The Cardinal Signs of Parkinson’s Disease – From a Patient’s Perspective

Let’s begin with a caveat. No two cases of Parkinson’s disease will follow the exact same course. We are like snowflakes… similar to each other in so many areas, but ever-so-slightly different with the details.

That being said, there are four so-called ” Cardinal Signs” of PD. They are…

1. Resting Tremor

2. Rigidity

3. Bradykinesia

4. Postural Instability

Think of it as a great Big Brain Buffet, where you can help yourself to a little of some, a lot of the other, very little of one and none of the fourth one. I’m not going to spend a great deal of time on the signs I don’t have. Why not? Because I don’t have them. I’ll tell you what they are, what you should watch out for, but I will not be able to offer any real practical advice on things I don’t experience on a daily basis.

Let me put it this way. When I went to the Big Brain Buffet in late 1999, I decided I’d help myself to several serving spoons full of Rigidity and Bradykinesia, just a little bit of Resting Tremor, and I decided to pass on the Postural Instability.

Now that I approach the 12th anniversary of my first visit to the Buffet, I’ve found that I heap my plate FULL of the Postural Instability and Bradykinesia. The Deep Brain Stimulation I had in 2007 keeps my appetite for the Rigidity to a minimum and I’ve never really had all that much of a taste for the Tremor in the first place, so I leave that alone.

But to get a diagnosis of PD, you have to display at least three of the cardinal signs, and you can’t join our exclusive little club unless those signs have a positive response to Parkinson’s medications.

So, let’s get started with…

A. Resting Tremor

It is exactly what it says it is. A back and forth rhythmic tremor that happens when the affected extremity is at rest. If you have tremor when you move or use your hands, you probably have something other than PD.

Here’s what my friends at the National Parkinson Foundation say about tremor.

Tremor (shaking) is a rhythmical movement that can’t be controlled, often starting in one hand.

As the Parkinson’s tremor usually appears when a person’s muscles are relaxed, it is called a ‘resting tremor’.

This means that the affected body part trembles when it is not doing work, and it normally improves when a person begins an action. Anxiety can also make tremor worse.

It is estimated that tremor occurs in about 70% of people with Parkinson’s, so not everyone with the condition has this symptom.

So, lucky me, I’m one of the 30% who doesn’t really show much in the way of tremor. If I get stressed, my right hand will shake a little. But mostly, all the tremor you’re going to get out of me is a little back and forth in my right forefinger and thumb.

Therefore, if you want to know more about tremor, you’ll have to ask someone else. I recommend the National Parkinson Foundation’s very helpful website, chock full of information for newly diagnosed folks, veterans like me, and our caregivers.

So… moving on.

B. Rigdity.

When my medication is working and my DBS devices are turned on and tuned in, rigidity is not a problem for me. But I can tell you what it feels like.

When I was going through the screening for the Deep Brain Stimulation in Early Parkinson’s Disease Clinical Trial at Vanderbilt University in 2007, I had to go through something I lovingly referred to as “Droolfest.” This was an eight-day period where I had to stay as an inpatient in the Clinical Research Center at the Medical Center in Nashville with no Parkinson’s medications. After I had the surgery, there were four more Droolfests where I had to turn off the devices and stop taking the meds for eight days.

It’s pretty self-explanatory, actually. Imagine your muscles. Right now they move pretty smoothly, don’t they. Now, imagine them being made of pliable plastic. They can move, but only with difficulty. When you walk, your leg muscles protest. They get sore. Hell, they get PISSED. They don’t LIKE the fact that your brain isn’t sending them the correct messages. They send pain signals back to the brain, hoping your brain will get the idea and start sending the correct messages.

But your brain isn’t getting enough (or any, for that matter) of that critical neurotransmitter called “dopamine.” That’s why you have Parkinson’s. Your brain tries to tell your leg muscles that it’s doing the best it can. But your leg muscles just don’t care. They get sore. The soreness makes them even MORE stiff. They decide you’d be much better off with your butt in a recliner and a bowl of chips on your lap. And they’re right.

