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Young male with suspected bulbar MND - advice on next steps

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    Young male with suspected bulbar MND - advice on next steps

    Hi there,

    First off: thanks to all the great people who take the time to reply to posters on this forum. I really appreciate what you do and am grateful for the time you give. I'm posting because I'm a young man who is being investigated for suspected motor neurone disease (bulbar onset) and would be grateful for some advice on navigating neurologists, and the best way to rule in or out MND as efficiently as possible.

    Despite MND being put on the table (more details to follow), I have what I have been told is a slightly unusual medical history, which for a long while didn't fit clearly into MND nor any other known condition.

    My problems began in August 2018 when after a run I spontaneously lost the ability to burp. It was very abnormal, as though the back of my throat/top of the back of my neck had suddenly gone slack. I was no longer able to control the muscles there to initiate a burp. Unfortunately, this, like all of my other symptoms did not improve with time. Muscles simply disappeared and never returned. At the same time I began having hundreds (between 100-200) microburps, hiccups and yawns every day, as well as random aches and pains throughout my body, and just generally feeling ill.

    I saw a few GPs and specialists who didn't really take my problem seriously and I put it to the back of my mind. However, this became increasingly difficulty as my inability to burp led to increasingly severe bouts of trapped air and progressive bloating. From August onwards my core muscles began progressively weakening, a process which has now - three years on - advanced to a point where I can no longer do sit ups or rise normally out of bed. In February/March 2019 I suddenly began suffering from facial muscle twitches. These began under my eyes, and I thought little of it, but quickly advanced to include my cheeks, lips, chin and jaw.

    Then, in June 2019, the same thing that had happened with my throat happened with the muscles under my tongue/chin. I spontaneously lost the ability to swallow. I could not for the life of me activate a swallow and when I could the muscles were spasmy and uncoordinated. Again, several muscles seemed to have vanished and were not working. Others were cramping painfully or contracting way too hard or too fast. There was a little tingling and clicking sensations around my Adam's Apple but no real pain or sensory symptoms. I was unable to swallow anything satisfactorily for a few weeks but then I managed to retrain myself and trigger a swallow by puffing out my cheeks and using different muscles. Over time my swallowing improved a little to the point where I could swallow though badly, although the muscles that had disappeared did not recover. From that point on my swallowing has slowly worsened and although I can now still swallow, it is becoming very precarious.

    During this swallowing episode, the twitches migrated from my face to my neck and abdomen where there remained for the next few months as I began experiencing slowly worsening neck and core weakness, often with aching and cramping. Shortly after this the twitches moved to my shoulders and arms which slowly weakened in turn, and my legs. Now, three years on from the burping episode, and two and a half years on from the sudden swallowing issues I can no longer do any sports - because my muscles buckle - work out nor exercise with any intensity. Day to day tasks are becoming increasingly difficult. I can no longer chop hard vegetables one-handed, due to poor arm strength, and am beginning to have profound difficulty with things like bottle caps, particularly using my left hand. All of the progressive weakness has gone hand in glove with prominent wasting, spanning my neck, shoulders, chest, abs, arms and legs.

    Because my weakness began so insidiously and because of my age (26) I had a hard time getting physicians to take my symptoms seriously to start and it is only recently that a doctor has explicitly said to me that they think I might have MND (having studied my history and noted brisk reflexes, clinical weakness and wasting in an exam). They have referred me to a local neurologist, however I have been told to expect a waiting time of about a year, so have opted to go private and am awaiting an appointment. Unfortunately, the only neurologist I can see in my area (Scotland) is a generalist with expertise in headaches - he doesn't specialise in neuromuscular disorders. Is this person going to be able to rule in or rule out MND effectively, or is it worth pushing to be seen by an MND specialist? I would be very grateful for some advice on this front.

    The other thing I would love some advice on is if there is anywhere that I can find reliable information on about my outlook, if this is, as it is increasingly seeming, indeed MND? I understand bulbar is associated with a worse prognosis but that young age and a long delay until diagnosis augurs well. Is there a realistic chance that I could keep going with some decent quality of life for say, another five years? And if I can is there a realistic hope that some new treatments that could stop MND progressing might arrive in that time? (This is a particularly important question for me as it will inform my life decisions)

    Thank you,

    Flex

    EDIT: I should add that at this point the main mimics of MND have been ruled out. My creatine kinase levels are normal, ruling out polymyositis and other autoimmune diseases as well as muscular dystrophies, and I also have a normal autoimmune profile - I tested negative for myaesthia gravis antibodies. I've had MRIs which offer no structural explanations for my problems. If anyone knows anything else which could be causing my symptoms which has not yet been ruled out, I would be keen to know.
    Last edited by FlexyWex101; 1 February 2022, 21:11.

