On August 28, 2025, Stanford’s CurePSP Center for Care and Brain Support Network co-hosted a webinar providing an overview of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD).
The terrific speaker was Stanford behavioral neurologist Dr. Niyatee Samudra. She provided clear, in-depth explanations of these two conditions, from their underlying biology to diagnosis and management. While these two “atypical parkinsonian disorders” share some features of Parkinson’s – like slowness of movement – there are important differences.
The webinar concluded with an audience Q&A, moderated by Joanne Riskin, member of Brain Support Network’s local PSP Caregiver Support Group. Audience questions covered treatment considerations, research, and further exploration of the differences in these two disorders.
Dr. Samudra is co-director of Stanford’s CurePSP Center for Care. The Stanford Center is co-directed by the Stanford Memory Disorders Center and Stanford Movement Disorders Center.
Speaker slides – coming soon!
For additional information, check out these links:
Dr. Niyatee mentioned the most common type of PSP, Richardson’s Syndrome, plus four other types, including PSP-parkinsonism. Here’s a short blog post on these five types
Dr. Niyatee noted that the only way to confirm a PSP or CBD diagnosis is via brain donation. In addition, brain donation is the only way to validate future PSP or CBD biomarkers. Learn about brain donation for PSP, CBD, and look-alike disorders.
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Elizabeth Wong with Stanford Parkinson’s Community Outreach attended the webinar and took notes. Here are the “key takeaways” and her detailed notes.
Key takeaways:
PSP & CBD vs. Parkinson’s Disease: Compared to classic Parkinson’s, PSP and CBD typically progress more quickly and do not respond well, if at all, to the standard Parkinson’s medication, levodopa.
Key “Red Flags”: Certain symptoms should raise suspicion for an atypical disorder. The most important red flags include early falls (especially backward falls for PSP) and changes in eye movements (especially difficulty looking up and down).
The Diagnosis Challenge (Syndrome vs. Pathology): The diagnosis is complex because the symptoms a person shows during life (the clinical “syndrome”) do not always match the underlying brain disease found at autopsy (the “pathology”). For example, a person with the symptoms of Corticobasal Syndrome (CBS) might have PSP pathology, or vice versa. A definitive diagnosis is only possible by examining brain tissue.
Treatment is Symptomatic and Team-Based: While there are no cures yet, a multidisciplinary team is crucial for managing symptoms, ensuring safety, and improving quality of life. This team should include physical, occupational, and speech therapists, as well as early involvement with palliative care to help with symptom management and goals of care planning.
Research Offers Hope: The top priority for researchers is developing biomarkers (like a blood test or special scan) to diagnose these diseases earlier and more accurately. This is the essential first step to being able to test new tau-targeted therapies that are currently in clinical trials.
“PSP and CBD Overview”
Speaker: Niyatee Samudra, MD, behavioral neurologist, Stanford
Moderator: Joanne Riskin, Brain Support Network’s PSP Caregiver Support Group
Webinar Hosts:
- CurePSP Center of Care at Stanford (co-directed by Stanford Memory Disorders Center and Stanford Movement Disorders Center)
- Brain Support Network
Webinar Date: August 28, 2025
Summary by: Elizabeth Wong, Stanford Parkinson’s Community Outreach
Introduction to Parkinsonism: The Umbrella Term
Parkinsonism is a broad, umbrella term that describes a collection of movement-related symptoms common to several different conditions. The key features of Parkinsonism include:
- Bradykinesia: Slowness of movements. This is a core feature.
- Tremor: Shaking, often at rest.
- Rigidity: Stiffness in the muscles.
- Other Symptoms: Can also include postural instability (stooped posture, poor balance) and freezing of gait (the feeling of feet being stuck to the floor when trying to walk).
