I've had a very bad week and need some help/advice

[Kitchener]

Hello All

This is my first post on this website, or any of this kind for that matter. I am in my early 40s with two great kids 8 & 5 and a really fantastic wife. At the beginning of last November (5 months ago) I started noticing twitching in my right bicep. It wasn't particularly severe but I thought it was curious anyway. I see my GP once a year for a check-up, due at the end of the November. During the check-up I mentioned the twitches which he described as fasciculations. He pretty much told me to ignore them but if they were bugging me after a while he would write up a referral to a neurologist. The twitching didn't go away over Christmas so in January I got a referral to a consulant neurologist in Perth, Western Australia.

In the five months since I started to notice twitching in my right bicep it has spread to my right deltoid, right lats, left deltoid and left tricep. The symptoms are much weaker on the left side. The intensity of the twitching hasn't actually changed much on the right side, maybe a little worse, maybe not. I am still strong eg. went water-skiing over the weekend. However, I have noticed a bit of strength deterioration in my right arm over the 5 months. I can still lift a beer carton with my right arm alone, but it is weaker than the left. It actually always has been, but I think it is fair to say there has been some deterioration after 5 months twitching. Also I do a handgrip test (one of those spring grips you get in gyms). I can hold it closed 2 minute no probs with my left hand, but only approx 45 secs with difficulty with my right.

Anyway, my neurologist has now seen me twice. He's not a particularly warm individual, about 60 years old. He first gave me a thorough check over, and sent me off for an MRI. He then conducted an EMG on me using both electrical shock and needles to the muscles. Summary results last week are as follows:

MRI - no damage around the neck, nerves exit spinal column cleanly
EMG - electrical tests show no issues with nerve sheaths; muscle tests show electrical spikes coincident with twitches in affected areas. Very slight suspicion that there may also be something going on in the legs, but very uncertain (flutters on EMG machine rather than spikes).

Based on this evidence, the neurologist's logic is as follows:
Twitches are not being caused by a neck issue
Twitches are not being caused by damage to individual "nerve channels" (axons) as they are fine
Therefore problem is nerve to muscle pathology
This means it is most likely a MND "of some sort"

He has freely admitted that he is not a dedicated expert in this particular area (understandable given the rarity of the condition, although I'm sure would know more than most) but he "can't see how it could be anything else". He is however organising a second opinion.

As you can imagine, I've had a very rough week since this conversation/diagnosis. My life was just fine before lunchtime last Wednesday, and I really didn't think I had a problem before the test.

My question to you & the advice I am seeking is as follows: is it possible that this neurologist has been a bit hasty in the diagnosis? The reading I have done would suggest that as MND is a diagnosis by exclusion, it can take a very long time to diagnose. It also looks like there are many variants, often overlapping, with subtle to significant differentials in prognosis.

I would be very grateful if anyone has a view on this. Many thanks.

 

[smwelder]

wow sorry you have to post here. there is many test that should be done . with ALS it's ruleing out every thing else under the sun then diagnosed can be given . my wife and i were told the only sure way to diagnosed is at Autopsy . get a second opinion. but if it is im so sorry be cause theres nothing that can change hearing thoes three letters ALS. all my best wishes to you brother as you walk this path of uncertainty . remember your never alone

 

[trfogey]

My question to you & the advice I am seeking is as follows: is it possible that this neurologist has been a bit hasty in the diagnosis? The reading I have done would suggest that as MND is a diagnosis by exclusion, it can take a very long time to diagnose. It also looks like there are many variants, often overlapping, with subtle to significant differentials in prognosis.

I would be very grateful if anyone has a view on this. Many thanks.

Seems very hasty to me. Is your description of your EMG and NCV (the electric shock test) what the neuro actually told you, or have you resummarized his summary?

The reason I ask this is that you don't describe anything wrong with you that shouts "MND". Twitches don't really mean anything unless they are accompanied by other symptoms such as clinical weakness, cramps, spasticity, or muscle atrophy. Even then, MND is only one of several differential diagnoses that the neuro would start to evaluate.

The "weakness" in your hand that you described doesn't sound like clinical weakness, but more like exercise intolerance or simply less muscular stamina or endurance in your nondominant hand (you are left-handed, aren't you?). A clinically weak hand wouldn't be able to close the handgrip, for example, while the other hand closes it easily.

Were there other things that the neurologist commented on or noted when he did his manual clinical exam -- pushing and pulling on on your arms, legs and neck, watching you wiggle your tongue around in your mouth, etc.? How about anything your GP has said?

Definitely get a second opinion and try not to worry about things until you've gotten the second opinion. It might not be a bad idea to talk to your GP and have him get the actual EMG results from the neuro for your and his direct review. You wouldn't be the first person we've seen here that had a neuro scare them to death with an offhand remark that he shouldn't have made.

