Tag Archive | forgetfulness

The Rare Dementias: Progressive Supranuclear Palsy

This post will begin a continuing series of posts that looks at rare types of dementia. Today’s post will look at the dementia of Progressive Supranuclear Palsy.

Since this blog is primarily original and unique content that provides real education and real resources and understandable information that you can actually use (you’ve no doubt noticed that, unlike many of the blogs that purport to “talk” about Alzheimer’s Disease and dementias, Going Gentle Into That Good Night does not flood your email each day with many, many blog posts that are either old news, political advocacy, or simply forwarded items from some other source, but instead does the medical and scientific research and translates that into usable and practical information for you), I  will provide a clarification that I often do here.

Too many times, I hear the term “Alzheimer’s Disease” used to characterize any and all neurological damage, decline, and death.

That characterization is wrong. 

Dementias refer to the full category of unique terminal neurological diseases that result in irreparable damage and destruction in the brain that lead to cognitive impairment, cognitive decline, and are always ultimately fatal. 

Alzheimer’s Disease is one type of dementia. However, not everyone who suffers from dementia has Alzheimer’s Disease. And sometimes people who suffer with dementia have more than one type of dementia happening concurrently (these are called mixed dementia diagnoses). 

I’ve had people who’ve never dealt directly with dementias kind of wave me off with a “whatever!” reaction when I correct them on this. And I understand that if someone hasn’t had to deal with these dementias day in and day out, they really don’t know and it doesn’t seem like a big deal.

However, for those of us who are dealing with it day and day out – or who may be in the future dealing with it day in and day out – it’s a really big deal.

Why? Precision in defining and understanding the types of dementia is absolutely critical both in caring for our loved ones, but also in effectively addressing the symptoms and behaviors associated with each dementia.

I’ll explain this in an analogy.

Let’s say you or I got diagnosed today with hypertension (high blood pressure). The normal pharmacological treatment is a medication to lower the blood pressure and possibly statins or beta channel blockers to supplement the efficacy of the main medication.

Your physician gives you the diagnosis of hypertension followed by the statement “it’s about the same thing as cancer,” and then sends you to the local hospital for radiation treatment, to be followed by chemotherapy.

Absurd, right? And yet while this may be obviously absurd to nearly everybody, a lot of those people don’t make the connection to the same absurdity of classifying all dementias as a single type of dementia.

But this underscores why it’s just as important to know about the different types of dementias and how to address and respond to the unique areas of the brain they affect as it is to know that you don’t treat hypertension with radiation and chemotherapy.

I have full-information posts on this blog about some of the more common types of dementias and symptoms/behavioral treatments:

And that’s why I want to cover some of the rarer types of dementia now. There’s no substitute for education.

We’re all, if we haven’t already been, going to be personally effected by these diseases at some point in our lives.

We can either be knowledgeable or we can spend time that we should be focusing on our loved ones playing catch-up because we didn’t take advantage of this opportunity to learn about these neurological diseases now.

Progressive supranuclear palsy (also known as Steele-Richardson-Olszewsky syndrome) is a rare neurological disease that, over time, irreversibly damages neurological cells, primarily in the brain stem, causing a severe degradation of gait, balance, complex eye movements, and thinking.

Because gait and balance are primary symptoms of progressive supranuclear palsy, this neurological disease is often misdiagnosed as Parkinson’s Disease. However, progressive supranuclear palsy has unique characteristics that differentiate it from Parkinson’s Disease.

brain-stem-progressive-supranuclear-palsy

The brain stem handles many of the critical functions of the body, including eye and mouth movement, balance, breathing, consciousness, swallowing, sensitivity to external conditions (heat, cold, pain, pleasure, etc.), and hunger. 

The earliest symptoms to emerge with progressive supranuclear palsy are with gait and balance, leading to unexplained falls. Frequently the falls are described by those experiencing them as sudden attacks of dizziness.

Other early symptoms include marked personality changes, including apathy, increased irritability, increased aggravation, inexplicable angry outbursts, and increased forgetfulness.

As progressive supranuclear palsy advances, the characteristics that distinguish it from Parkinson’s Disease begin to appear in vision problems.