Parkinson’s Disease vs. Atypical Parkinsonian Disorders
Under the umbrella of Parkinsonism, there are two main categories:
1) Parkinson’s Disease (PD):
This is the most common form, often called “idiopathic” or “classic” Parkinson’s. It is a progressive neurological disease that typically progresses slowly in most people. A key feature is that the movement symptoms usually improve significantly with the medication levodopa.
2) Atypical Parkinsonian Disorders:
Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD) fall into this category, along with Multiple System Atrophy (MSA) and Dementia with Lewy Bodies (DLB).
These conditions share some symptoms with PD but have important differences. They typically progress more quickly, with more advanced symptoms appearing within one to three years of diagnosis. The movement symptoms tend to improve less or not at all with levodopa.
“Red Flags” for an Atypical Parkinsonian Disorder
Certain symptoms can act as “red flags” that suggest a person might have an atypical disorder rather than classic Parkinson’s disease. These include:
- Early Falls: Particularly falls that occur early in the disease course and are often directed backward.
- Gaze Palsy: Changes to eye movements, especially vertical gaze (difficulty looking up and down).
- Apraxia: The inability of a body part (hand, arm, leg) to carry out a learned, desired movement, even though the muscle strength is intact; difficulty with completing purposeful, familiar activities.
- Examples: Trouble opening a door, operating a TV remote, or using kitchen tools.
- Poor Response to Levodopa: A lack of significant improvement in motor symptoms after a trial of Parkinson’s medication.
The Underlying Pathology: What is Tau?
Both PSP and CBD are classified as “Four-Repeat (4R) Tauopathies.” A tauopathy is a disease caused by the abnormal accumulation of the tau protein.
Normal Function of Tau: Tau is a protein found mostly in the axons of neurons. Its normal job is to regulate the stability of microtubules, which are like the internal scaffolding or transport systems within a nerve cell.
Pathological Tau: In diseases like PSP and CBD, the tau protein misfolds and clumps together inside brain cells, forming toxic inclusions that lead to cell death. The type of inclusion can help pathologists distinguish between diseases:
- Tufted Astrocytes: A common finding in PSP.
- Astrocytic Plaques: A common finding in CBD.
- Ballooned Neurons: The nerve cells (neurons) themselves can become swollen and appear “ballooned” due to the pathological process in CBD.
- Coiled Bodies: Can be found in both PSP and CBD.
3R vs. 4R Tau: The tau protein exists in six forms (isoforms). Three have three “repeats” (3R Tau) and three have four “repeats” (4R Tau). (The “R” stands for “repeat.”) In 4R tauopathies like PSP and CBD, the clumps are primarily made of the 4R tau protein. This is different from other tauopathies like Pick’s disease (a 3R tauopathy) or Alzheimer’s disease (a mix of 3R and 4R).
Distinguishing Corticobasal Degeneration (CBD) vs. Corticobasal Syndrome (CBS)
These terms are often used interchangeably, but they mean different things. It is a complex but important distinction.
Corticobasal Degeneration (CBD): This refers to the pathological disease itself—the specific pattern of abnormal tau protein deposits and brain cell changes that a pathologist sees under a microscope after death. It is a definitive brain disease diagnosis.
Corticobasal Syndrome (CBS): This refers to the clinical set of symptoms a person exhibits during life. The classic symptoms of CBS include:
- Stiffness, clumsiness, and difficulty with movement, usually asymmetric (starting on one side of the body).
- Abnormal limb posturing or involuntary movements, such as Alien Limb Syndrome (involuntary limb movements combined with a loss of control or sense of ownership over that limb). Alien Limb Syndrome can manifest in several ways:
- Limb Levitation: The arm or leg may float up on its own, with clumsy, uncontrolled movements.|
- Inter-limb Conflict: One limb may actively interfere or conflict with the other (e.g., one hand unbuttoning a shirt while the other tries to button it up). This can be related to a disconnection in the corpus callosum, the part of the brain connecting the two hemispheres.
- Groping and Grabbing: The hand may involuntarily grope for or grab onto objects, which is often a sign of frontal lobe involvement.