    #2
    In February 2021, you posted extensively on another ALS forum in which you had been seen by a Neurologist, had an EMG, and were told that you didn't have ALS.

    One year on, and you're obviously still troubled by what's going on, so maybe go back to that Neurologist who has your medical history and test results and go from there?
    ​Diagnosed 03/2007. Sporadic Definite ALS/MND Spinal (hand) Onset.
    Eye gaze user - No functional limbs - No speech - Feeding tube - Overnight NIV.

    Comment


      #3
      Originally posted by Ellie View Post
      In February 2021, you posted extensively on another ALS forum in which you had been seen by a Neurologist, had an EMG, and were told that you didn't have ALS.

      One year on, and you're obviously still troubled by what's going on, so maybe go back to that Neurologist who has your medical history and test results and go from there?
      Hi Ellie,

      Thanks for replying to me - I really do appreciate it. Unfortunately, seeing that neurologist isn't a practical option, since they are in a different country to the one I am in now; geographically, it is easier to see someone else. However, I have been in email contact with that original neurologist who has asked to be kept in the loop.

      I did indeed have an EMG too, about a year ago. After this, I was reassured that I did not have MND, however, as I write, my symptoms have carried on progressing, mimics have been ruled out and I am now being told, albeit by a GP rather than a neurologist, that MND is suspected. Apparently, EMG is not a perfect test for MND and especially in bulbar cases it is not uncommon that it is 'clean' in the early stages and/or in non-symptomatic limbs. I have emailed a few neurologists asking for clarification on this, but have unfortunately not heard back.

      I know that writing is difficult for you so please do not reply to this unless you feel compelled (really!), but do you have any advice for me in terms of getting a diagnosis? I must admit that given the failure of my original neuro to appreciate the full extent of my weakness when I saw him a year ago and, to be frank, a history of bad experiences with doctors, I have lost a lot of faith in their abilities and wonder whether it is worth me pushing to be referred to a dedicated MND centre, such as Oxford's? My thinking behind this is that it would ensure a reliable diagnosis, there is little chance of signs being overlooked etc. MND or no MND, I am finding it increasingly difficult to thrive with my progressive issues and the possibility of a fatal disease hanging over me. For the sake of my mental health, I need clarity as soon as possible.

      Thanks again and all best regards,

      Flex

      Comment


      • I recall something similar on TV.

        I think a nodule on the spine or spinal cord that was very difficult to spot was causing similar symptoms.

        It was a benign nodule with total recovery.

        MRI it, but ask for a thorough inspection of the tracts behind the nose.

        Last edited by MNDConnect; 2 February 2022, 08:56. Reason: Removed comment which breaches forum guidelines

        Comment


          #5
          FlexyWex101 I'm sorry to hear about your concerns. The only person who can diagnose MND and answer your questions is a neurologist. No one on the forum is qualified to give their opinion on your symptoms.

          If you would like to discuss your concerns then please do give us a call on 0808 802 6262 Monday-Friday 9am-5pm.

          To all forum members - just a reminder that by registering and using this forum, you are agreeing to the forum guidelines. One of these guidelines is being kind to each other and breaches of this guideline will not be tolerated.

          Many thanks

          Rachel
          MND Connect Adviser
          MND Connect
          Contact us on 0808 802 6262 or at [email protected]

          Comment


            #6
            Originally posted by FlexyWex101 View Post
            ... do you have any advice for me in terms of getting a diagnosis?
            No, sorry, I can't suggest a diagnostic pathway - you have a complex medical history due to meds you were on, and the doses at which you took those meds.


            ​Diagnosed 03/2007. Sporadic Definite ALS/MND Spinal (hand) Onset.
            Eye gaze user - No functional limbs - No speech - Feeding tube - Overnight NIV.

            Comment


              #7
              Originally posted by MNDConnect View Post
              FlexyWex101 I'm sorry to hear about your concerns. The only person who can diagnose MND and answer your questions is a neurologist. No one on the forum is qualified to give their opinion on your symptoms.

              If you would like to discuss your concerns then please do give us a call on 0808 802 6262 Monday-Friday 9am-5pm.

              To all forum members - just a reminder that by registering and using this forum, you are agreeing to the forum guidelines. One of these guidelines is being kind to each other and breaches of this guideline will not be tolerated.

