding tube when swallowing becomes a problem.

This is a tube that goes into a stomach’s abdomen from an opening made in the skin to offer the body the nutrition it needs.

Treating Eye Problems

If a person with PSP has problems controlling their eyelids, a doctor may recommend injections of botulinum toxins like Botox which can help relax muscles around the eyelids. The effects of such injections last for about three months.

Artificial tears and eye drops can be helpful when an individual is experiencing dry eyes by reducing irritation and lubricating them.

Regular Exercise

Regular exercise might help to strengthen muscles while improving posture and preventing stiffening of joints.

Therapies

Certain therapies can help persons with PSP such as:

Physical Therapy

This may help to improve flexibility in some affected individuals. A physiotherapist will offer advice on how a person can make the most of their mobility through exercise without overexerting themselves.

They can also teach proper breathing exercises that a person can practice to reduce the risk of developing aspiration pneumonia which is described as a chest infection that is a result of food particles falling into the lungs.

Language and Speech Therapy

A speech and language therapist can help a person with PSP to improve speech and swallowing issues. The professionals can teach various techniques that help make a person’s voice as clear as possible.

They can also recommend suitable communication devices and aids that may be useful as the disorder progresses. The therapist can collaborate with a dietician to recommend the proper foods to eat as well as various swallowing techniques.

Occupational Therapy

Occupational therapists can offer expert advice on how to increase safety while preventing trips and falls as the affected person goes by their daily activities.

For instance, many people with progressive supranuclear palsy can benefit from installing bars along their baths that help them get them in and out.

The professionals can also help identify potential hazards in the home such as badly secured rugs, poor lighting, and crowded corridors and walkways that can increase the risk of falls.

Final words

It is advisable for an affected person to talk to their doctor about all the symptoms they are experiencing.

This allows the professional to consider the best treatment option. PSP is not a fatal disorder, but it can result in complications like pneumonia which can be life-threatening.

Other complications that can be caused by PSP include suffering serious head injuries or breaking bones as the likelihood of falls increases.

Research is on-going into new treatments of PSP and a better understanding of the rare disorder.

Parkinson’s Disease Dementia (PDD)

parkinson's disease dementia

A majority of people living with Parkinson’s disease (PD) end up with Parkinson’s’ disease dementia a year or so after diagnosis.

The Alzheimer’s Association reports that about 50-80% of people with Parkinson’s disease end up developing dementia.

It is primarily a result of the changes that happen to the brain because of PD affecting both the structure and chemistry of the organ.

Initially, Parkinson’s disease starts by affecting the region in the brain responsible for movement. It leads to a variety of symptoms like shakiness, shuffling, tremors, muscle stiffness, challenges with initiating movement, stooped posture and lack of facial expressions.

After some time, the changes in the brain may also start to affect other parts of the brain. Specifically, the ones that handle various mental functions, which can include memory as well as the ability to reason or pay attention.

Parkinson’s Dementia: Symptoms, Diagnosis, Treatment, and more

Essentially, persons with PD start to experience a decline in good sense and thinking.

The primary changes that happen to the brain because of Parkinson’s’ disease dementia are linked to abnormal microscopic deposits mainly composed of a protein known as alpha-synuclein.

These deposits also go by the name Lewy bodies.

Researchers are still looking into how the brain exactly becomes damaged, leading to dementia. Find out more crucial details about the disease below.

Symptoms

symptoms
Scientists have identified several symptoms that might be an indication that a person has Parkinson’s’ disease dementia, such as:

Any person who has PD and experiences the symptoms above needs to notify their doctor.

This enables the professional to observe the individual for both cognitive and movement changes.

When talking about the symptoms, it is also important to note that the appearance of one cognitive sign does not automatically mean that dementia will develop.

Risk Factors

risk factors
Some risk factors have been identified concerning what can make a person with PD develop dementia-like symptoms.

  • Advanced age (70+) when a person develops PD
  • Sleeping too much during the day
  • More severe movement impairment symptoms when compared to other people with Parkinson’s
  • Cardiovascular disease
  • Experiencing hallucinations before the other dementia symptoms set in
  • History of mild thought impairment
  • Having specific symptoms that result in a person having trouble when they want to take a step or a person who suddenly stops as they are walking

Diagnosis

diagnosis
To date, there is still no specific test that doctors can use to determine whether a person has Parkinson’s’ disease dementia.

However, there are some guidelines that medics use for the diagnosis process that include:

1. The fact that a person already has Parkinson’s disease and has lived with the illness for a year or more before dementia symptoms showed up.

2. A positive diagnosis of Lewy body dementia when the symptoms that relate to the disease either develop first or are accompanied by symptoms that affect movement. Diagnosis may also occur if the movement symptoms affect a person who has been living with Lewy body dementia for a year.

Outcome

outcome
Many changes occur when a person develops Parkinson’s’ disease dementia. A decline in problem-solving, reasoning and thinking are some of the hallmarks of the disease.

The illness can make a person less independent. Progression to advanced stages can also affect concentration, memory, communication, and ability to understand when other people speak.

Treatment

treatment
Sadly, there is still no cure for Parkinson’s disease dementia. Worth noting is that the illness often goes unrecognized, perhaps because diagnosis is not a simple task.

The condition is progressive, where it becomes worse as the illness continues to destroy the cells in the brain.

The rate of progression also varies.

Nonetheless, experts continue to offer recommendations that persons with PD dementia can use to manage the illness mainly aiming at relieving symptoms like:

Use of medication

use of medication
A physician may prescribe drugs like cholinesterase inhibitors that can help with symptoms like sleep disturbances, visual hallucinations, and changes in behavior and thinking.

Individuals with the illness can also take antidepressants to deal with depression.

If you have the illness, you should never take any drug without consulting your doctor to be on the safe side. You should also be careful to avoid taking medications that can escalate cognitive impairment.

Diseases like diabetes, high blood pressure and high cholesterol should also be treated quickly as these are known to affect the brain.

Deep brain stimulation

deep brain stimulation
This is one of the treatment options that is highly contradictory.

A clinical trial by scientists from University College London reported that deep brain stimulation is safe and can be tolerated by persons with Parkinson’s disease dementia.

Different therapies

different therapies
Persons who have PD dementia may also benefit from therapies such as speech, occupational, and physical which can help to improve communication and movement.

Staying physically active and eating right

staying physically active and eating right
Research shows that eating a balanced diet, physical exercise, taking limited alcohol and getting enough sleep are some measures that persons can take to promote brain health.

Closing Thoughts

Even though Parkinson’s’ disease dementia is an illness that has been around for years there is still a lot of information we need to know about it.

Researchers continue to try and figure out its exact causes through numerous studies and clinical trials. This may help to figure out appropriate prevention and treatment measures.

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