Living Frequently Asked Questions

Community run "day programs" can be suitable for patients. Consider these resources: 1. Alzheimer Society Dementia Programs and Services 2. Parkinson Canada Exercise and Wellness Resources Answer. (10/2024)

The SCOPE Program (Seamless Care Optimizing the Patient Experience) at University Health Network (UHN) in Toronto is a patient-centered initiative designed to improve the quality of care, coordination, and overall experience for patients navigating complex medical conditions, particularly those requiring multidisciplinary care. SCOPE Website SCOPE optimizes patient care by providing coordinated and personalized healthcare experiences. It is designed to address the complexities of care for patients with chronic, complex, or multisystem conditions, ensuring that they receive timely, appropriate, and continuous care throughout their treatment journey. SCOPE brings together a multidisciplinary team of healthcare professionals who collaborate to provide comprehensive care. The team may include: Physicians (e.g., specialists and general practitioners) Nurses Care coordinators Social workers Pharmacists Physiotherapists, occupational therapists, and speech-language pathologists Dietitians and other specialists as needed Here’s how you can access the program: Consult your primary care physician or specialist to discuss the need for the SCOPE program and obtain a referral. Contact UHN for information about the program and confirm the referral process. Undergo an assessment by the SCOPE team to determine eligibility. Engage with the care coordination team, which includes communication and support for both patients and caregivers. If you're unsure about the process or need additional help, contacting UHN Patient Relations. They can provide specific instructions on the SCOPE program's availability, eligibility, and enrollment. Answer (11.2024)

PSP has a minor genetic component. One of the genes tested for is microtubule associated protein tau (MAPT). MAPT is most associated with PSP but the illness is not genetic. Answer. (08/2020)

The pathologically of CBD is classified as a tauopathy (accumulation of the tau protein in the brain) Answer. (07/2024)

MSA pathologically classified as a synucleinopathy (accumulation of the protein alpha synuclein) Answer. (07/2024)

1. MSA-P (Parkinsonian): Striatonigral degeneration implies Parkinsonism with some degree of cerebellar dysfunction 2. MSA-A (autonomic): Shy-Drager syndrome reflects a predominance of autonomic failure 3. MSA-C (cerebellar): Olivopontocerebellar atrophy indicates primarily cerebellar defects with minor degrees of parkinsonism Answer (10.2019)

Midbrain atrophy associated with PSP and other atypical PSP variants gives the appearance of the head of a hummingbird, and the pons (part of the brainstem) forms the bird's body. While the hummingbird sign is suggestive of PSP, it is not unique to the disease. Early in the disease, the atrophy may not be pronounced enough to detect the hummingbird sign. Some other neurodegenerative disorders might also mimic the sign. Diagnosis of PSP often combines clinical features, imaging findings, and exclusion of other causes.

We do not know what are the causes of PSP and we do not know why people get PSP. When a person dies, we see abnormalities in the brain that are consistent with PSP pathology. PSP pathologically is classified as a tauopathy (abnormal accumulation of the tau protein in the brain) Answer. (08/2020)

Coming soon. Answer. (07/2024)

PSP is often associated with swallowing problems, especially as the disease progresses. Think about what affects the other muscles, that rigidity and stiffness that you see in the neck and in the trunk and in the limbs, it is also happening in the muscles of swallowing It is very common for people with PSP or CBD to have swallowing problems. We always ask, if there is any coughing with eating or drinking, if there is any regurgitation and something is coming out of the nose. And then if the person says yes, and if it is happening consistently, we send people for a swallowing assessment. A speech language pathologist can come to your home and assess the swallowing. And if they feel like you need a more invasive assessment, they will refer them for a test where they have to drink something and they take X-rays at the same time. Answer. (08/2020)

MRIs look at the brain as it degenerates (shrinks) over time. So, there are those tests. There are no blood tests or checking how your thyroid function is doing. We do not have those kinds of markers yet. The neurological test that we do is really about checking for rigidity, for slowness, we check coordination, imbalance, eye movements, muscle strength, so we check all those things. And yes, we grade them. If you do a test at home like a bicep curl and if you do it once a week for 50 weeks, you are going to see if that person change. So, in that way, yes, you can do it as part of a movement routine. Answer. (08/2020)

