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Atypical parkinsonisms are defined as neurodegenerative conditions that are not Parkinson’s disease.

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are neurodegenerative disorders that can mimic Parkinson’s disease in the early stages, but have their own unique features, symptoms and treatment options. Both PSP and MSA are uncommon, each affecting five or six individuals out of every 100,000 people, or about 3,300 to 4,000 Canadians in total. Parkinson’s disease affects more than 100,000 Canadians.

As part of Parkinson Canada’s 2018 Expert Webinar Series, Dr. Connie Marras gave an online presentation in November, on the signs, symptoms and treatments of PSP and MSA, including an update on current research, leading to hope for the future. Dr. Marras is an associate professor of neurology at the University of Toronto and a neurologist at the Toronto Western Hospital Movement Disorders Centre.

Dr. Marras began the webinar by defining the term parkinsonism as a syndrome of abnormal movement, characterized by tremor at rest, slowness, rigidity and impaired balance.

“Although an individual may not have all of these symptoms, parkinsonism is a ‘placeholder’ term, used until further investigation brings results in a diagnosis,” Dr. Marras said.

The majority of cases of parkinsonism will have Parkinson’s disease. Some cases will have movements impaired by stroke, medications, or other conditions. Other cases of parkinsonism will have PSP, MSA or other atypical parkinsonism.

How do PSP and MSA differ from one another and from Parkinson’s disease? In the webinar, Dr. Marras explains how they involve different areas of the brain. All three conditions involve the basal ganglia, the area of the brain affecting movement. In MSA, additional areas of the brain are affected, including the cerebellum, affecting balance and coordination, and the brain stem, affecting autonomic functions, such as blood pressure and bladder function. In PSP, the upper part of the brain, the cortex, is affected, impacting cognitive functions and behaviour. It does not impact the area of the brain affecting memory.

At the microscopic level, the biological processes of the two diseases differ by involving two different proteins. MSA involves the accumulation of the protein alpha-synuclein, while PSP involves the accumulation of the protein tau.

“So, there are fundamental differences between these conditions, despite some of their similarities,” said Dr. Marras.

As the diseases progress, different symptoms become evident, reflecting the different areas of the brain affected. PSP symptoms can include a prominent loss (especially downward) of eye movement cognitive impairment and impulsive behaviour. MSA symptoms can include low blood pressure with standing/sitting, poor urine control, impotence, a nighttime breathing disorder and coordination difficulties.

Symptoms and treatments

Symptoms and the current treatments for PSP and MSA can also differ between the two diseases and from the symptoms and treatments for Parkinson’s disease.

Balance difficulty

Balance difficulty may be more dangerous in people with PSP due to impulsivity.

“Someone may suddenly decide they need to get something across the room and then get up quickly and start across the room and then fall. They cannot process that they need to wait for assistance to do this,” says Dr. Marras, “making for a very challenging caregiver situation.” It means constant supervision may be necessary. Cognitive impairment means they may not have insight into their condition.

Balance can also be an issue in MSA. Unfortunately, levodopa is not as effective in these conditions as it is for Parkinson’s disease. Treatment usually consists of physical therapy and occupational therapy to maintain safety and optimize mobility. Physical protection such as hip and knee protectors can be helpful.

“One man even wore the padded headband used by curling players to protect his head,” reports Dr. Marras.

Eventually, walkers and wheelchairs are important mobility aids. Although they are often regarded negatively as signs of disease progression, they do help people move more safely and for longer distances.

Swallowing difficulties

Swallowing difficulties are also common to both PSA and MSA. It can be the most dangerous symptom, resulting in pneumonia and choking episodes that can be fatal. The loss of coordination or slowness of the swallowing muscles may allow food, liquids and saliva to enter the airway instead of being directed down the swallowing tube. Particles in the airways may result in infections and pneumonia.

Dr. Marras recommends an assessment by a speech-language pathologist to help identify and manage this symptom.

The decision to have a feeding tube, or not, often comes up in the later stages of this disease, when caregivers are concerned about nutrition and feeding by mouth becomes very slow or difficult. Dr. Marras recommends having a conversation with the person with the disease about the pros and cons of a feeding tube, in advance of needing it, while they are still able to make such a decision and communicate it.

Speech impairment

Speech impairment is common in both conditions, with it being more so in PSP. Speech can become slurred and soft, or even inaudible. Medications are not very helpful and treatment usually consists of speech therapy, which can be costly. A self-guided exercise program is available online. You can find it by googling LSVT Loud Homework Helper. It does require the person to be cognitively capable and motivated to practice. If speech is more impaired, other communication aids can be used, such as tablets where you can point to commands or picture books with familiar requests. Hospital-based communication clinics with experience in augmentative and alternative communication can help individuals use these tools.

Behaviour changes and cognitive impairment with PSP

Behaviour changes and cognitive impairment are often symptoms of PSP. Inappropriate laughter or crying, or other behaviours, especially if they become disruptive, can be treated with various medications. But the impulsivity of PSP can be difficult to manage and may require constant supervision.

Executive dysfunction can be addressed by minimizing multitasking and emphasizing routine. Activities, such as day programs, can be beneficial for both the person with PSP and the caregiver, offering stimulation and respite, respectively.

Impaired eye movements with PSP

Progressive loss of eye movement, particularly downward, is a common symptom of PSP. This symptom makes it difficult for an individual to see food on a plate, or to walk down stairs. Managing this symptom can be as simple as raising objects closer to eye level. If the eyes are not coordinated, double vision can result. This can be resolved by covering one eye.

Breathing difficulty with MSA

There can be an involuntary closure of the vocal cords leading to a high-pitched sound, usually made during sleep for people with MSA. You should report this to your doctor, who will refer you for a sleep assessment.

Poor bladder control with MSA

Urinary incontinence (accidents) and urinary retention can be symptoms of MSA. Timed trips to the bathroom and protective garments may help with incontinence and there are some helpful medications. When urine is retained in the bladder, infections may occur. A medication review may help identify any drugs you are taking that may increase urinary retention. You may also need a catheter to empty your bladder, either intermittently or permanently.

Research

Dr. Marras sees two benefits to understanding recent research into PSP and MSA: Research offers hope for the future and it can be empowering to people who become involved in related research studies.

According to Dr. Marras, one of the most exciting areas of current PSP research involve anti-tau antibodies. Two North American studies now entering clinical trials will assess whether an anti-tau antibody medication can slow the progress of the disease by inhibiting the accumulation of the protein tau.

In France, a study with 160 participants is looking into the quality of life for people with PSP, as well as for their caregivers. “I was very glad to see this study underway, looking into the experience of the caregivers as well. It is very important to understand how we can support them too,” says Dr. Marras.

There are several studies underway looking at ways to use imaging to identify PSP and MSA and distinguish them from Parkinson’s disease earlier.

More information about PSP and MSA can be found on the Parkinson Canada website at www.parkinson.ca. You can view Dr. Marras’ webinar and hear the question and answer session, at your convenience, on the Parkinson Canada YouTube channel. Visit the Knowledge Network to access future webinars and past recordings.

If you are interested in keeping track of current research on PSP and MSA, or would consider participating is such research, Dr. Marras recommends visiting clinicaltrials.gov for more information. You can also find information about clinical trials and those seeking participants on Parkinson Canada’s website.