 

[Kitchener]

Hi trfogey
Really appreciate the feedbackback. You are right - this guy has scared me and my wife enormously. He did a clinical examination with a few questions about lifestyle, took a bunch of reflexes and tested muscle strength manually. The EMG/NCV is my summary but I could see what was going on. He did stated that the NCV showed the axons were normal in 2 nerves he tested. The EMG with needles in muscle showed flickering spikes in the muscles that are fasciculating and nothing really noticeable in the ones that weren't. He spent a long time looking at the sceen when he had the needle in my leg muscles and I had a good view - there were no spikes when muscle relaxed, just a gentle sinusoid waveform with some slight fluttering. After the exam he said MND was really sneaky and there was "just a burr of something there"
He didn't do anything like measure muscle size, get me to waggle tongue or throw or catch a ball. I did have to stand on one leg - no probs. FYI right bicep = left bicep in circumference, and I have no co-ordination issues arms or legs at this time
In answer to the muscle weakness I am reasonably sure there has been some deterioration in the right arm, although I didn't notice it in day to day life, I only saw it when I checked after this "working diagnosis" (neuro's words). I am right handed for writing & throwing, left handed for pretty much everything else. He didn't ask what I was.
My real anxiety now following the dreadful week I have had and quest for knowledge is that I can't see why the neuro would go straight for MND unless he had a high level of conviction. What does he know that I don't? (apart from 13 years med school and 30 years practice experience?) I know there is a protocol about when ALS/MND should be considered and as far as I can tell I have no UMN symptoms and only a couple of LMN symptoms. An yet this august fellow has gone straight for the biggie - am I being naive and trying to grasp at straws? I am a big boy and if this is my fate I will meet it head on, but I truly believe the collective knowledge in this forum would dwarf a neuro's in Perth, Australia no matter how respected and august.

I really appreciate your coming back to me and help. Also thanks to smwelder for replying too - I'm humbled by your interest and willingness to help

 

[wright]

Hello Kitchener

In my opinion, your neuro should be beaten to within an inch of his life. Even if he suspected MND, he shouldn't have shared his thoughts. It is ethical to be honest with your patients but it is good bedside manner not to share such a thought if you are not qualified (and he says he isn't). It's like immediately saying you need a new engine in your car if it doesn't start (check the gas gauge first or any number of other things that are more probable).

Given your story and the tests he has done and the fact that most were normal, MND should not have been mentioned at all. Could it be one of the differential diagnoses? Yes, but in my opinion, a remote one. There are simply waaaaaaaaaaaaaay too many other things it could be and most likely is. He is correct: he is not an expert in this field of neurology, so he should leave it to those who are, which thankfully he has. For him to say that he doesn't know what else it could be, shows his knowledge is limited, because I assure you, it could be many, many other things (as I already stated).

Can I sit here and tell you that you don't have some type of MND? No, I cannot. However, I can sit here and tell you that what you have shared doesn't point directly towards it. Wait for the second opinion with someone who is qualified and do your best to forget about what your first neuro said. It truly shouldn't have been said, not just because it was cruel, but also because of the evidence at hand.

I wish you peace and please keep us informed.

 

[Kitchener]

Thanks Wright
Your words are very comforting. I have managed to get an appointment with a second neuro next week and I'll be sure to post after that, win or lose. At least I am armed with a lot more info this time around, so I'll be able to ask a few questions instead of sitting there like a pudding last time. FYI whilst the neuro was running through his thoughts with me and my wife, she started to faint and had to lie down in the next room. He just kept going without a pause. I guess he skipped the bedside manner part of his training....

 

[joni51]

Wow he is one bad Doctor is all I can say!

 

[Kitchener]

Hello all
Thanks so much for your help so far. I've just received a copy of the neuro's examination and EMG report. My GP is reluctant to say too much about it, and the neuro has referred me for a second opinion. To me it doesn't look great, but I would appreciate a view if anyone feels they can provide one.

The report which I will share with you proceeds as follows:

On examination: He weighed 77kgs (170lbs). There is persistent fasciculation in the right deltoid, biceps and forearm muscles, with occasional fasciculation in the left deltoid and right quadriceps seen. There was a hint of weakness in finger extension and flexion on the right and of abductor digiti minimi (outside of hand) and abductor pollicis brevis (base of thumb). Muscle tone was normal but reflexes were brisk in the limbs (my note: this has always been the case), both plantars downgoing. Cranial nerve examination was normal. He could jump on either leg alone without difficulty. The probel is more persistent than just benign fasciculation. There is a hint of weakness in the right finger flexors and extensors and intrinsic hand muscles.

EMG: INTERPRETATION OF RESULTS
1. The (R) median and ulnar motor and sensory conduction studies are normal
2. Needle examination shows widespread fasciculation and chronic partial denervation and reinnervation changes; more recent denervation (R) first dorsal interosseous in (R) & (L) arms, (R) leg and (L) sternomastoid

CONCLUSIONS
1. This EMG is consistent with a widespread low-grade denervation process with fasciculation at anterior horn cell level.
2. The implications will be discussed with the patient and his wife and a second opinion will be arranged.