But it’s not just your legs. Your arms are wondering just what the hell is going on, too! And now that you’re sitting down, you want your arms to move, to reach into that bowl and tell your hands to open and close around a CHIP? Nothing doing, bub, until you shoot down the correct messages. Otherwise, your forearm and upper arm muscles are going to get sore and irritated and even more stiff. And don’t bother with the Ben Gay. Parkinson’s disease LAUGHS at Ben Gay. It makes FUN of Ben Gay. It MOCKS Ben Gay.

And did I mention your neck? Your neck muscles are wondering why after a long day of holding your big, heavy head up where it belongs, the brain isn’t sending the normal signals so they can move more freely and be elastic like they used to be. So your neck muscles hurt and…

CRAMP!

And as soon as you convince your sore and tired hand that feels like it’s been squeezing tennis balls all day to rub the cramp out of that muscle, the other side of your neck says, “Oh yeah?”

CRAMP!

Then your hand cramps because it’s terribly unfair of your brain to expect your hand to rub cramps out of other muscles when the muscles in your hand aren’t getting the correct signals either and on and on it goes until you go to bed.

Then you lie there, unable to unclench. You notice your head is not sinking into your pillow. You have to force yourself to relax. You start with your neck, work your way down to your shoulders, then your back, now your abdomen. Your pelvic muscles get the idea, then your thighs, finally your calves then your feet. And you’re fine.

Until you need to roll over.

CRAMP!

That, my friend, is Rigidity. Your doctor can determine just how rigid you are by telling you to relax so he or she can move your limb around. If they feel a racheting motion, that is referred to as “cogwheel rigidity.”

But, as I said earlier, the meds help and so does the DBS. So, let’s continue our little stroll through the cardinal signs of PD with…

C. Bradykinesia

(Sing along to the theme from “The Brady Bunch “

Here’s the story of a working body

And it used to move so swiftly and so well.

You could run a mile or more with little bother.

But all that’s gone to Hell!

It’s the story of a guy named Parky.

Who way long ago in 1817

He first noticed guys moved slow and really shaky

And that just wasn’t keen.

Until one day when the doctor wrote an essay

And he knew then it was much more than a hunch

When he wrote An Essay on the Shaking Palsy

And that’s the day we all became the Parky Bunch.

OK, now that we have the Brady references out of the way…

Bradykinesia simply means ” slowness of movement.”

Try this.

Scratch your nose.

Notice how your hand lifted from your lap as your shoulder raised your arm and your forearm rotated your hand into position and the correct fingers for scratching extended themselves and automatically found their way to that itchy spot on your nose and then the fingers moved back and forth until the itch was gone and then your shoulder rotated your upper arm back to its resting position and your forearm turned your hand back to where it was in the first place and it went back down to your lap.

When you have bradykinesia, the same thing happens.

Very.

Very.

Slowly.

If you’re not also fighting rigidity, the motions are fluid but slow.

This is generally referred to as the most disabling symptom of PD. And it’s frustrating, especially in the early stages, because one moment you’re gliding right along and then you’re the pokey little puppy! And no one really knows why.

And that’s why, especially early in the disease, some folks think you’re faking it.

“BUT YOU LOOK FINE!” they insist.

“I feel fine, right now.”

“ARE YOU SURE IT’S PARKINSON’S?”

“My doctor said so, and I trust his judgment.”

“I THINK IT’S NONSENSE. GET A SECOND OPINION! I GET LITTLE TWITCHES SOMETIMES, TOO. I THINK IT’S JUST THAT THE DOCTORS WANT YOUR MONEY.”

“Gee, you really think so,” I say as I reach for the baseball bat.

“OH, YEAH. IT’S ALL A BIG…”

WHAP!

It’s that kind of insensitivity that drove me nuts…

Hang on a sec. He’s still moving.

WHAP!

WHAP!

WHAP!

OK, where were we?

Oh, yes. It’s that kind of insensitivity early in the course of the disease that actually causes one to question whether or not he HAS the disease. And I was no different. For awhile I denied it. I stopped taking the meds. I looked for other reasons for the signs and symptoms. But finally, in early 2007, a kind and thoughtful neurologist told me that not only did I have Parkinson’s, but it was a fairly classical case thereof.

I still have the bradykinesia. Not so much in my hands, because I can still type fairly quickly. The meds and DBS help. But my arms, my legs, my face, my neck they tend to move pretty slowly.

