Dr Saghafi, Would Tightness In My Arm That Is Showing

Brief Answer:
Not an UPPER MOTOR SIGN

Detailed Answer:
For all the identical reasons I mentioned in my other note to you a few moments ago....your sense of TIGHTNESS and TIREDNESS after using the limbs as well as the sense of IMBALANCE between arms in terms of each limb's ability to apply force is a very clear and present NEUROLOGICAL SYMPTOM of some type of neurological dysfunction. These are not NEUROLOGICAL SIGNS of an UPPER MOTOR NEURON TYPE.

It is very difficult to conduct a valid and fully UNBIASED neurological examination on oneself.....Even a NEUROLOGIST examining himself can be fraught with bias, prejudice, and skewed results and should not be undertaken. Physical examinations should always be performed by someone other than the the person suffering from the problems. Make sense?

Cheers Kind Sir!

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Brief Answer:
Not an UPPER MOTOR SIGN

Detailed Answer:
For all the identical reasons I mentioned in my other note to you a few moments ago....your sense of TIGHTNESS and TIREDNESS after using the limbs as well as the sense of IMBALANCE between arms in terms of each limb's ability to apply force is a very clear and present NEUROLOGICAL SYMPTOM of some type of neurological dysfunction. These are not NEUROLOGICAL SIGNS of an UPPER MOTOR NEURON TYPE.

It is very difficult to conduct a valid and fully UNBIASED neurological examination on oneself.....Even a NEUROLOGIST examining himself can be fraught with bias, prejudice, and skewed results and should not be undertaken. Physical examinations should always be performed by someone other than the the person suffering from the problems. Make sense?

Cheers Kind Sir!

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Brief Answer:
Labs and CT scan are not appearing as uploaded on my end

Detailed Answer:
I so very sorry that you are feeling this way about what's going on. Wish there was more to offer you from 600 miles away except to repeat what I've said previously which is that it certainly would appear that something might be going on and there's little doubt that you have more symptoms and things causing you to feel that something very aggressive is afoot organically but it's really not hanging as part of any of the common motor neuron, dystrophic, or other neurodegenerative diseases that I've seen or diagnosed.

So for what it's worth I have to stick with my original list of diagnostic possibilities which does NOT INCLUDE the likely chance of ALS, PMA, or SMA. I am certainly of the opinion that the videos you sent showed fasciculations but there are so many more likely things to cause those fasciculations than ALS...as you know.....so if there were a way that could have a talk session with yourself and allow the picture to play out without any rush to judgement until you can be seen by a neuromuscular person who I think your other neurologist was going to set up for you later on this month....then, you may find some improvement in your symptoms....

Perhaps, you can contact TECHNICAL SUPPORT on this platform to help you discover the way of attaching files so that I can see them as well. I will also tell you that I'm not at all great to read CT scans of kidneys or abdominal organs...bo brother...DO NOT EVEN TRY to upload images of your belly or pelvis and expect to know what the heck I'm looking at unless it's labeled! LOL! I will believe any radiology report that since things look good....and that of course, is definitely solidly good news.

BTW, I get cramping as well in my hands (usually the one I write with) as I'm becoming like the aging cheese in the barrel in Roquefort, England. It's not a concern of mine that the condition is much more common and takes much less effort for my fingers to extend and twist for a minute until either I straighten them out or they release on their own. There are lots of reasons for muscles to become dystonic in that fashion, it is not just an upper motor phenomenon. You could see that more easily if you learned the therapeutic technique of BIOFEEDBACK and then, see if that could work for you in order to turn down the gain on at least the fasciculations if not the feelings of muscle tightness.

Check this article's abstract out....very interesting. I've not read the entire article and have requested a copy of the full paper from the authors. But perhaps, you can relate to the abstract's content- "Another Perspective on Fasciculations When is it not Caused by the Classic Form of Amyotrophic Lateral Sclerosis or Progressive Spinal Atrophy":

https://www.researchgate.net/publication/0000_Another_Perspective_on_Fasciculations_When_is_it_not_Caused_by_the_Classic_form_of_Amyotrophic_Lateral_Sclerosis_or_Progressive_Spinal_Atrophy

BTW, I'd like to make sure you are fully clear on the fact that sending me messages through this platform as you've done is NOT A BOTHER, never was, and never will be...I'm available to answer questions and give opinions if you seek them. I'm just as interested is getting to the bottom of things as you are at this point so all information is good...just gotta keep an open mind to my perspective on things and not be irritated that I'm not willing to change an opinion since I'm simply playing by the information I've gathered on you personally by examining you, HEARING about lab/imaging results, and listening to you tell the story as to the way things have progressed over the past 14 weeks.

If I were to find evidence or hear something that would cause me to want to revise my opinion I promise you'll be the first to know. :0

Cheers Kind Sir!

This query required 60 min. to read, research, analyze, and prepare response.