- Limb Levitation: The arm or leg may float up on its own, with clumsy, uncontrolled movements.|
- Problems with thinking or language.
Asymmetric Atrophy: A key feature of CBD is often the shrinkage, or atrophy, of the brain in the frontal (front) and parietal (side) regions. In some cases, this atrophy is asymmetric, meaning one hemisphere of the brain is more affected than the other. This asymmetry is not always present.
The Complicated Overlap:
Not everyone with CBS symptoms has CBD pathology. The symptoms of CBS can also be caused by other underlying diseases, including Alzheimer’s disease, PSP, or other frontotemporal dementias.
Not everyone with CBD pathology has CBS symptoms. A person with confirmed CBD pathology at autopsy may have presented in life with a very different set of symptoms, such as those that looked more like PSP (Richardson’s Syndrome), an Alzheimer’s-like memory dementia, or a primary language disorder.
Prevalence and Disease Course
Corticobasal Degeneration (CBD):
- Age of Onset: The typical onset is around 64 years old, with a range of 45 to 77 years.
- Gender: Men and women are affected at equal rates.
- Disease Duration: The mean duration of the disease is about seven years, although there is a significant range and it depends on when the person was diagnosed.
Progressive Supranuclear Palsy (PSP):
- Age of Onset: The onset is very similar to CBD, with some reports suggesting a slightly later start.
- Gender: Men and women are affected at similar rates.
- Disease Duration: The mean duration is also similar to CBD.
Diagnostic Criteria and Early Uncertainty in PSP
Diagnosing PSP, especially early on, is very challenging due to its overlapping symptoms with other conditions. To help standardize the process, neurologists use criteria developed by the Movement Disorder Society:
- The person must be age 40 or older.
- The disease must have a slowly progressive course. This is to rule out acute events like a stroke.
- The diagnosis is then based on the presence of specific symptoms across four key domains: ocular motor dysfunction (eye movements), postural instability (balance), akinesia (slowness/freezing), and cognitive dysfunction.
Levels of Certainty: The criteria allow for different levels of diagnostic certainty based on which symptoms are present. This reflects the reality that it can be very difficult for a physician to be sure of the diagnosis at the beginning of the journey. The level of certainty might be low at first and increase over time as more definitive symptoms emerge.
More Definitive or Typical Symptoms for PSP: The presence of certain key features increases the certainty of a PSP diagnosis. These include:
- Dysfunction of eye movements, particularly the vertical gaze palsy.
- Postural instability, especially frequent falls that are directed backward.
- Gait freezing that develops within the first three years of the disease.
- Specific cognitive changes, such as the non-fluent type of primary progressive aphasia.
By ranking the combination of these symptoms, a neurologist can determine how likely it is that a person meets the clinical criteria for PSP.
Progressive Supranuclear Palsy (PSP)
Pathology: Under a microscope, PSP is characterized by tau inclusions like tufted astrocytes and globose tangles. On an MRI, it is often associated with atrophy (shrinkage) of the midbrain.
Clinical Syndromes: Like CBD, the underlying pathology of PSP can cause a variety of different clinical syndromes.
- Richardson’s Syndrome: This is the most common and “classic” type of PSP. Its key symptoms include:
- Difficulty with eye coordination and focus (especially looking up and down).
- Loss of balance and frequent, often backward, falls.
- Slow or slurred speech.
- Stiffness in facial muscles, leading to a look of surprise or staring.
- Trouble swallowing (dysphagia).
- Neck stiffness.
- Other Syndromes: PSP pathology can also cause symptoms that look more like classic Parkinson’s (PSP-Parkinsonism), corticobasal syndrome (PSP-CBS), primary language problems, or behavioral changes.
Cognitive and Mood Symptoms in PSP and CBD
These non-motor symptoms are often the first to be noticed and can be very challenging.
In PSP:
- Executive Dysfunction: Trouble with planning, problem-solving, and other high-level thinking tasks.
