Being a rare progressive condition that is not often highlighted or discussed, the diagnosis of Progressive Supranuclear Palsy, can be overwhelming. It is a serious neurodegenerative disease that affects the parts of the brain that control voluntary movement and coordination, leading to symptoms like muscle stiffness, poor balance, and difficulty swallowing. As a rare disease, research on causes and treatments is still ongoing. Here, we offer a quick guide on everything you need to know about Progressive Supranuclear Palsy and how you can treat it.
Understanding Progressive Supranuclear Palsy
Progressive Supranuclear Palsy is a neurodegenerative condition that causes problems with balance, speech, swallowing, and vision. It is a rare disease that affects people over the age of 60 and occurs due to progressive damage in the brain cells that are located just above the nerves, controlling eye movement. Brain damage occurs due to the abnormal build-up of a protein called tau, which cannot be broken down properly by the patient\’s brain. While Progressive Supranuclear Palsy can be linked to certain genes, it is not a hereditary condition.
Symptoms of Progressive Supranuclear Palsy
Progressive Supranuclear Palsy symptoms are very similar to several other known conditions, and it is therefore easy to misdiagnose. The symptoms develop gradually at first, and then progress faster. Some of the common symptoms that patients may notice include:
- Muscle stiffness
- Problems with balance and movement (a heightened tendency to fall)
- Sudden mood changes
- Fatigue
- Slow, slurred speech
- Softened voice
- Difficulty controlling eye and eyelid movement (especially looking downward)
- Blurry or double vision
- Sudden aversion to bright lights
- Difficulties with thought and memory
- Recklessness or poor judgement
- Impulsive acts like crying or laughing without cause
- Trouble swallowing (Dysphagia)
- Insomnia
- Varying between being overtly expressive and expressionless
- Having trouble finding the right word while speaking
Progressive Supranuclear Palsy Stages
As a progressive disease, PSP moves through various stages, each with differing degrees of symptom severity. In general, doctors classify cases into four main stages. They are:
Early Stage
This stage spans through the first year of Progressive Supranuclear Palsy. The patient is ambulant, but displays coordination problems and an occasional tendency to fall. They also experience changes in mood, may display apathy, and feel a reduced need to socialize. Vision problems may arise, with symptoms so mild that the patient may not be diagnosed.
Mid Stage
This stage spans through years two and three of the disease. The patient will still be ambulant, but require aid. Coordination and vision problems exacerbate, and patients may experience Dysphagia. The patient is likely to display apathy and risky behavior and will require regular supervision.
Late Stage
This stage spans through years three to six of the condition. Patients will require intensive care during this time. They will experience significant problems with mobility, coordination, and vision, and become much more withdrawn socially. Other symptoms include incontinence, Dysphagia, sleepiness, and pain.
End Stage
This is the end-of-life stage, and can last six to eight weeks. The patient will lose all or most consciousness and have severe disabilities. They will also be susceptible to acute infection.
Progressive Supranuclear Palsy VS Parkinson’s Disease
Progressive Supranuclear Palsy is often confused with Parkinson’s Disease, which is another neurodegenerative condition. Often, doctors will observe many of the symptoms of PSP in Parkinson’s and vice versa. The two, however, are not the same and require different treatment approaches.
The similarities between PSP and Parkinson’s include:
- Problems with balance and coordination
- Softened or slurred speech
- Problems with thinking and memory
- Muscle stiffness
- Commonly affecting people above the age of 60
The differences between PSP and Parkinson’s are as follows:
- In general, PSP progresses much more quickly and responds less to treatment than Parkinson’s Disease.
- Patients with PSP usually stand straight or bend backwards slightly. However, patients with Parkinson’s tend to lean forward more.
- Speech and swallowing problems appear earlier and are more severe in PSP than in Parkinson’s.
- Patients with Parkinson’s Disease commonly exhibit tremors, which are rare in cases of Progressive Supranuclear Palsy.
- Motor symptoms are more symmetric in PSP, while they typically show up asymmetrically in the early stages of Parkinson’s Disease.
