Happy MoNDay!
I’m not always a glass half full guy, but try at least to be an “at least there is something in the glass” kind of guy. I get asked quite a lot lately, and sometimes observed very intently, about my “condition” when I’m out and about. I’ve done my best to explain it but the truth is that it is a rare and somewhat hard to comprehend disorder. My intention is not to bug anyone who isn’t interested, but only those who are curious as to what is going on. During the diagnostic process, (and sometimes even after,) Tabby and I received a lot of kind and concerned messages with help of what I could be dealing with. Our diagnostic process through the medical system alone went something like this.
1. Crazy & Lazy
2. Pinched Nerve
3. Lyme Disease
4. Chronic Fatigue Syndrome
5. Mono (Epstein Barr Virus)
6. Multiple Sclerosis
7. Primary Lateral Sclerosis
There was also a heavy helping of testing for Lyme disease every few months I think. The point is, it took the Mayo Clinic and one of the leading doctors in motor neuron diseases, Dr. Appel, along with my astute and experienced local neurologist, Dr. Farmer, to finally figure out with quite a bit of certainty what was going on. With that said, I hope this helps shed light for those curious on what is a boring and odd subject.
My clever title of “MoNDay” has emphasis on the “MND” for a reason. Most people are at least somewhat familiar with the ice bucket challenge which raised awareness for a neurological disease known as ALS or Lou Gerighs Disease. The disease is a progressive and usually shortens ones life significantly. Below I’m going to do my best to explain the conditions of Motor Neuron Diseases as best I am able.
There are basically two types of motor neurons, both upper and lower, that allow us to send signals and activate our muscles. The upper neurons carry the signal from the brain to the spine and the lower neurons finish the task by telling the muscle to activate. The upper motor neuron malfunctions cause the signals to error which results in spasticity or tightening of the muscles. This is painful if not under control and when a muscle is spasming even lightly, it is as if you are doing twice the effort for a simple task. Eventually, fatigue sets in and the muscle becomes tired and difficult to move. Failure of the upper motor neurons and no lower motor neuron involvement is referred to as PLS or Primary Lateral Sclerosis.
At the other end of the spectrum is a condition known as PMA Primary Muscular Atrophy. In this disease the lower motor neurons are primarily affected with little upper motor neuron malfunction. However, because the lower motor neurons are what actually activate the muscle, with those neurons dying, the muscle atrophies and cannot be used.
In the middle of the spectrum sits the insidious disease known as ALS or Amyotrophic Lateral Sclerosis. In this instance, both upper and lower motor neurons are malfunctioning at a rapid pace. The disease is fatal with no real treatments.
There are several other variants, and if you are curious you can read more at the National Institute of Neurological Disorders and Stroke Association.
The thing is, if you are going to get one of the three, PLS is the “better” of the options due to the life expectancy being pretty normal. The thing about the diseases are that they are somewhat like a spectrum rather than distinct buckets. No two people seem to have the same course of disease, (perhaps like many medical conditions), although there are several hallmark indicators which can help identify and group patients. Most PLS patients progress slowly overtime “eventually” needing a mobility aid such as a cane or even a wheel chair. Mine seemed to go rather quickly for me. I can still stand and I haven’t lost control of my muscles, but walking even short distances might as well be like taking an extended release sleeping pill, except my muscles will be tight and twitch for the next few days. I feel compelled to mention that I wasn’t exactly a couch potato in the year leading up to the onset.
Just this last weekend, and for the last two months, my jaw, vocal cords, and tongue have become tired. Things start off normal, then as the day goes, with much effort in anything, I start sounding like a lisping drunk. I never thought I would be one to get tired from talking too much but that is yet another facet of the disease.
One’s odds of getting the disease called PLS are estimated to be around 1 every 660,000 people. In other words, there are probably around 500 true PLS patients in the US. This is probably due to several factors. First, since it is rare, most neurologists will never see a PLS case let alone a general physician. Second, do to the spectrum of disorders with motor neuron diseases, it can take several years to bucket the type of disease. Third, aside from electromyograms which is layman for jabbing needles into your muscles and nerves while the doctor wiggles them around, there is little testing that can be done besides ruling out other things. There is no blood test.
Three things I try to keep in mind.
Enjoy the rest of your MoNDay and please go easy on my best shot at medical explanations. Remember, I studied business in college, not anatomy.
Links to Info about PLS
https://sp-foundation-org.presencehost.net/understanding-pls-hsp/pls.html
https://www.mndassociation.org/wp-content/uploads/02c-primary-lateral-sclerosis.pdf