- Apraxia of Speech: Effortful, halting, distorted speech. Can have difficulty with grammar.
- Apathy: A lack of interest or motivation in things that were previously enjoyable.
- Disinhibition / Impulsivity: A change in the ability to inhibit words or responses.
- Depression & Irritability: Very common mood changes.
In CBD:
- Similar executive dysfunction is common.
- Problems with visuospatial function (knowing where things are in space).
- Non-fluent aphasia (language problems) can also occur.
- Hallucinations are generally less common in both PSP and CBD compared to other atypical Parkinsonian conditions like Lewy Body Dementia.
Overlap of PSP and CBD with Behavioral Variant Frontotemporal Dementia (bvFTD)
Another complex area of overlap is how both PSP and CBD can present with the symptoms of Behavioral Variant Frontotemporal Dementia (bvFTD).
What is bvFTD? bvFTD is a clinical syndrome characterized by significant changes in behavior and personality due to the degeneration of the frontal and temporal lobes of the brain. A brain scan shows decreased metabolism in these frontal areas in a person with bvFTD compared to a healthy control.
Diagnostic Criteria for Possible bvFTD: A diagnosis of possible bvFTD requires that a person exhibits three out of the following six core features:
- Disinhibition: Increased impulsivity, such as saying rude or inappropriate things without the usual social “brakes.”
- Apathy: A loss of interest, motivation, and drive.
- Loss of Sympathy or Empathy: A reduced ability to understand or care about the feelings of others.
- Compulsive or Ritualistic Behaviors: Repetitive actions, sometimes in a way that could be harmful.
- Hyperorality or Dietary Changes: A tendency to put more things in the mouth or significant changes in eating habits.
- Dysexecutive Profile: Significant problems with frontal lobe functions like planning and problem-solving, as seen on neuropsychological testing.
Levels of Certainty:
- A diagnosis of probable bvFTD requires meeting the criteria for “possible” bvFTD, plus evidence of significant functional decline and brain imaging that shows atrophy or other changes in the frontal and/or temporal lobes.
- A diagnosis of definite bvFTD requires all of the above, plus either a known genetic mutation that causes bvFTD or pathological evidence from a brain autopsy.
Diagnosis During Life
A definitive diagnosis of PSP or CBD can only be made by examining brain tissue after death. During life, the diagnosis is clinical, based on the pattern of symptoms.
Current Limitations: There is currently no biomarker (like a blood test or specific scan) that can definitively tell us if a living person has PSP or CBD pathology. The significant overlap in symptoms makes it very difficult to be certain.
What We Can Do Now:
- Rule Out Alzheimer’s Disease: Since Alzheimer’s can sometimes cause CBS symptoms, we can use established Alzheimer’s biomarkers (amyloid PET scans, spinal fluid tests) to see if it is the underlying cause.
- FDG-PET Scan: This scan looks at sugar metabolism in the brain and can show patterns of decreased activity that are suggestive of PSP or CBD, but it is not definitive.
- Neurofilament Light Chain (NfL): This is a blood or spinal fluid marker of general neurodegeneration. It can be elevated in PSP and CBD but is not specific enough to make a diagnosis on its own.
- DaTscan: This imaging scan can confirm that there is a problem in the brain’s dopamine system, which confirms a Parkinsonian syndrome. However, it cannot differentiate between Parkinson’s disease, PSP, CBD, or MSA.
Treatment and Management
Disease-Modifying Therapies: At present, there are no available treatments that can slow down or stop the progression of PSP or CBD.
Symptomatic Treatment:
- Levodopa: A trial of this Parkinson’s medication is often given. A lack of response helps to differentiate these conditions from classic PD.
- Antidepressants: Often used to treat the mood symptoms of anxiety, depression, and sometimes apathy.
The Multidisciplinary Team: This is the cornerstone of management. The team should include:
- Neurologist (Movement and/or Memory Specialist)
- Primary Care Provider (to manage other health issues)
- Physical Therapy (PT): To implement fall prevention strategies and work on mobility.