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Brief Answer:
Labs and CT scan are not appearing as uploaded on my end

Detailed Answer:
I so very sorry that you are feeling this way about what's going on. Wish there was more to offer you from 600 miles away except to repeat what I've said previously which is that it certainly would appear that something might be going on and there's little doubt that you have more symptoms and things causing you to feel that something very aggressive is afoot organically but it's really not hanging as part of any of the common motor neuron, dystrophic, or other neurodegenerative diseases that I've seen or diagnosed.

So for what it's worth I have to stick with my original list of diagnostic possibilities which does NOT INCLUDE the likely chance of ALS, PMA, or SMA. I am certainly of the opinion that the videos you sent showed fasciculations but there are so many more likely things to cause those fasciculations than ALS...as you know.....so if there were a way that could have a talk session with yourself and allow the picture to play out without any rush to judgement until you can be seen by a neuromuscular person who I think your other neurologist was going to set up for you later on this month....then, you may find some improvement in your symptoms....

Perhaps, you can contact TECHNICAL SUPPORT on this platform to help you discover the way of attaching files so that I can see them as well. I will also tell you that I'm not at all great to read CT scans of kidneys or abdominal organs...bo brother...DO NOT EVEN TRY to upload images of your belly or pelvis and expect to know what the heck I'm looking at unless it's labeled! LOL! I will believe any radiology report that since things look good....and that of course, is definitely solidly good news.

BTW, I get cramping as well in my hands (usually the one I write with) as I'm becoming like the aging cheese in the barrel in Roquefort, England. It's not a concern of mine that the condition is much more common and takes much less effort for my fingers to extend and twist for a minute until either I straighten them out or they release on their own. There are lots of reasons for muscles to become dystonic in that fashion, it is not just an upper motor phenomenon. You could see that more easily if you learned the therapeutic technique of BIOFEEDBACK and then, see if that could work for you in order to turn down the gain on at least the fasciculations if not the feelings of muscle tightness.

Check this article's abstract out....very interesting. I've not read the entire article and have requested a copy of the full paper from the authors. But perhaps, you can relate to the abstract's content- "Another Perspective on Fasciculations When is it not Caused by the Classic Form of Amyotrophic Lateral Sclerosis or Progressive Spinal Atrophy":

https://www.researchgate.net/publication/0000_Another_Perspective_on_Fasciculations_When_is_it_not_Caused_by_the_Classic_form_of_Amyotrophic_Lateral_Sclerosis_or_Progressive_Spinal_Atrophy

BTW, I'd like to make sure you are fully clear on the fact that sending me messages through this platform as you've done is NOT A BOTHER, never was, and never will be...I'm available to answer questions and give opinions if you seek them. I'm just as interested is getting to the bottom of things as you are at this point so all information is good...just gotta keep an open mind to my perspective on things and not be irritated that I'm not willing to change an opinion since I'm simply playing by the information I've gathered on you personally by examining you, HEARING about lab/imaging results, and listening to you tell the story as to the way things have progressed over the past 14 weeks.

If I were to find evidence or hear something that would cause me to want to revise my opinion I promise you'll be the first to know. :0

Cheers Kind Sir!

This query required 60 min. to read, research, analyze, and prepare response.

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Brief Answer:
Reports reviewed

Detailed Answer:
Thanks for attaching the reports (multiple times!) HA! And again, thank you for continuing to funnel me your questions. I wish there were a bit more I could help with but that would involve the State of Ohio having to invade and conquer the territories of Pennsylvania, New York, and Connecticut so that my license would be valid to practice to your borders! HAHA!

It's noted that your vitamin D from April which is very low and we may have spoken about this before.

I always correct all of our neurology patients (no matter what may be going on with them) and shoot for a target of 60-80 ng/ml. That means in your case I would prescribe for you weekly gelcaps of cholecalciferol (D3) of 50,000 IU's x 8 weeks then, start a daily regimen of 2000 IU. I'd repeat another serum panel in 8-10 weeks and adjust as necessary. Remember, vitamin is a hormone and like all hormones there is a "sweet spot" for them to be optimally effective in an individual.

The rest of the labs seem to be in order just as you said including calcium, thyroid hormones, pancreatic enzymes, etc.

As far as the pill not going down is concerned...I have similar trouble at times with tablets or capsules if they're over a certain size or if I've not drunk fluids and the mouth is dry. So long as you're not drooling and able to swallow saliva without it going down the wrong way (i.e. choking) then, swallowing your pills will not result in anything going down the trachea...that you can be sure of....I always try flushing down with some liquid. Sometimes that even doesn't work because I just have this thing in my mind about pills above a certain size and even when I know I've got fluid in there....it's funny but the damn pill always seems to get STOPPED when I swallow everything else....it's a little hard to explain. So then, I'll resort to getting a little yogurt, apple sauce, pudding....or something creamy and stick the pill somewhere in there....I've even asked my wife to do it so I can't SEE where it is...and then, I just use a spoon and GULP it down like a DOG! LOL....

