Monday, 20 January 2014

Primary Lateral Sclerosis uncovered

In our monthly newsletter, someone with PLS (the sort of MND I have) sent in this description by a chap called Galen trying to explain the condition. It’s rather good. (I hope he won't mind me quoting it verbatim. As you can tell he has a sense of humour - which is quite helpful in the circumstances.)

If I start telling people about upper and lower neurons I can almost see their eyes glaze over as they decide I must have some need to tediously explain PLS. They then avoid me. If I launch into how PLS may be a "gentler and kinder" form of ALS, or Lou Gehrig’s Disease, people often fixate on the ALS part and have even asked me why I'm not dead yet and how much longer do I expect to live. When I tell them I have a rare condition that causes a loss in communication between the brain and motor functions, most people feel I've been technical enough and sufficiently succinct.

If folks are still curious I can launch into examples. Walking over to a table to set down a glass of water is something that is done almost without thought by most people, however, the communication with my brain and the rest of my body has gotten so that I have to concentrate hard to even walk to the table. Trying to carry a glass of water is likely to overtax the limited capacity I have remaining, with the likely result being that both I and the glass will wind up on the floor. Or it can be more insidious. If I'm about to get out of a chair I may have devote virtually all of my resources into planning and executing exiting the chair, and may actually wind up ignoring anyone trying to talk to me while I try to stand. It isn't because others are boring or anything, rather I have to focus so hard on getting up, it blots out other things.

It's not a problem with the brain, we remain as sharp as ever. It's not our muscles, they work fine. It's communication between the two that is the problem. Walking requires intense communication with our balance sensors, our brain, our legs, feet, even our toes. Our consciousness isn't aware of it, yet it requires constant fine- tuning. Throw a monkey wrench into that communications system and you wind up with someone who can barely stagger anymore, a symptom many of us can relate to. Sometimes one side is affected more than the other, and you get someone like Ronnie, or it can be pretty symmetrical, like Flora, or it can add to an already serious personality disorder, like me.

It can have strange results. You can be at a funeral and your brain can be saying: "Whatever you do, don't make a scene," but because the message gets garbled, your mouth muscles say: "Roger, brain. Commencing uncontrollable laughter immediately." Or you can think you told your mouth to say to the UPS guy: "I'm feeling fine, and you?" only to have him look up at the sky and say: "You're right, it does look like rain."

We are constantly compensating, whether or not we are aware of it. Maybe that is why we get tired all the time "for no reason." We are always having to figure out new ways for our brain to signal our muscles to do stuff. Our brain may be so busy devoting time to keeping essential functions going that it can't worry about things like walking and talking.


  1. Great narrative on living with PLS... My name is Carol and I was diagnosed with PLS in 4/2000. I believe the most frustrating thing, in my case, is losing my ability to speak & be understood. Sure, the progression of my PLS has been slow.. I now use a power chair, rotator walker, given up driving a vehicle... These losses were mourned and finally accepted.
    I find comfort in keeping a sense of humor, a strong positive attitude, thankfulness, gratitude for what I am able to do & faith that good things happen every day.
    I will look for your books, your blogs & perhaps we could connect through e-mail or Facebook.
    Blessings to you...

    1. Hi Carol
      I'm sorry you have to face this pestilential condition. It does keep bringing bereavements in its wake, doesn't it? Every now and then I have to shout (by email) to a long-suffering friend of mine who hears and reassures me it's all right to grieve!
      You're also right that good things happen even when it's very hard-going.
      It would be nice to be in touch via Facebook. The email address link on this blog is dead, but I can let you have an alternative via Fbk message.

  2. Carol, the courage of yourself, Michael and others leaves me humbled. I visit Michael's blogs ( not because I have PLS) because I find them encouraging, humorous and challenging.
    As I said, your faith and courage in coping with your losses are a blessing to those of us who read about how your life is now. A huge thank-you to you.

    1. Thanks, Ann. You're a great encourager. I guess we're all pilgrims on a progress.

  3. I was diagnosed with PLS 13 years ago and the problems I have are slightly different from yours, maybe mine hasn't progressed like yours. One day this past month I found that emptying a jar of spaghetti sauce wasn't as easy as it used to be. I had a sudden spasm and ended up with spaghetti sauce all over the stove, the walls, etc. It's totally unpredictable. Walking is difficult, so is climbing stairs. When I sit for a long time, I have trouble getting up, but I can't stand for long either. It's a matter of trying to put the least amount of strain on the body. I try to keep a positive attitude too, but it can be difficult, especially when I'm falling down while trying to vacuum. I can still drive and talk, though an occasional twitch while driving or a sudden wrong swallow are indications that the end of those activities is coming. The worst is when I need help to get up after I've fallen. Or when I'm befogged by my medication. Every day can be a different challenge.