So, all, what do you think? (I am big enough to handle bad news but I struggle with uncertainty). Please don't pull any punches if you have a firm opinion on the above. I was also told by the GP that neurological ailments can come in many forms and you can't tick all the boxes all the time; there are milder forms and severe forms of everything - is he right?

As ever, yur feedback is so appreciated

 

[laurel]

Wright is our expert in interpreting EMG reports etc. I'm sure he will see your latest post and comment. I'm not sure whether you have enough posts yet to private mail him, but you could try.
Laurel

 

[wright]

Hello Kitchener

It is still a bit difficult to interpret the results, because they were only done in your right arm (it would be nice to compare the other arm and legs) and I'm not able to see the raw data but I'll do my best to help you.

The EMG is saying that there is denervation, which is the loss of contact between axons of a nerve and muscle cells (axons are what make-up nerves . . . think of a nerve as a rope and the axons as the individual strings of that rope). The denervation is occuring in multiple muscles. Therefore, something is causing damage to multiple nerves.

Reinnervation is also taking place, which means when the damaged axons lose contact with their muscle cells, other healthy axons (of that same nerve) take over for them. This is also taking place in multiple areas. Reinnervation typically takes months to be detected by an EMG.

The nerve conduction study states that the sensory nerves have not been damaged and it also states that the insulation around those sensory nerves is intact. The insulation around the motor nerves (motor nerves are those nerves that contact your muscles) is intact as well. I know this because it states the nerve conduction study is normal.

Overall interpretation:

Something is damaging just the axons of your motor nerves and it has been going on for some time. That could be caused by a number of things . . . and yes . . . ALS can be one of those things, so that differential is on the table but so too will be a number of neuropathies. You're not going to know until you get that second opinion from the neuromuscular neuro. I wish I could have simply told you ALS is off of the table but it still doesn't mean you have it.

Keep in mind that you can't have ALS until you have been diagnosed with ALS, so remain hopeful. I wish you peace during this time and please write to us whenever you feel the need.

 

[Marjorie R. Wilcox]

From the terms you used in your post and the things you mentioned, I would only guess, mind you, that you might just be exercising too much which can cause the twitches. Strained muscles do strange things. It sounds like you keep testing your strength with weights and talk about lifting a "beer carton". It sounds like you have frequent workouts at the gym. It just might be that... Long long ago when I tried out for cheerleading... all the practices of high jumps caused my legs to twitch and they gave out when I went downstairs. After a while I adjusted. I too, think your neurologist should have tested instead of voicing his opinion. Doesn't sound like a MND

 

[laurel]

Kitchener please check in with us after you see the specialist neurologist. Best of luck! My husband had a differential of ALS and finally was diagnosed with CIDP--not an easy disease but treatable. I'll be keeping my fingers crossed and saying a prayer.
Laurel

 

[Kitchener]

Hi Wright
I really appreciate the feedback, it's a great help. I do have the raw data as well, but as I'm getting my second opinion this week it'll probably be that data I will be posting shortly. Your explanation is much clearer than the one I had from my neuro and I value the candour. I understand ALS is very much on the table from the EMG, but the difference between you and my first neuro is that you believe that there are a number of other neuropathies possible, whereas he said "I just don't see what else it could be". I like your approach much better!
Anyway, I'll post again later in the week with an update to the drama.
Thanks also to Marjorie and Lauren for posting too. While you guys don't know me your support is really appreciated and I thank you for it.

 

[Kitchener]

Hello All
I had my appointment with the peripheral neurology specialist today, and good-ish news I suppose. She didn't totally agree with my first neuro, but didn't totally disagree either. She reckons its 50:50 motor neurone vs some other neuropathy. Her view was based on the fact I have no UMN symptoms at all, and she wasn't sold on the EMG report as far as it pertained to my legs. Offsetting this she believes there was somectable weakness in my right hand. I am due for another EMG focussing on the possibility of conduction block in the arms (whilst my first conduction test was normal, she wasn't happy that the nerves used for the test were particularly relevant and didn't rule out neuropathy in other nerves). Also I'm up for a lumbar puncture to check if there is inflammation and/or an auto-immune issue. All this accompanied with a bit of bloodwork to get a couple of pieces of the jigsaw the first neuro didn't think he needed.
Interesting she said I was "funny" - in that I didn't really fit in any neat boxes. She certainly seemed a lot more on the ball than the other chap.
So in summary it seems I'm still work in progress, and I have certainly a lot more hope than this time last week. I fully understand that I could be put straight on my backside if the new tests don't help out with the diagnosis and in fact firm the odds up towards ALS. However, I'll take each day as it comes.....

 

[laurel]

Fingers crossed for you Kitchener. The neuro. sounds on the ball. Let's hope for something like MMN or CIDP. Keep us posted please. Continuing to say prayers for you.
Laurel