If that still doesn't work then, I'll find out if the pill can be crushed (call pharmacist) or if a cap whether or not you can just unscrew the capsule and sprinkle on something....that usually works.

If you are still feeling you're having trouble with swallowing after all those tricks then, your doctor may wish to send you for a swallowing evaluation using the old barium or cookie swallow routine. Again, in general the rule of thumb is that if patients can swallow anything comfortably and there's no drooling or choking on saliva then, it is highly unlikely that a swallow test will indicate any form of aspiration of food on the test and it just becomes a matter of finding a way to slice, dice, crush,, or otherwise, make more manageable items for swallowing purposes.

Your friendly local GI docs may have other tricks up their sleeves but NEUROGENIC PHARYNGEAL OR ESOPHAGEAL WEAKNESS is clinically ruled out if liquids are going down (which includes saliva) without aspiration complications...make sense?

This query required 85 min. to read, research, analyze, and prepare response.

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Brief Answer:
Reports reviewed

Detailed Answer:
Thanks for attaching the reports (multiple times!) HA! And again, thank you for continuing to funnel me your questions. I wish there were a bit more I could help with but that would involve the State of Ohio having to invade and conquer the territories of Pennsylvania, New York, and Connecticut so that my license would be valid to practice to your borders! HAHA!

It's noted that your vitamin D from April which is very low and we may have spoken about this before.

I always correct all of our neurology patients (no matter what may be going on with them) and shoot for a target of 60-80 ng/ml. That means in your case I would prescribe for you weekly gelcaps of cholecalciferol (D3) of 50,000 IU's x 8 weeks then, start a daily regimen of 2000 IU. I'd repeat another serum panel in 8-10 weeks and adjust as necessary. Remember, vitamin is a hormone and like all hormones there is a "sweet spot" for them to be optimally effective in an individual.

The rest of the labs seem to be in order just as you said including calcium, thyroid hormones, pancreatic enzymes, etc.

As far as the pill not going down is concerned...I have similar trouble at times with tablets or capsules if they're over a certain size or if I've not drunk fluids and the mouth is dry. So long as you're not drooling and able to swallow saliva without it going down the wrong way (i.e. choking) then, swallowing your pills will not result in anything going down the trachea...that you can be sure of....I always try flushing down with some liquid. Sometimes that even doesn't work because I just have this thing in my mind about pills above a certain size and even when I know I've got fluid in there....it's funny but the damn pill always seems to get STOPPED when I swallow everything else....it's a little hard to explain. So then, I'll resort to getting a little yogurt, apple sauce, pudding....or something creamy and stick the pill somewhere in there....I've even asked my wife to do it so I can't SEE where it is...and then, I just use a spoon and GULP it down like a DOG! LOL....

If that still doesn't work then, I'll find out if the pill can be crushed (call pharmacist) or if a cap whether or not you can just unscrew the capsule and sprinkle on something....that usually works.

If you are still feeling you're having trouble with swallowing after all those tricks then, your doctor may wish to send you for a swallowing evaluation using the old barium or cookie swallow routine. Again, in general the rule of thumb is that if patients can swallow anything comfortably and there's no drooling or choking on saliva then, it is highly unlikely that a swallow test will indicate any form of aspiration of food on the test and it just becomes a matter of finding a way to slice, dice, crush,, or otherwise, make more manageable items for swallowing purposes.

Your friendly local GI docs may have other tricks up their sleeves but NEUROGENIC PHARYNGEAL OR ESOPHAGEAL WEAKNESS is clinically ruled out if liquids are going down (which includes saliva) without aspiration complications...make sense?

This query required 85 min. to read, research, analyze, and prepare response.

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Brief Answer:
Variable times of progression for confirmed BULBAR forms of ALS

Detailed Answer:
Good morning Sir- Answering your questions on bulbar ALS in terms of speed of progression. Studies are really variable on that subject since this form of ALS is thought to be only 1/3 as frequent as ALS itself. This means that instead of the usual 1/100,000 incidence rate that is frequently quoted for the most common form of ALS seen in the spectrum of motor neuron diseases the rarity of the bulbar form is believed to be in the 1/300,000 neighborhood. Also, across most studies you will find that the majority of people with this form of ALS are women (at least 65-70%) and the median age of ONSET to the disease is in the 6th decade of life (68 years). Furthermore, because it is seen so very rarely as its own entity the time between what is thought to be symptom onset and diagnosis can be as little as 3 months or several years depending upon how diligent patients are to bring themselves to medical attention and also how diligent doctors are to recognize patients that need referral to ALS centers for definitive diagnosis.

However, of the several epidemiological reports I've reviewed for you I'm seeing an average time of progression from what is likely symptom onset through the course of the entire disease process as about 40 months.

