The Unholy Trinity

There are three components of neurodegenerative diseases, namely the physical, cognitive, and emotional. With SCA3 ataxia, the common theme across all three components is loss of control due to loss of brain function:

  • Physical loss of control results in degradation of muscular coordination, from the eyes to the toes.
  • Cognitive loss of control results in degradation of memory function, of the ability to concentrate, of the ability to learn, etc.
  • Emotional loss of control results in having mental health problems such as phobias, panic attacks, and depression.

Parkinson’s disease is readily acknowledged to involve these three components, but spinocerebellar ataxia isn’t. Is this perhaps because more people with Parkinson’s disease have been studied? Parkinson’s disease is at least 63 times as prevalent as spinocerebellar ataxia.

Parkinson’s disease primary affects the basal ganglia. SCA primarily affects the cerebellum. But I am learning that the basal ganglia are involved with some of the SCAs, and possibly SCA3 in particular. After I explore that, I will summarize my findings.

Here’s what I think about SCA and SCA3 in particular, based on my own experiences. I’m not an expert. I need to emphasize that on this particular topic, there’s a lot of conjecture on my part.

Loss of control

I want to emphasize the importance of this term. Ataxia is all about gradual loss of control across brain function. This loss of control term is the E = mc2 of ataxia, if you will. Any ability that requires effort and/or coordination, even subconsciously, degrades over years of time. This includes abilities typically taken for granted, such as walking and swallowing, as well as abilities such as emotionally “holding it together” when at the dentist’s office.

There are people who truly expend no effort when others do. For example, if you’ve overcome stuttering (as I did, for the most part), chances are that it takes a certain amount of control to avoid stuttering every time you speak, even when you are successful. With ataxia, you lose that control, and the speech impediment comes back. On the other hand, if you never experienced stuttering before in your life, then ataxia won’t bring it on. The same goes for other emotional/psychiatric issues, such as phobias and depression.

Physical impairment

Most if not all people with ataxia experience physical degradations first, and most descriptions of ataxia stop there. Now that I have been experiencing ataxia for at least eleven years and getting a distinct taste for the loss-of-control aspects of it, and especially finding that Parkinson’s disease is readily acknowledged to have loss-of-control aspects across the physical, cognitive, and emotional realms, I can’t help but think that, of course, ataxia has these three components as well; of course, it’s not all physical.

You’re ignoring me

I have a few ideas for why the cognitive and emotional aspects are generally ignored:

  • Perhaps the physical degradations are the root cause of the other problems. For example, maybe continuous, daily mental exhaustion impairs short-term and long-term memory. Maybe panic attacks are caused by arguably rational fears such as of not being able to breathe due to swallowing difficulties.
  • By the time you have serious cognitive and emotional issues, you can no longer clearly communicate due to physical issues with speech. Your cognitive and emotional problems go unexpressed and unexamined.
  • The cognitive and emotional issues are secondary to the physical issues. The physical symptoms get worse and compound, whereas the secondary issues don’t follow the same accelerating spiral (my guess: exponential vs. linear degradation). Ataxia is rare enough that the secondary issues don’t get much attention.

Cognitive impairment

In at least the first of my two decades knowing about SCA3, there was a universal emphasis on how those with the disease wouldn’t experience any cognitive impairment. Even in those early days, I could tell that this was the cheerleading side of the medical community trying to boost the dignity of those with the disease by telling them that in spite of their physical degradation, they were as smart as ever. I have not known many people with SCA3, but from the outside looking in, it appeared to me as if people who suffer for a long time begin to make irrational choices. Cognitive impairment? Well… I saw it more as shifting priorities.

I participated in a study for two years that evaluated over time the participants’ physical, cognitive, and emotional states. I shouldn’t be jumping to any conclusions here, but for myself it’s plain to see: what I have been describing as mental exhaustion is, in fact, some level of cognitive impairment. I am operating at an ever-shrinking cognitive capacity. Specifically, my short-term memory is pathetic, as is my ability to concentrate, and my ability to recall things I used to know and memories I used to have is waning. I’m sure that the rate and limits of decline are much lower compared to something like Alzheimer’s disease.

I’m at a point with my long-term memory where I know generally what I can’t remember, but I can’t remember specifics. For example, I am one who has remembered many defining moments throughout my life, many in a school setting when I was fairly young, some later in my career. I used to sit back occasionally and reflect on those moments. Now, I sit back to reflect on those moments, and I can’t remember them!

An impaired short-term memory pretty much puts a stop to learning. I have difficulty looking at 7-digit numbers, looking away, and immediately trying to recall the digits. Though I don’t systematically test myself, this is quantifiable. And this limitation comes into play for any kind of information I’m trying to process and learn, not just a few numerical digits.

I know there’s nothing here to prove that my memory (i.e., brain function) is being affected by ataxia. This is when healthy people like to jump in and say, “I have the same problem!” I’m at the stage where I’m less willing to account for my cognitive decline being the nebulous result of mental stress and more willing to conclude that ataxia is causing me to lose control of my cognitive abilities.

Here’s a study that acknowledges SCA’s impact on cognition:

Emotional aspects

In the study that I’m participating in, the emotional questions I get mostly have to do with depression, suicide, vertigo, and panic attacks. They’ve recently started asking about uncontrolled laughing and crying, so I assume there’s a trend for that among participants; that’s clearly consistent with the theme of loss of control.

I saw the conclusions of a study that said that eventually dealing with a fatal disease can negatively affect one’s outlook, along the lines of depression, but that SCA3 by itself doesn’t cause clinical depression. I will add, if it truly took no effort for you not to be clinically depressed without ataxia, then having ataxia will likely not bring on depression. If you are prone to clinical depression, then the loss of control aspect of ataxia is more likely to bring on depression.

I am seeing all of my emotional/psychiatric weaknesses becoming amplified. For example, I had mild claustrophobia a few times in my early life. In recent years, I now experience mild claustrophobia (i.e., mild panic) whenever I get even a little restricted, such as tangled up in my own blankets when I’m in bed, or getting into a small car in heavy winter clothing.

The older I get, the more irrational my existing fears and reactions become. I’ve never liked hypodermic needles, but in my early twenties I was able to leave it at that: extreme dislike. In my thirties, I would become lightheaded if I stood up immediately after being poked. Now, I can faint sitting upright in a chair while having blood drawn. It’s all about panic and irrational fear around needles being inserted into my body; I have no problem with blood.

Another area of increased fear is laughed at by many, and even I laugh at it myself, which is fear of spiders. Even comically large, stuffed-animal spiders make my skin crawl. I know it’s irrational, but I can’t help it, and it’s getting worse.

The other areas where I experience panic and irrational fear all have to do with speaking: socially, publicly, and over the phone. Note that I am a reformed stutterer, which is a topic unto itself, especially as it creeps back up on me. I correlate my speaking phobias to having an unnatural need to swallow excessively.

Again, there’s nothing here to prove that ataxia is involved. I realize that many people without ataxia have phobias around these very issues, but my point is that these are only existing emotional weaknesses in myself becoming amplified, as my emotional control is weakening. I have no new areas of weakness. I have never had a fear of flying (and have flown), never been carsick or seasick in cars and boats, I don’t fear snakes or germs, etc.

Added 2014-12-23: Here’s the first webpage I’ve seen that acknowledges issues similar to what I put forth on this page:

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