- Occupational Therapy (OT): To provide assistive devices and suggest environmental modifications to improve safety and independence.
- Speech-Language Pathology (ST): To help with speech changes and critically, to manage swallowing difficulties.
- Nutrition support for dysphagia.
- Palliative Care: To help define goals of care, manage complex symptoms, and provide support for patients and families. Early involvement is highly beneficial.
Prognosis and Advanced Disease
It’s important to have early conversations about advanced care planning and goals of care. Certain symptoms can predict a more rapid progression:
- Older age at disease onset.
- Early falls.
- Early cognitive symptoms.
- Early swallowing difficulties (dysphagia), which can lead to complications like aspiration pneumonia.
- Early eye movement abnormalities.
Research and Future Directions
The field is working intensely to find better ways to diagnose and treat these diseases.
Biomarker Development: This is the number one priority. The goal is to develop tests (blood, spinal fluid, or imaging) that can detect the specific tau pathology of PSP and CBD early in life, even before major clinical symptoms appear. Pathological brain changes of these diseases happen long before clinical symptoms appear. If we rely only on a person’s symptoms for diagnosis, a lot of damage has already occurred. The goal is to develop biomarkers that can “capture” patients in this preclinical stage, even before they have symptoms.
Tau-Targeted Therapies: Many clinical trials are underway for drugs that target the abnormal tau protein in various ways, such as:
- Reducing tau production.
- Inhibiting the aggregation (clumping) of tau.
- Helping to clear abnormal tau from the brain.
- Using immune therapies to target tau.
Improving Diversity in Clinical Trials: Diversity is critical for understanding what types of therapies will work for whom, as there may be different mechanisms or considerations relevant to different groups. Ensuring a diverse participant pool is essential to conducting the best and most applicable science.
Innovative Trial Design: Researchers are exploring more creative and efficient clinical trial designs, such as “basket trials” (testing multiple drugs in one disease population) or “umbrella trials” (testing one drug across multiple related diseases), to speed up the process of finding what works.
Question-and-Answer Session
Q: Are you familiar with advertising for honey combinations to treat dementia and Alzheimer’s, claiming it removes cadmium from the brain?
A: No, I’m not familiar with that at all. I would have to learn more about it. I am not aware of a mechanism where cadmium is specifically involved in either of these conditions. I would want to see more data before commenting further.
[Editor’s Note: When a substance is described as a treatment (or cure) for multiple conditions, this is a red flag for medical misinformation or quackery. Also, if a substance such as honey was helpful as a treatment for dementia, wouldn’t everyone know about it?]
Q: Are diminished language abilities common in both PSP and CBD?
A: Yes, it is relatively common in both, though not every patient will experience it, especially early on. The types of changes I see are fewer words, mispronouncing words, and inconsistent-sounding speech (apraxia of speech). A decrease in voice volume (hypophonia) is also true and can be seen in many Parkinsonian conditions.
Q: Can you have PSP and Parkinson’s disease at the same time?
A: This is a good and complicated question. It is possible for a person’s brain to have more than one type of pathology contributing to their symptoms. You can have PSP pathology and also have alpha-synuclein-related changes (which are seen in Parkinson’s disease) in your brain as well. This can be confusing because a test for alpha-synuclein might be positive, but the main driver of the person’s symptoms could still be the PSP pathology.
Q: Is impulsivity common and how dangerous is it?
A: Yes, impulsivity is common, especially over time, and it can make it hard to keep a person safe. Someone might not recognize an unsafe situation, like an uneven step, and fall as a result. They may also be impulsive in what they say, or have trouble controlling bodily functions. They may not have the “brakes” that others have on certain actions, so having another person around for supervision is very important for safety.
Q: Is there real value for the family in getting a specific diagnosis of PSP vs. CBD during life?