Of course, you know that I've not had a chance to see or examine you since our initial encounter in April which is barely 2 months ago and at that time (even with the FLAGS raised having to do with fasciculations in the calf muscle in the right leg and the other concerns you initially had) there were no signs of any neurodegenerative disease of the ALS type so any way you want to dice it at this point your progression of the rest of the symptoms you're describing having now to do with a lot of head and neck muscle involvement would be considered extraordinarily rapid when compared to the known epidemiology of bulbar ALS even if we were to assume that in fact, that's what might be going on to explain your symptoms.

I agree that the bleeding in the stool is not a related phenomenon and I believe represents hemorrhoidal issues. If the blood you are referring to is actually INCLUDED in the stool making it tarry black or something of that nature then, that's a different story and you'd want to consult your GI doctor about specifically testing for that entity....but in any event it wouldn't have anything to do with the primary set of symptoms at hand that we've been discussing having to do with fasciculations, limb weakness, etc. You need to get a lot more roughage into your diet to get that constipation under control. 3-5 good portion sizes of green leafy vegetables daily with a little balsamic vinaigrette will take hold in a matter of 2-3 days! :0

I hope those tidbits of information answer the questions you've posed Kind Sir and as always you know the score....It's that GOOD NEWS/BAD NEWS thing....right? Ha!

The Good News is that I'm available to answer your questions and help you along any time you need it..... The Bad News is that I'm 600 miles off your front porch and they won't let me out of the hospital to come out and say, "Hey!" LOL.....but hey, the trees and flowers are really starting to bloom now down here in the LAND....so you know the door's always open.

Your commentary and critiques are always highly regarded and appreciated.

This query has thus far required 135 min. to read, research, analyze, and prepare response.

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Brief Answer:
Variable times of progression for confirmed BULBAR forms of ALS

Detailed Answer:
Good morning Sir- Answering your questions on bulbar ALS in terms of speed of progression. Studies are really variable on that subject since this form of ALS is thought to be only 1/3 as frequent as ALS itself. This means that instead of the usual 1/100,000 incidence rate that is frequently quoted for the most common form of ALS seen in the spectrum of motor neuron diseases the rarity of the bulbar form is believed to be in the 1/300,000 neighborhood. Also, across most studies you will find that the majority of people with this form of ALS are women (at least 65-70%) and the median age of ONSET to the disease is in the 6th decade of life (68 years). Furthermore, because it is seen so very rarely as its own entity the time between what is thought to be symptom onset and diagnosis can be as little as 3 months or several years depending upon how diligent patients are to bring themselves to medical attention and also how diligent doctors are to recognize patients that need referral to ALS centers for definitive diagnosis.

However, of the several epidemiological reports I've reviewed for you I'm seeing an average time of progression from what is likely symptom onset through the course of the entire disease process as about 40 months.

Of course, you know that I've not had a chance to see or examine you since our initial encounter in April which is barely 2 months ago and at that time (even with the FLAGS raised having to do with fasciculations in the calf muscle in the right leg and the other concerns you initially had) there were no signs of any neurodegenerative disease of the ALS type so any way you want to dice it at this point your progression of the rest of the symptoms you're describing having now to do with a lot of head and neck muscle involvement would be considered extraordinarily rapid when compared to the known epidemiology of bulbar ALS even if we were to assume that in fact, that's what might be going on to explain your symptoms.

I agree that the bleeding in the stool is not a related phenomenon and I believe represents hemorrhoidal issues. If the blood you are referring to is actually INCLUDED in the stool making it tarry black or something of that nature then, that's a different story and you'd want to consult your GI doctor about specifically testing for that entity....but in any event it wouldn't have anything to do with the primary set of symptoms at hand that we've been discussing having to do with fasciculations, limb weakness, etc. You need to get a lot more roughage into your diet to get that constipation under control. 3-5 good portion sizes of green leafy vegetables daily with a little balsamic vinaigrette will take hold in a matter of 2-3 days! :0

I hope those tidbits of information answer the questions you've posed Kind Sir and as always you know the score....It's that GOOD NEWS/BAD NEWS thing....right? Ha!

The Good News is that I'm available to answer your questions and help you along any time you need it..... The Bad News is that I'm 600 miles off your front porch and they won't let me out of the hospital to come out and say, "Hey!" LOL.....but hey, the trees and flowers are really starting to bloom now down here in the LAND....so you know the door's always open.

Your commentary and critiques are always highly regarded and appreciated.

This query has thus far required 135 min. to read, research, analyze, and prepare response.

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Brief Answer:
Explanation of symptoms still better by something OTHER than MND

Detailed Answer:
Good morning Kind Sir and thank you for these additional clarifications as there are several things that support the contention that you are less likely suffering from some form of MND such as ALS especially the bulbar form since you are seeming to have more significant symptoms now in the head and neck compared to the limb and trunks....and here are the points I would look at in what you've written compared to what we know so far.....