I walk slowly. When I’m on the treadmill, I can generally get the speed up to 2.5 mph without difficulty. Over that speed, things get goofy. And a lot of that, I think, has to do with our NEXT cardinal sign…

D. Postural Instability

This is a tricky one. It’s something they start testing for right away when you’re diagnosed. Every time you see a neurologist, they get behind you, give you a little tug, and see how many steps – if any – it takes for you to keep from falling.

Up until early 2008, I could generally catch myself with one or two steps. Now, I keep stepping backwards until I either hit something, someone catches me, or I fall.

I am confused on the reasons for postural instability. And I don’t really care. I just know I have it. I walk with a hunched over, forward lean (unless I have my walker and really, really think about it, then I stand up straight and tall until I forget again and find myself hunched over). My gait is severely hampered. If I’m on the treadmill or holding on to my walker, I can take longer, more striding steps than I can if I’m not holding on to anything. Then, it’s little tiny, flatfooted steps.

I am clear on SOME of the reasons for this, but to explain it will cause us to wander off into the sciency weeds a bit.

Here’s an example of what I’m talking about.

My wife gets up this morning. Coffee’s already brewing, so I know there will be no beating for me. At least not yet. She approaches, I extend my arms for a hug and, out of force of habit, close my eyes for our good morning kiss.

I feel myself starting to tip backwards. I take several little backwards steps as I open my eyes. My wife grabs my by each shoulder to steady me. THEN we kiss.

This is the result “proprioception.” Put as simply as possible, it’s one of those $20 words that means the ability to sense the position of your body and the strength and effort being employed in movement.

Jeez. Even the SIMPLE explanation is whonky!

Let’s do a practical demonstration.

Stand up. Don’t argue with me, just do it.

Put your feet side by side. Stand there for a minute. Are you weaving from side to side? Probably. A little anyway. Why aren’t you falling?

Proprioception, gentle reader. Your brain and your eyes and your nerves and your muscles are all working in concert to keep you balanced.

Close your eyes now. See how long you can stand there without feeling the need to open your eyes and catch yourself.

See? Your eyes are an important part of your ability to retain your balance. If you can stand there all day with your feet together and your eyes open, you’re normal. If you can do it for a minute or more with your eyes closed, you’re normal.

I can stand with my eyes open. When I close them, I start to sway like a tall building in an Earthquake.

It is the rare day when I don’t ALMOST fall. But the house I’m living in is small enough to where there’s almost always something close by in grabbing distance to steady myself. But I do fall.

Sometimes it has nothing to do with vision. Not long ago, I was getting off the throne in the bathroom and turned to flush. I turned incorrectly. See, in my current condition, one must turn by moving one’s feet, not by pivoting one’s body. If I twist my upper body and leave my feet planted, I will fall.

As I did in this instance.

I took several stutter steps backwards until the backs of my calves touched the rim of the bathtub. Then I fell into the tub.

I had fallen and couldn’t get up.

Luckily Gail heard the crash and rushed into the bathroom. There she saw me, my feet protruding from the tub, my underwear bunched around my ankles. She helped me. She always helps me.

And there, we have touched on the four cardinal signs of Parkinson’s disease. Now, we’re going to delve a little deeper and I will show you what it’s like to freeze when you walk, what it’s like to choke on your food, what it’s like to walk towards something and suddenly find yourself walking backwards, what it’s like to be walking more or less normally and suddenly find you are taking faster, quicker steps to keep from falling.

Like I said earlier, we’ll try to keep from getting too sciency here. Unless you WANT to read about how Parkinson’s is primarily a malfunction of the corpus striatum, composed of the caudate and putamen, is the largest nuclear complex of the basal ganglia. The striatum receives excitatory input from several areas of the cerebral cortex as well as inhibitory and excitatory input from the dopaminergic cells of the substantia nigra pars compacta (SNc). These cortical and nigral inputs are received by the spiny projection neurons, which are of 2 types: those that project directly to the internal segment of the globus pallidus ( GPi ), the major output site of the basal ganglia, and those that project to the external segment of globus pallidus ( GPe ), establishing an indirect pathway to GPi via the subthalamic nucleus ( STN ).

Otherwise, we’ll try to keep things simple.

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