The first two vision problems to materialize are blurry vision and difficulty controlling eye movement. It is usually when these symptoms appear that progressive supranuclear palsy can be accurately diagnosed.

People suffering with this neurological disease will, by this point in the disease process, experience severe difficulty voluntarily shifting their eyes to a downward gaze and in controlling their eyelid movements. 

This lack of control over eye movement can result in exaggerated or infrequent blinking (infrequent blinking can cause serious damage to the eyes including severe dryness and ulcers on the surface of the eye) and not being able to close the eyelids or to open the eyelids.

As these symptoms appear, other symptoms also emerge. These include increasingly slurred speech and frequent difficulty in swallowing solid food and liquids (dysphagia).

There are currently no definitive treatments for progressive supranuclear palsy.

There is some research that has shown botulinum injections (Botox) can be an effective treatment for the involuntary eye movements associated with this neurological disease. However, Botox carries its own risks and the benefits of using this as a potential therapy must be weighed against those.

The normal treatment for gait and balance problems – medication and physical therapy – have, to-date, proven ineffective. Therefore, sufferers of progressive supranuclear palsy must use weighted walkers and must have human assistance when walking. Eventually, the problems become severe enough and safety becomes a high enough concern that people suffering from progressive supranuclear palsy are confined to wheelchairs.

The symptom of dysphagia is the most dangerous and the most likely to have a fatal outcome. This is because of choking and the high likelihood of aspirating food back into the lungs. Aspiration often leads to pneumonia, and in this class of neurological diseases, pneumonia is very often fatal.

The only way to address this aspect of progressive supranuclear palsy is to change to the texture of the food. The National Dysphagia Diet Levels is the standard progressive list used to determine food consistency levels as dysphagia worsens.

However, at some point, all ability to swallow anything will be lost, so even if pneumonia is avoided or successfully treated, the time will come when people suffering from progressive supranuclear palsy will not be able to take any kind of nourishment or hydration. This, then, would be the other eventual cause of death from this neurological disease.

Gracious Goodbyes – Missed Connections

I don’t think any of us who’ve been through this journey through dementias and Alzheimer’s Disease with our loved ones doesn’t have events like “Gracious Goodbyes – Missed Connections” describes.

Somewhere back in the farthest corners of our minds, when even the simplest things escape our short-term memories (I’ve had so many of these lately when I have not been, when asked a direct question, in a sort of on-the-spot situation, that I knew the answer to like the back of my hand, able to find the answer – I suspect rationally that it is stress and overload, but there’s always that nagging fear that this is the beginning of a journey I’ve already been on and don’t want to go on again), we wonder if this the beginning of our own journeys into dementias and Alzheimer’s Disease.

Ironically, I am not related biologically to my mom, who suffered from vascular dementia, Lewy Body dementia, and Alzheimer’s Disease. So, based on biology and logic, I shouldn’t worry. However, I know so little of my own biological background and medical history that I realize everything in this arena is totally up in the air.

On the one hand, I don’t know that I really care, in the big scheme of things. After all, I’m human. Therefore, by default, I’m terminal. The unknowns are when, how, why, what, and where. My hope (and prayer) has always been quick, soon, and with as little fuss and muss as possible.

I don’t want anyone to have to execute my DNR and my living will, both of which give me the quickest exit possible from this physical life. I’d rather God just step in, end my life in a flash, without leaving the agony of honoring my wishes to those that would have to make those decisions. I know they would, but I’d rather spare them the pain of having to do it.

But I also do not want my loved ones to have to go through the prolonged process of me dying the slow death, first mentally, then physically, that dementias and Alzheimer’s Disease bring. So, in that sense, I do care. Not for myself or for my life physically, but for those who would have to deal with these diseases if they come.

So, in the back of my mind, I don’t worry so much as I pray that I’m spared this particular way of exiting physical life. Each time I forget something I know I know, when I’m put on the spot to remember it, brings a twinge of anxiety, a moment of wondering, a slightly deeper intake of breath for what might be, what could be, but what I hope and pray will never be for me or for my loved ones.