"....my voice has been hoarse now for at least 14 weeks or more, (I noticed it in XXXXXXX when we had small talk before I left. I had been up for 36 hours). It’s sometimes slightly more noticeable than other times. I attributed it to stress and lack of sleep. I’ve also noticed my jaw cracking and things of that nature. "

>>>>> Of course, I never heard your voice until the first time we spoke by telephone before you came to XXXXXXX and I did not hear any evidence of problems with PHONATION (i.e. hoarseness). At our last conversation I don't recall noting anything significantly different about your voice compared to when we first spoke. If this were to be pegged as ALS and more specifically the BULBAR subtype which is known to primarily exhibit symptoms of head and neck problems to include hoarseness, swallowing difficulties, etc. before making its way downward....then, why no notable change in your voice in a 4-6 week time span between the first and last times we spoke by phone? Also, the quality of your voice is definitely affected by other factors (as you mentioned) such as fatigue, going many hours without sleep or good sustenance/hydration, upper respiratory viral syndromes which have been rampant so far this spring. Another important thing you remark is that "sometimes it is SLIGHTLY more noticeable than other times." Conventional wisdom regarding the progression of MND is that it never really the case to have "good days and bad days" with the disease. It is ongoing and relentless and it is just a matter of speed of progression but things aren't better or worse nor do people with MND typically tell us that an attributable is more or less noticeable on a day by day basis....

And so for all these reasons I'm not able to place this symptom into any type of probable or definite category for MND or ALS.

Jaw cracking is not a recognized part of the evolution of MND in my experience with diagnosing and managing patients confirmed to have the disorder.


"How could all this change swallowing wise be so noticable since Memorial Day weekend?"

>>>>>>> ALS appears slowly and progressively. Swallowing problems that occur as yours have within a matter of days from perfectly normal as when I saw and last spoke to you on the phone when there were no complaints of deglutition to now when this has become a primary problem can only happen in a few disorders such as STROKE, or ACUTE STREPTOCOCCAL PHARYNGITIS, TRAUMA TO THE NECK resulting in acute swelling, etc. Otherwise, the bulbar dysfunction which is the basis for NEUROGENIC SWALLOWING DYSFUNCTION is a longer and more protracted course of evolution. Patients will generally notice occasional coughing or the unusual uptick in frequency of things going down the wrong way and then, this over the course of several months or more more noticeable abnormalities such as drooling may appear before it then, becomes more apparent that passing boluses of food and liquid are either clearly more effortful or result in laryngeal penetration with later It should be evaluated by a swallowing evaluation test which can actually "see" small amounts of food penetration into places (even in the absence of coughing or choking) that can confirm the incompetency of sphincter muscles necessary to keep food going down the pipe and can detect actual first phase swallowing difficulties in passing food from the oral cavity to the back of the mouth where reflex deglutition takes over. As far as you've revealed to me in the past couple of weeks since complaining of this as a major symptom I've not heard of the appearance of any other PREMONITORY symptom to the XXXXXXX difficulty with passing food nor has a swallowing evaluation been performed.

Therefore, again, based upon available information or lack thereof, I'm unable to place this symptom into a category of significance when it comes to supportive evidence for the presence of a MND type such as ALS and especially of the bulbar form where swallowing dysfunction is a major feature of the disease.

"How could my tongue have a noticeable twitch in it one day that can be actually seen then not appear the next. Then, come back to twitch at the very, very tip of my tongue when I feel completely stressed out?"

>>>>>Please recall that I've commented in the past that tongue fibrillations or fasciculations are one of the most difficult physical markers for patients to properly and adequately visualize and detect of all the fasciculation activity associated with both MND and NON-MND disease states. It really takes a particular position of the tongue in the mouth and a trained professional such as a neurologist to make sure that the tongue is not being somehow engaged so that it is not perfectly relaxed and then, to be able to detect INVOLUNTARY from VOLUNTARY movements of the tongue or perhaps more appropriately, SUBCONSCIOUS movements that are NOT FASCICULATIONS. The best statement you could've made to illustrate this point is the one you did when you mention the confusion you are feeling based on twitches that come and go day to day (we already discussed this issue in a previous statement up top). Also, another very commonly related thing that people worried about ALS talk about (which does not tend to happen in real life) is that fasciculations occur at the tip of the tongue.

They rarely are seen that far into the periphery of the muscle...they are much more likely to occur and be seen in the belly of the muscle. Also, tongue fibrillations or fasciculations are typically imperceptible to patients because the fibers involved are so small compared to the size of other muscle bundles in the arms and legs that one does not really sense when it's happening. Since the tongue is not PHYSICALLY MOVED in the mouth by the activation of only a few fibers it is very difficult for the body to be aware of the movements. They really need to be visualized and unless someone is being seen in the very earliest part of the disease process- usually by the time fasciculation activity is picked up by doctors in the tongue there is usually atrophy which can be noticed as well due to the time it takes for denervation to take place.