A: In many cases, we can’t know the distinction for sure during life because the symptoms can overlap so much. Right now, the main reason it would matter is for clinical trials, as researchers are trying to develop therapies that are specific to one or both of these conditions. In terms of the treatments that are currently available today, it may not be critical to know the difference.
Q: What is the best way to learn about clinical trials?
A: The best resources are ClinicalTrials.gov, organizations like CurePSP, and talking to your neurologist, especially if they are at an academic medical center. For a clinical trial, you typically have to be able to travel to the trial site for multiple screening and study visits, which can be a challenge for those living far away or in another country.
Q: Can you talk more about the eye movements in PSP?
A: The eye movement changes are very specific for PSP. The earliest and most common is a vertical gaze palsy, which is difficulty looking up and down, particularly looking down. Other issues can include lid apraxia (difficulty opening the eyelids) and even dry eyes, which may be due to a reduced blink rate.
Q: Is Deep Brain Stimulation (DBS) being studied for PSP and CBD?
A: There have been a few case reports for DBS in PSP, but overall, studies have not shown a benefit. In Parkinson’s disease, DBS candidates cannot have significant cognitive changes, which is often a challenge for patients with atypical Parkinsonism as those changes can occur early. I don’t know if DBS will be as effective for CBD and PSP as it is for Parkinson’s.
Q: My husband with CBD gets confused when he sits up from lying down. Could this be a drop in blood pressure?
A: It is a little unusual for blood pressure to be significantly affected in CBD. Conditions associated with the alpha-synuclein protein (like MSA, Lewy Body Disease, or Parkinson’s) are more likely to have this drop in blood pressure (orthostatic hypotension). It could also be possible that medications are causing blood pressure changes. It is a good thing to bring up with his doctor for a full assessment.
Q: You also mentioned “subcortical dementia.” Is this the same as other kinds of dementia, versus what you call Alzheimer’s?
A: I think “subcortical dementia” is a bit of an older term, and I regret including it in my presentation. However, the general concept it conveys is that the problem originates in the frontal parts of the brain and is associated with slowness. This is different from a classic “cortical dementia” like Alzheimer’s disease, where the most prominent feature is a profound difficulty with memory. In Alzheimer’s, a person may seem to learn information in the short term, but later they are unable to remember it at all. In contrast, with a “subcortical” pattern, the person may be able to recall information if given enough time and cues or reminders. The information may not be completely lost, but it’s harder to access.
Q: How common is depression with these diseases?
A: I think depression is so common it would probably be almost universal if we asked about it more. It is common in all neurological diseases. If it can be treated with antidepressants, that is an amazing gift to a person’s quality of life.
Q: How does impulsivity actually present itself?
A: It can present in several different ways. Physically, it might look like taking a step where it is not safe, causing a trip or fall, because the person with the condition might perceive the situation as safe when it is not. Verbally, especially in someone with a behavioral variant frontotemporal dementia presentation, it can manifest as a reduced ability to control what they say, leading them to say things that others might think are mean or rude. It can also involve trouble controlling bodily functions in socially inappropriate situations. Essentially, they may not have the “brakes” that others have on certain actions.
Q: How common is anger in PSP and CBD? Does one of the diseases have it more than the other?
A: Anger can be relatively common in both conditions. It’s not clear if one disease has more anger than the other. Anger and irritability often coexist and can be related to other behavioral changes, such as a decrease in empathy or increased impatience, particularly in those who have a behavioral variant frontotemporal dementia presentation. It also ties into the issue of impulsivity.
Q: A single incident of alien limb happening—is that significant if it’s just happened once, or can you expect it to continue?
A: That’s an interesting question. It’s hard to answer definitively. It’s important to clarify if it was a single episode on a single day or if it has happened only one time ever. Sometimes, alien limb can be caused by other things like strokes or even a brain tumor, so it’s not specific only to degenerative disorders. You would want to make sure other causes have been ruled out. In terms of recurrence, in most of my patients who have alien limb, I do see that it does recur. It can wax and wane—there are times when it seems to go down—but I don’t always know how to predict that.