In MSA, there is an extreme drop in blood pressure that can occur. When we give some of the medications, we try to counteract that it can actually cause hypertension. Sweats and chattering can happen when your blood pressure drops. What will happen is there will be a reflexive high blood pressure right after that and so, these episodes do go with MSA. Answer. (08/2020)

These are very heterogeneous diseases and not everybody progresses in the same way. The things we look for in the later stages of the disease is the swallowing part. The other one is the immobility. So, in the later stages of the disease people become wheelchair-bound. Sometimes it does happen where people are having falls and it is an earlier stage, and their cognition is perfectly preserved, and they have no swallowing problems. There is heterogeneity but as people have swallowing problems and are confined to a wheelchair, we say that is the final stage. If they start to have pneumonias, then that is what people die from because you cannot treat them over and over again and we think that is the final phase. When people have a lot of immobility where they develop posturing where their arms might be stuck in some position then that lends itself to sores and to infection. That too, is an advanced stage. Answer. (08/2020)

Dystonia is a movement disorder characterized by involuntary muscle contractions that cause abnormal, repetitive movements or postures. It can affect a single muscle, a group of muscles, or the entire body. In PSP it can result in... A backward tilting of the neck Grimacing or expressionless face Limb stiffness and abnormal posturing In CBD it can result in... A hand becomes stiff and difficult to use Voluntary task-related movement difficulty Limb rigidity In MSA it can result in... Forward flexion of the neck Limb rigidity Reduction in ability to perform fine motor tasks Answer (11.2024)

CBD affects different networks. Some people with CBD will have a profound language problems and cannot speak. They know what they want to say but they have trouble putting all the letters and sounds together to get out the right word. They also lose their ability to spell and to write words. And then you think it is just a language network that is affected. But over time, the other networks also get affected. Their memory might start to decline and their visual spatial processing. Some people will develop apraxia. Sometimes it is just speech where you cannot organize all the sounds to get the word out Sometimes it is movement apraxia so you want to brush your teeth and you must pick up a toothbrush, bring it to your mouth, and you have to make this kind of movement. It looks so easy for us but if you have CBD, that motor programming does not work. CBD has a lot of that inability to organize things in the brain properly and causes an inability to effectively communicate what you want the limb to do. In terms of the cognitive it can be quite heterogeneous. It could be language, it could be what we call executive like organizing, being able to pay attention to things. It could be memory, just regular verbal memory, visual memory, and it could be visual processing. It is a very heterogeneous disease and so people present with different patterns. It is one of the reasons it is hard to diagnose, and often gets diagnosed fairly late. Answer. (08/2020)

Consider a quad cane with a sturdy 4 footed bottom. Those with 2 handles are helpful for standing from seated on one's own Answer. (12/2024)

Tablets and other audio devices where patients can trigger audio commands can be helpful. As symptoms progress, however and dexterity and motor function worsen, these become less useful. Caregiver communication books made for non-verbal adults experiencing cognitive decline, aphasia or dementia contain large letters, images and common prompts are a popular tool. Answer. (10/2024)

Prism glasses are specialized lenses designed to shift or bend the visual field by using prisms embedded in or attached to the lenses. They can be prescribed after a thorough evaluation of the patient’s vision and mobility issues or purchased over the counter or online. Prism glasses can potentially help in these ways... 1. Improved Eye Movement Compensation: PSP often causes difficulty in moving the eyes vertically (up or down), known as vertical gaze palsy. Prism glasses can shift the visual field, allowing patients to see objects without requiring significant vertical eye movement. 2. Enhanced Posture and Balance: PSP patients often adopt a forward-leaning posture (due to rigidity and postural instability). Prism glasses can help by redirecting their vision upwards, reducing the need to tilt the head or strain the eyes. 3. Visual Field Correction: Some PSP patients experience visual misalignments or double vision. Prism glasses can realign the visual inputs from each eye to improve clarity and reduce strain. 4. Aid in Daily Activities: By improving visual access to surroundings, prism glasses can help PSP patients navigate their environment more safely and independently. They are limited in these ways... 1. Prism glasses are not a cure for PSP but are a supportive tool. 2. Patients may require an adjustment period to adapt to the altered visual field. 3. Prism strength and design may need to be customized for the individual, ideally by a neuro-ophthalmologist or low-vision specialist for optimal effectiveness. Answer (11.2024)

The Etac Turner Pro is a turn aid that offers safe patient turning with standing support. It enables a good posture to minimize the risk of injury for the carer when transferring.