Another very important point you mention at the end of that whole statement is "Then, come back to twitch at the very, very tip of my tongue when I feel completely stressed out?" In my opinion, this is the recognition that there is a psychological trigger of STRESS that can bring on twitches you are seeing or feeling in the tongue which at other times do not exist. We do not see this type of "come and go" phenomenon with MND and especially not ALS of the bulbar or any other type. When it's there, it's there and it's only a matter of speed of progression. If it's coming and going then, in my opinion, "It ain't there." Make sense?

And so for all the reasons above I cannot reconcile this symptom as being part of a probable or definite issue related to a MOTOR NEURON PROBLEM of any sort such as ALS or any of its subtypes.


I did address something on the link you sent me regarding the difference between fascics seen in ALS and those seen in benign cases of fascics such as BFS. I also appreciate the article you sent for me to read and have added it to my library of resource information. It will serve well in some areas of discussion. However, I think I did also mention the disagreement I have with some of what they've explained regarding the timing of fasciculations in ALS as being one of the earliest and even "initial" sign noticed in ALS. This is stated in the abstract and I do take exception to this from both an experiential as well as literature point of view. Actually, to be fair, fasciculations generate a lot of arguing in the medical community and that's a shame. That's because the public focuses so intensely on it and are looking for definite position statements. However, the hell of it is that fasciculations have never truly been FULLY UNDERSTOOD in terms of what they are and how to distinguish them when involving actually DENERVATION from their target vs. IRRITATION of the nerve that doesn't necessarily cause denervation to occur but where there is sufficient blockade of normal electrical conductance so as to give the appearance or effect of denervation. Numerous investigators have made claims that telling the differences in waveforms using various techniques of ultrasound or EMG can separate things such as BFS and ALS....but the truth is there is no universally accepted protocol and MND still remains a CLINICALLY DIAGNOSED condition with the utilization of tests and studies as a way of EXCLUDING other potential causes.

So again, I want to underscore just exactly what the role is of your upcoming electrical study and how much more importance is given to the clinical examination and the neurological signs found as well as the history of the symptoms when it comes to making a diagnosis. Of course, one can always throw in PATHOLOGICAL DIAGNOSIS as a "wild card" test if you will and some may choose to argue that it is the FINAL STEP in being able to finally say YEA or NAY....and in truth....it is NOT. Pathological diagnosis by surgically excising both nerve and muscle samples to send for testing will only be useful in the VAST MAJORITY OF CASES....if taken from an individual who is already CLINICALLY suspected as having a probable or definite form of MND. In otherwise, "MND FREE" subjects....pathology will always come out NEGATIVE for the disease which is of no XXXXXXX since clinically there was never any suspicion.

So please don't think that if someone offers you a biopsy to PROVE the presence or absence of disease that you should jump on that band wagon.....because all you're doing is donating good tissue that you could better use for the rest of your life in moving your arm or leg...maybe you're helping a newbie lab technician make some block samples for staining and for that reason you'll have contributed in some material way to the advancement of medical science....but personally, I'd prefer to let that new kid get his experience elsewhere instead of loading up normal tissue of mine to go under a microscope! LOL....

Your final question of whether or not I've noticed a continual pattern of firing in fasciculations in BFS? I've seen such a variety of patterns of BFS that I can tell you that there is nothing that can be noticed in a patient who has this condition different from an ALS patient except to say that typically if I see a patient with full body twitching and no obvious signs of muscle atrophy, neurogenic weakness, or the presence of upper or lower motor neuron signs then, I'm already thinking in terms of BFS and not ALS....but not so much by the pattern or firing synchrony of the fasciculations...but by the lack of other salient features of a motor neuron problem. If I'm seeing a patient for the first time with fasciculations that are localized or otherwise restricted to a small region of a limb but may be continuously firing....(e.g. eyelid)....again, I'm not jumping to any conclusions about ANYTHING until more history and examination taken place. But continuous vs. non-continuous firing doesn't really enter my mind so much as whether or not UPPER and/or LOWER MOTOR SIGNS are present.

That is in part where I (and others) diverge with some of what is written in this article about any type of synchronous firing of fasciculations vs. not and the whole concept of fasciculations being an INITIAL symptom of ALS. I will need to see many more authors supporting that claim by data because that really does fly in the face of conventional wisdom which has held since the earliest days of description of ALS and the other since then, recognized forms of MND.

Whew....well, that was a marathon, wasn't it.....hope it made sense. I corrected all the grammar and orthography I could along the way but I just want to get this off to you so I can get to the hospital now and deal with some other issues (vets to be sure!)....take care as always Kind Sir.....and let me know if there's anything more I can do for you between now and whenever.