              Many thanks

              Rachel
              MND Connect Adviser
              Dear Rachel,

              Thank you for taking the time to reply to me. I understand that noone here can diagnose me and that a diagnosis is rather within the purview of a neurologist. I am of a mind to give you a call on the helpline, however I don't want to take your time - time that could be spent on someone with perhaps greater problems - unless you think it would really be useful. My chief issues aside from the symptoms are: (1) difficulty getting a diagnosis (as noted, I have had a history of disappointments with doctors and even now am struggling to get really expert input into my situation, with many false starts and delays - I could really use some advice for navigating these); (2) mental health struggles (as I'm sure you can imagine, facing increasing physical problems with the possibility of a fatal diagnosis looming over me makes life very difficult, and any tips for dealing with this would be greatly appreciated); (3) information for if I am diagnosed with MND (what is the current state of research into the condition? Is there a realistic chance of a treatment/cure, something to at least stop progression in, say, the next 5 years? 10 years? How long could I be expected to live with the help of non-invasive ventilation, carers etc.). Not having access to this information makes it very hard to stay positive and chart a course forward.

              Thanks again for your reply and all best regards,

              Flex

              Comment


                #8
                Originally posted by Graham View Post
                I recall something similar on TV.

                I think a nodule on the spine or spinal cord that was very difficult to spot was causing similar symptoms.

                It was a benign nodule with total recovery.

                MRI it, but ask for a thorough inspection of the tracts behind the nose.
                Thank you, Graham. I must admit my hopes are pretty low at this point, but I shall certainly raise this as a possibility with my doctors. It has been suggested that an abnormal hormone profile may be behind at least some of my symptoms - I was have repeatedly tested below normal on testosterone, for example - though I have never read of low test causing progressive weakness and loss of function of the kind I am undergoing. Interestingly, low testosterone is apparently quite common in MND patients.
                Flex

                Comment


                  #9
                  Originally posted by Ellie View Post
                  No, sorry, I can't suggest a diagnostic pathway - you have a complex medical history due to meds you were on, and the doses at which you took those meds.

                  That's no problem. And thanks again for replying, Ellie. Flex

                  Comment


                    #10
                    I thought I better provide an update on how things are going. In short: I've seen a neurologist who specialises in detecting motor neurone disease and he has said that he does not believe I have ALS. Unfortunately, he was unable to say what I do have, which is frustrating as my weakness, atrophy and so on is continuing and has now reached the point where my muscles are shaking violently and struggling to do simple exercise tasks - my arms are particularly bad and buckle rather than breaking falls - a health risk. I passed the neurological exam but felt a little frustrated that the muscles it seemed to test were not the ones where I am weakest. Mystifyingly, the neuro also said that I have no atrophy, despite the fact that I have picture evidence of clear changes in several muscles in my body. So, unfortunately I still have my doubts and, of course, the problem of my worsening symptoms, BUT I am doing my best to trust that the neurologist knows what he's doing and to put ALS out of my mind. I believe that something is wrong with me but it's clear that it's (a) either ALS presenting in a subtle way and being missed due to some factors (which I won't bore you by listing), or (b) something idiopathic that nobody has heard of or has a label for, perhaps involving my connective tissue. Either way, I'm tired of spending so much time and money searching for answers that are not forthcoming. I've decided to live my life as best I can and will only be returning to the doctors if and when I am actually unable to live day-to-day life. Flex.

                    Comment


                      #11
                      I just wanted to provide another update. Unfortunately, in the time since my last appointment I have continued to slowly decline. I now need to use hands to push myself up from the loo, for instance, which I didn't before, and am having worse problems cutting food among other things. There's more noticeable fatigue and difficulty holding things as well. The other thing I'm noticing is progressive sphincter muscle weakness. I wouldn't describe myself as fully incontinent but I have had to drop trousers a few times at very short notice to avoid crapping myself - it's part of ongoing issues in that department that I've had since my symptoms began.

                      After another GP appointment confirming my weakness and worsening issues I've been referred to a neurologist for early December. He's a general neuro because unfortunately on the NHS my area has no MND specialists. Given what's ruled out already, the referral was for suspected MND. I'm hoping he can provide me more clarity, or worst case refer me on to a dedicated MND centre for more answers. The (protracted and exhausting) process continues... One thing every doctor agrees on now is that that MND specialist who tried to dismiss my symptoms as natural aging and anxiety was talking nonsense.

                      Comment


                        #12
                        Originally posted by FlexyWex101 View Post
                        The other thing I'm noticing is progressive sphincter muscle weakness. I wouldn't describe myself as fully incontinent but I have had to drop trousers a few times at very short notice to avoid crapping myself - it's part of ongoing issues in that department that I've had since my symptoms began.
                        Sphincter muscles are not typically affected by MND.

                        Comment


                          #13
                          Originally posted by fjay View Post

                          Sphincter muscles are not typically affected by MND.
                          My understanding is that while full on failure of sphincter muscles is uncommon there is biochemical evidence of involvement and many MND patients experience mild weakness of the sort I am experiencing.

                          Comment

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