Diagnosing Progressive Supranuclear Palsy
Given the similarity PSP shares with other conditions, including Parkinson’s Disease, it can take a while to get a definitive diagnosis. Typically, doctors will conduct a brain scan (such as MRI or PET) to check for the build-up of the tau protein.They also conduct cognitive tests to assess thinking, learning, and memory abilities. The doctor will usually call in a neurologist with expertise in PSP to ask patients several questions about their symptoms. These questions attempt to discover whether patients are experiencing mood swings, loss of interest in things they once liked, change in their sleeping patterns, whether they have been falling more often, or experiencing muscle stiffness.
Treatment for Progressive Supranuclear Palsy
There is currently no cure for PSP and no way to slow down its progression. However, treatment can keep the symptoms under control and ensure the best quality of life possible. Typically, several therapists and healthcare professionals will work together to create a tailored treatment plan for the patient. The common components of a PSP treatment plan include:
- Physical Therapy: This focuses on improving balance, posture, coordination, and fine motor skills, while also relieving muscle stiffness. The therapist will recommend a variety of stretching and strengthening exercises to target multiple muscle groups. They may also recommend special walking shoes or frames to help the patient move about safely without losing balance.
- Occupational Therapy: This focuses on enabling the patient to do daily tasks like eating and bathing independently. In addition to tailored exercises, this can also involve teaching the patient how to use assistive devices, making recommendations on home safety — such as adding grab rails
- Speech and Language Therapy: This helps to train the muscles in the tongue and mouth for safe chewing and swallowing, as well as clearer verbal articulation.
- Medication: The doctor can prescribe medicines to treat specific Progressive Supranuclear Palsy symptoms, such as muscle stiffness, depression, sleep problems, or fatigue. Some of the medications used by Parkinson’s Disease patients may also help.
- Spectacles: Eyeglasses fitted with prism lenses can help patients with Progressive Supranuclear Palsy to look downward.
- Feeding Tubes: Especially in later stages of the condition, the doctor may prescribe feeding tubes to help with swallowing.
FAQs
- Is PSP worse than Parkinson’s?
While there are several similarities between PSP and Parkinson’s, PSP generally progresses much faster and doesn’t respond as well to treatment as Parkinson’s.
- Is PSP a terminal illness?
While PSP itself is not fatal, it results in complications such as Pneumonia and fall-related injuries that can be fatal.
- Does PSP run in families?
While the genetic cause of Progressive Supranuclear Palsy is unknown in most cases, it can sometimes run in the family.
- Does PSP cause Dementia?
PSP causes multiple cognitive problems, including Dementia and trouble with learning and decision-making.
- How quickly does PSP progress?
Most cases of Progressive Supranuclear Palsy progress over five to seven years from the time of diagnosis.
- Do PSP patients sleep a lot?
A common complaint in PSP is insomnia, or an inability to sleep.
- How long can a person live with Progressive Supranuclear Palsy?
The average life expectancy of a patient with Progressive Supranuclear Palsy is about six to seven years from the diagnosis.
- What is the difference between Parkinson\’s and Progressive Supranuclear
Palsy?
PSP is a Parkinsonian disease — this means that there are several symptoms in common between PSP and Parkinson’s. However, PSP progresses much faster than Parkinson’s. In addition, PSP patients usually don’t exhibit tremors, which Parkinson’s patients nearly always do.
- Is Lewy Body Dementia the same as PSP?
PSP and Lewy Body Dementia are both types of Non-Alzheimer’s Dementia. However, Lewy Bodies are detected in only about a tenth of PSP cases.
- Can PSP cause Strokes?
PSP patients may have strokes, but it is very rare.
- Is PSP similar to ALS?
PSP like ALS is a rare disease which is also progressive. Both diseases gradually weaken the ability to move voluntarily and cause slurring of speech and trouble swallowing.
- What part of the brain is affected by PSP?
PSP affects multiple parts of the brain, including the Substantia Nigra, Basal Ganglia, Pars Reticulata, and Subthalamic Nucleus. All of these affect voluntary movement, coordination, eye movement, and swallowing.
In conclusion, while there is no cure for PSP at present, treatment options can help to alleviate the symptoms and prolong the patient’s lifespan. It is important, therefore, to get a prompt diagnosis and commence suitable treatment so that complications are avoided as much as possible.