Cheers!

This query has thus far required 255 min. to read, research, analyze, and prepare response.

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Brief Answer:
Explanation of symptoms still better by something OTHER than MND

Detailed Answer:
Good morning Kind Sir and thank you for these additional clarifications as there are several things that support the contention that you are less likely suffering from some form of MND such as ALS especially the bulbar form since you are seeming to have more significant symptoms now in the head and neck compared to the limb and trunks....and here are the points I would look at in what you've written compared to what we know so far.....

"....my voice has been hoarse now for at least 14 weeks or more, (I noticed it in XXXXXXX when we had small talk before I left. I had been up for 36 hours). It’s sometimes slightly more noticeable than other times. I attributed it to stress and lack of sleep. I’ve also noticed my jaw cracking and things of that nature. "

>>>>> Of course, I never heard your voice until the first time we spoke by telephone before you came to XXXXXXX and I did not hear any evidence of problems with PHONATION (i.e. hoarseness). At our last conversation I don't recall noting anything significantly different about your voice compared to when we first spoke. If this were to be pegged as ALS and more specifically the BULBAR subtype which is known to primarily exhibit symptoms of head and neck problems to include hoarseness, swallowing difficulties, etc. before making its way downward....then, why no notable change in your voice in a 4-6 week time span between the first and last times we spoke by phone? Also, the quality of your voice is definitely affected by other factors (as you mentioned) such as fatigue, going many hours without sleep or good sustenance/hydration, upper respiratory viral syndromes which have been rampant so far this spring. Another important thing you remark is that "sometimes it is SLIGHTLY more noticeable than other times." Conventional wisdom regarding the progression of MND is that it never really the case to have "good days and bad days" with the disease. It is ongoing and relentless and it is just a matter of speed of progression but things aren't better or worse nor do people with MND typically tell us that an attributable is more or less noticeable on a day by day basis....

And so for all these reasons I'm not able to place this symptom into any type of probable or definite category for MND or ALS.

Jaw cracking is not a recognized part of the evolution of MND in my experience with diagnosing and managing patients confirmed to have the disorder.


"How could all this change swallowing wise be so noticable since Memorial Day weekend?"

>>>>>>> ALS appears slowly and progressively. Swallowing problems that occur as yours have within a matter of days from perfectly normal as when I saw and last spoke to you on the phone when there were no complaints of deglutition to now when this has become a primary problem can only happen in a few disorders such as STROKE, or ACUTE STREPTOCOCCAL PHARYNGITIS, TRAUMA TO THE NECK resulting in acute swelling, etc. Otherwise, the bulbar dysfunction which is the basis for NEUROGENIC SWALLOWING DYSFUNCTION is a longer and more protracted course of evolution. Patients will generally notice occasional coughing or the unusual uptick in frequency of things going down the wrong way and then, this over the course of several months or more more noticeable abnormalities such as drooling may appear before it then, becomes more apparent that passing boluses of food and liquid are either clearly more effortful or result in laryngeal penetration with later It should be evaluated by a swallowing evaluation test which can actually "see" small amounts of food penetration into places (even in the absence of coughing or choking) that can confirm the incompetency of sphincter muscles necessary to keep food going down the pipe and can detect actual first phase swallowing difficulties in passing food from the oral cavity to the back of the mouth where reflex deglutition takes over. As far as you've revealed to me in the past couple of weeks since complaining of this as a major symptom I've not heard of the appearance of any other PREMONITORY symptom to the XXXXXXX difficulty with passing food nor has a swallowing evaluation been performed.

Therefore, again, based upon available information or lack thereof, I'm unable to place this symptom into a category of significance when it comes to supportive evidence for the presence of a MND type such as ALS and especially of the bulbar form where swallowing dysfunction is a major feature of the disease.

"How could my tongue have a noticeable twitch in it one day that can be actually seen then not appear the next. Then, come back to twitch at the very, very tip of my tongue when I feel completely stressed out?"

>>>>>Please recall that I've commented in the past that tongue fibrillations or fasciculations are one of the most difficult physical markers for patients to properly and adequately visualize and detect of all the fasciculation activity associated with both MND and NON-MND disease states. It really takes a particular position of the tongue in the mouth and a trained professional such as a neurologist to make sure that the tongue is not being somehow engaged so that it is not perfectly relaxed and then, to be able to detect INVOLUNTARY from VOLUNTARY movements of the tongue or perhaps more appropriately, SUBCONSCIOUS movements that are NOT FASCICULATIONS. The best statement you could've made to illustrate this point is the one you did when you mention the confusion you are feeling based on twitches that come and go day to day (we already discussed this issue in a previous statement up top). Also, another very commonly related thing that people worried about ALS talk about (which does not tend to happen in real life) is that fasciculations occur at the tip of the tongue.