Q: Is PSP inherited? Can my children get it?
A: There are rare cases where genes are involved in PSP, such as a mutation in the MAPT gene. This is not a common situation. The best way to explore this is to meet with a genetic counselor, who can review your family history and discuss whether genetic testing makes sense.
Q: Stem cell therapy – does that help?
A: I believe there is some research happening with stem cells in progressive supranuclear palsy, but I don’t know the current status of that research right now. I do know that it has been looked into, but I am not sure at what point it is in human clinical trials.
Q: Do we know what actually triggers the tau protein to become abnormal and cause PSP?
A: No, in most cases, we do not understand the specific trigger. In some rare instances, the cause is genetic, such as a mutation in the MAPT gene. Beyond that, the trigger is unknown. It’s possible that there is a general degenerative process occurring, especially since people can have co-existing pathologies like Alzheimer’s disease. There may be environmental factors involved, but at this time, no specific environmental triggers have been identified.
Q: Is misdiagnosis of PSP or CBD common, and are these conditions often underdiagnosed?
A: Yes, to both. The difficulty is that the symptoms can be subtle and easily confused with other conditions. In a classic case, like Richardson’s Syndrome with its typical eye movement abnormalities and backward falls, a diagnosis of PSP might be made more quickly, but even then, there can be a delay. However, if the main early symptom is something less specific, like executive dysfunction, it can be very hard to diagnose. These diagnostic delays and misdiagnoses also depend on the familiarity of the physician with these relatively rare conditions.
Q: How is sleep impacted for patients with PSP?
A: Sleep is very much affected in profound ways, largely due to brainstem dysfunction caused by the disease. This can lead to several issues:
- Sleep Apnea: It is common for people with PSP to have sleep apnea.
- Sleep Fragmentation: The brainstem dysfunction causes sleep to be very fragmented and broken up.
- Difficulty Initiating Sleep: Some research has suggested that it is harder for patients with PSP to get to sleep. This may be a distinguishing feature from other Parkinsonian conditions like Lewy Body Disease, which can be associated with excessive sleepiness.
Q: My husband has PSP and falls. Some months he has no falls, and some months he falls a lot. Is it common for falls to happen in spurts like that?
A: That’s so interesting. Yes, I do think that kind of variability is common, both in things like falls and in symptoms like impulsivity. I would want to play detective a little bit in that situation and think, “Is there something different about these months? What was going on? Is the environment different at all?” But yes, I have seen that happen.
Q: Do you have any particular message or advice for caregivers?
A: I just really want to tell them to take care of themselves as much as humanly possible. This is so hard, and everything that you do is amazing, like, truly superhuman. It’s a very hard job, and it’s hard to maintain that sense of self. Find ways, if it’s possible through a family member or a paid caregiver, to take some time for yourself.
Q: When is the right time for hospice to come in?
A: In theory, hospice is best looked at when a physician thinks there are six months remaining for a person to live. In neurodegenerative conditions, it’s very challenging to know when that six-month mark is present. That’s why being connected with an outpatient palliative care clinic can be very helpful, because they can continue to reassess and help determine if a person fits that stage.
Q: What is the leading cause of death in these conditions?
A: Because of the neurological changes that affect the brainstem and the ability to swallow, probably a leading cause of death is pneumonia from aspiration (food or liquid going down the wrong way). Infection, in general, is the most likely way death occurs.
Q: Is there any research being done with marijuana?
A: I am not sure about marijuana and PSP. I know it is being looked at for agitation in Alzheimer’s disease, but I’ll have to look to see if there’s anything specific to PSP.
Q: What can an occupational therapist (OT) offer?
A: Because these conditions are not expected to improve with therapy, the main role of an OT is to help with safety and to help the caregiver better assist the patient. This can include a home safety evaluation to reduce fall risk, recommending assistive devices, and finding ways to accommodate the situation.