    1. You describe PLS so well. You're right it affects individuals all differently. But it is hard as you sense impending failure of another bit of your body. And those sudden spasms are such a pain - the spaghetti sauce sounds spectacular. You pinpoint one of the strangest aspects in my experience - the difficulty of simply standing still. I can actually walk, with my rollator, longer than I can stand. My physio is very good and reminds me that doing anything with MND takes so much more effort than normal. She encourages me not to try to overexert. It's hard to stay positive all the time.

  4. You really are truly heroic.
    Watching the DVD "Of Gods and Men" the other evening I was very moved by this passage which was being sung as the monks were being marched away

    "We do not see your face, Infinite Love,
    but you do have eyes
    for you look upon us
    with a shining gaze"
    It's hard to remember this when things are tough but very comforting.
    Michael had this wonderful quote and commentary on one of his blogs, I cannot remember which one, but it helped me and I hope it may help you.

    "From “As You Like It”

    "Here feel we but the penalty of Adam,
    The seasons' difference, as the icy fang
    And churlish chiding of the winter's wind,
    Which, when it bites and blows upon my body,
    Even till I shrink with cold, I smile and say
    'This is no flattery: these are counsellors
    That feelingly persuade me what I am.'
    Sweet are the uses of adversity,
    Which, like the toad, ugly and venomous,
    Wears yet a precious jewel in his head;
    And this our life exempt from public haunt
    Finds tongues in trees, books in the running brooks,
    Sermons in stones and good in everything.
    I would not change it."

    I was struck by the expression, "Sweet are the uses of adversity". Our instinct is to run from adversity, to avoid pain. And yet there is something precious to be found in it. Exiled from all that is "civilised", what we might regard as the normal comforts of life, the Duke finds "good in every thing". This is poetry of course which can't be paraphrased - but, for example, the trees might speak of the beauty in each season of life and the miracle of rebirth, the brooks might be illustrating the infinite diversity and the wonderful interconnectedness of creation, and the stones teach that hardship is an integral part of existence; without it life would be bland.

    1. You might enjoy the next post, Ann, where Bo Stern says, 'May we have the faith to believe with Martin Luther King, Jr., that “unearned suffering is redemptive.” '

    2. I was diagnosed with PLS about two years ago. By the end of 2012 I had written 6 novels. The first was THE BETTER ANGELS, published 2000. The 2nd was 'MATE, the third was CIRCLES. Next was WELL! Then came WALL; the last was BUDDY. a story about Buddy Clark, who would have been a bigger star than Crosby and Sinatra. All (except the first) remain by choice unpublished--due mainly to PLS. I still write a weekly ed-opt column, published every Saturday by Late in 2013 I went to the Mayo Clinic in JAX; there I was diagnosed with .Corticocasal Degeneration. My original neurologist said the doctors at Mayo were full of beans; "I WILL GO TO MYGRAVE SAYING YOU HAVE PLS!" Despite his insistence, I will defend his diagnoses to the death--which according to me wife, could not come soon enough (at 83, she should not have to wait long). But I remain as sharp as ever (click on my ed-opt column if you need proof).

      R. A. Mills


    3. Thanks, Robert. You seem as free of self-pity as I hope I shall remain. Enjoyed reading your ed-opt entry and dipping into your blog. Best wishes.

  5. Yes, I do cling to that Michael.
    I wouldn't have the timerity to claim that my life is anything like as difficult as yours, Carols and a n other's.
    The difficulty for both Ian and I is that we are still battling here on behalf of all those who live here. Lately, with some degree of success (it's too long a story to go into)
    For me ,the difficulty is trying to distinguish between battling in a right way whilst being able to separate it from negativity and resentment at the way older people are perceived.
    I cannot begin to describe to you how deeply moving, encouraging, challenging and inspiring I found " Of God's and Men" I keep returning to some of the Psalms they chanted. Especially this
    "We do not see your face, Infinite Love,
    but you do have eyes
    for you weep through the oppressed and look upon us
    with a shining gaze
    that reveals your forgiveness"
    I found very much food for thought in Bo's post.
    As ever, 'thank-you'