There is a lot of evidence that aerobic exercise is good for the brain. Aerobic means things that make your heart rate go up. Aerobic exercise releases a molecule called brain derived neurotrophic factor. That molecule does not come in a pill form, it goes up when people exercise. Sometimes patients have a hard time because they do not even have control over the limb, even if they want to move it. So even passive movement is good because in that case, you keep the joints and the range of motion in the joints. Movement, if it is safe is good. Answer. (08/2020)

If you are causing pain, we do not like that. If you are doing some exercises, there can be a little bit of pain as when you exercise and you haven’t done that in a while but if there's constant pain, that should be checked out. Some people will develop what we call dystonia where you have a limb in a certain fixed position and that can be painful. In that case, you can talk to your doctor because sometimes we can do Botox to relieve some of the contraction of the muscle. Trying as much as possible to keep the limb from getting into a position where even any slight movement causes pain is something we should be aiming to do before a person gets to that point. Answer. (08/2020)

Yes, it is very variable. Some people have trouble swallowing liquids/fluids and they have no problems with most foods. Most people have to cut up their food pretty small. Most people cannot eat big chunks of meat and things like that and be okay. As the disease progresses, some people have no trouble if the food is cut up, but if they drink something they are really struggling. In that case, we do an assessment, and we will say, thicken your fluids. Other people really have trouble more with solids. Even if they cut things up small, and are diligent about chewing, they are still having a lot of trouble. Those people we will switch to a minced diet initially, and then if they continue to struggle, put them on a pureed diet. Sometimes it is just a matter of not mixing the solids and liquids at the same time. Swallowing is one of the impairments that progresses and so often people do lose the ability to swallow. Answer. (08/2020)

Repetitive transcranial magnetic stimulation (rTMS) is a non-invasive brain stimulation technique that uses magnetic fields to stimulate nerve cells in the brain. The effectiveness of rTMS in PSP is under investigation. It is not considered a standard treatment for PSP but rather an experimental approach. It targets motor symptoms and aims to improve motor function and reduce symptoms. It stimulates areas involved in cognition and emotion and promotes neuroplasticity, which may help the brain form new neural connections, potentially compensating for the damaged neural pathways in PSP. Answer. (10/2024)

We always have this discussion on what stage this is happening. Because even though you can get rid of all the food going in, and all the liquid going in, you are not going to get rid of people's saliva. Eventually people are choking on their saliva and what are we going to do about that? And so it is really important to have discussions about goals of care and what are we looking to do. Swallowing is a big issue. And sometimes people are at high risk of aspirating, which gives people pneumonia, which is, you know, the way people die from these diseases. Some people really like to have food in their mouths. And so, you think, well, that's for quality of life. It is important to recognize that the swallowing difficulties that happen with PSP are really common, and they can happen at different stages of disease. They are usually towards the later stages. Although it might stretch life out a little bit longer, it is not going to solve the problem because eventually you cannot swallow your saliva. Answer. (08/2020)

If a patient is not safe at home, they should be admitted to long-term care. We encourage people to get on several lists and pick places with reasonable waiting times. Answer. (08/2020)

ClinicalTrials.gov is an effective resource for finding ongoing studies. These links will take you to an up to late list of ongoing trials PSP clinical trials CBD clinical trials MSA clinical trials Answer. (10/2024)

The problem with CBD oil is that there are no good studies. We do know that CBD with the THC can be associated with psychosis and depression and does affect cognitive function and is associated with cognitive impairment. CBD has been shown to be quite good for pain, except that it cost the patient some cognitive points. So, it is a cost benefit scenario. Answer. (08.2020)

Melatonin is benign. Melatonin exists already in our brain and is important for setting your circadian rhythm. Often, we give melatonin to people, especially as a first line for people who have trouble getting to sleep. Everybody can have side effects to vitamins and minerals. So obviously everybody is different. We want to take note of that, but it is usually quite well tolerated. Answer. (08/2020)