Q: Are there stages of PSP?
A: Yes, there are stages. A significant milestone that marks a stage change is the transition from being ambulatory (walking) to being non-ambulatory and needing a wheelchair. That represents a significant loss of independence. The length of each stage is very specific to the person.
[Editor’s Note: Here are two Brain Support Network blog posts on stages or phases:
PSP Stages/Phases, by two caregivers (on the Johns Hopkins PSP Listserv)
Four Stages of PSP (PSP Association, UK)
Q: Why do people with PSP or CBD need reminders? Are they capable of learning?
A: It is harder for people with PSP to learn. In some cases, rather than constantly reminding them, it might be easier to find ways to simplify the task so it can be done more easily, perhaps even without their input.
Q: My family member has hallucinations. Is this part of PSP or CBD?
A: Hallucinations are not common in PSP and CBD. They are more commonly seen in the synuclein conditions, like Lewy Body Disease. If hallucinations are present, it might suggest there is another reason or co-existing condition.
Q: Can PSP and CBD coexist?
A: Typically, a person would not have both pathologies. However, what can happen is that the pathology of PSP can cause the clinical syndrome that looks like CBS, and the pathology of CBD can cause the clinical syndrome that looks like PSP.
Q: Is there any treatment for swallowing problems?
A: Unfortunately, there is no cure for the swallowing problem itself. A speech therapist should evaluate the person to make recommendations about modified food and liquid textures. They can also teach universal swallow precautions, like a “chin tuck,” which can be helpful.
Q: Is there any way to prevent falls?
A: It is very hard to prevent falls entirely, as they are a hallmark of the condition. One of the biggest and most important things is supervision. Having someone around to ensure the person is safe is critical. When a person is more prone to falls, it can be a good idea to bring things closer to the ground, like moving a mattress to the floor instead of having a high bed.
[Editor’s Note: Here’s a short publication from 2015, but not outdated, on addressing falls and swallowing problems in PSP ]
Q: My family member acts out her dreams. Is this part of PSP/CBD?
A: Acting out dreams (REM Sleep Behavior Disorder or RBD) is not typical for PSP or CBD. It is much more commonly seen in the synuclein conditions (Parkinson’s, LBD, MSA) and would be considered atypical for a tauopathy.
Q: Is apraxia of speech common, and can you explain what it is?
A: Apraxia of speech can be seen in these conditions. It is a hard concept to communicate, but essentially, apraxia is a difficulty with carrying out learned movements that a person was previously able to do. There are many types, including apraxia of limb movement (e.g., trouble throwing a ball) or apraxia of eye movements (trouble directing gaze, which is seen in PSP).
Apraxia of speech, specifically, is when the speech sounds inconsistent and doesn’t flow smoothly in terms of pitch or articulation from one sound to the next. It can be strongly associated with these 4R Tau syndromes (PSP and CBD). Sometimes, it can be the very first symptom a person has (Primary Progressive Apraxia of Speech), which then evolves into one of these broader syndromes over time.
Q: What causes falling in terms of the brain mechanisms?
A: Gait and balance involve the brainstem and the basal ganglia, both of which are impacted in these conditions. In addition, falls can happen because of frontal lobe issues, such as impulsivity.
Q: Can you talk about facility placement?
A: Facility placement is a hard discussion, but there are times when it is really challenging to take care of someone at home. A person can progress to needing this level of care more quickly in PSP as compared to other conditions like Alzheimer’s disease. It is something to think about and to connect with a social worker about, if possible, early on in the process.
Q: What can be done for photosensitivity (sensitivity to light)?
A: Treatment is mostly about modification. Things like dark sunglasses and hats are very helpful. Avoiding bright environments, closing blinds at home, and changing the brightness on computer or tech devices can also be good. Unfortunately, there isn’t a direct medical treatment for photosensitivity itself.