They rarely are seen that far into the periphery of the muscle...they are much more likely to occur and be seen in the belly of the muscle. Also, tongue fibrillations or fasciculations are typically imperceptible to patients because the fibers involved are so small compared to the size of other muscle bundles in the arms and legs that one does not really sense when it's happening. Since the tongue is not PHYSICALLY MOVED in the mouth by the activation of only a few fibers it is very difficult for the body to be aware of the movements. They really need to be visualized and unless someone is being seen in the very earliest part of the disease process- usually by the time fasciculation activity is picked up by doctors in the tongue there is usually atrophy which can be noticed as well due to the time it takes for denervation to take place.

Another very important point you mention at the end of that whole statement is "Then, come back to twitch at the very, very tip of my tongue when I feel completely stressed out?" In my opinion, this is the recognition that there is a psychological trigger of STRESS that can bring on twitches you are seeing or feeling in the tongue which at other times do not exist. We do not see this type of "come and go" phenomenon with MND and especially not ALS of the bulbar or any other type. When it's there, it's there and it's only a matter of speed of progression. If it's coming and going then, in my opinion, "It ain't there." Make sense?

And so for all the reasons above I cannot reconcile this symptom as being part of a probable or definite issue related to a MOTOR NEURON PROBLEM of any sort such as ALS or any of its subtypes.


I did address something on the link you sent me regarding the difference between fascics seen in ALS and those seen in benign cases of fascics such as BFS. I also appreciate the article you sent for me to read and have added it to my library of resource information. It will serve well in some areas of discussion. However, I think I did also mention the disagreement I have with some of what they've explained regarding the timing of fasciculations in ALS as being one of the earliest and even "initial" sign noticed in ALS. This is stated in the abstract and I do take exception to this from both an experiential as well as literature point of view. Actually, to be fair, fasciculations generate a lot of arguing in the medical community and that's a shame. That's because the public focuses so intensely on it and are looking for definite position statements. However, the hell of it is that fasciculations have never truly been FULLY UNDERSTOOD in terms of what they are and how to distinguish them when involving actually DENERVATION from their target vs. IRRITATION of the nerve that doesn't necessarily cause denervation to occur but where there is sufficient blockade of normal electrical conductance so as to give the appearance or effect of denervation. Numerous investigators have made claims that telling the differences in waveforms using various techniques of ultrasound or EMG can separate things such as BFS and ALS....but the truth is there is no universally accepted protocol and MND still remains a CLINICALLY DIAGNOSED condition with the utilization of tests and studies as a way of EXCLUDING other potential causes.

So again, I want to underscore just exactly what the role is of your upcoming electrical study and how much more importance is given to the clinical examination and the neurological signs found as well as the history of the symptoms when it comes to making a diagnosis. Of course, one can always throw in PATHOLOGICAL DIAGNOSIS as a "wild card" test if you will and some may choose to argue that it is the FINAL STEP in being able to finally say YEA or NAY....and in truth....it is NOT. Pathological diagnosis by surgically excising both nerve and muscle samples to send for testing will only be useful in the VAST MAJORITY OF CASES....if taken from an individual who is already CLINICALLY suspected as having a probable or definite form of MND. In otherwise, "MND FREE" subjects....pathology will always come out NEGATIVE for the disease which is of no XXXXXXX since clinically there was never any suspicion.

So please don't think that if someone offers you a biopsy to PROVE the presence or absence of disease that you should jump on that band wagon.....because all you're doing is donating good tissue that you could better use for the rest of your life in moving your arm or leg...maybe you're helping a newbie lab technician make some block samples for staining and for that reason you'll have contributed in some material way to the advancement of medical science....but personally, I'd prefer to let that new kid get his experience elsewhere instead of loading up normal tissue of mine to go under a microscope! LOL....

Your final question of whether or not I've noticed a continual pattern of firing in fasciculations in BFS? I've seen such a variety of patterns of BFS that I can tell you that there is nothing that can be noticed in a patient who has this condition different from an ALS patient except to say that typically if I see a patient with full body twitching and no obvious signs of muscle atrophy, neurogenic weakness, or the presence of upper or lower motor neuron signs then, I'm already thinking in terms of BFS and not ALS....but not so much by the pattern or firing synchrony of the fasciculations...but by the lack of other salient features of a motor neuron problem. If I'm seeing a patient for the first time with fasciculations that are localized or otherwise restricted to a small region of a limb but may be continuously firing....(e.g. eyelid)....again, I'm not jumping to any conclusions about ANYTHING until more history and examination taken place. But continuous vs. non-continuous firing doesn't really enter my mind so much as whether or not UPPER and/or LOWER MOTOR SIGNS are present.

That is in part where I (and others) diverge with some of what is written in this article about any type of synchronous firing of fasciculations vs. not and the whole concept of fasciculations being an INITIAL symptom of ALS. I will need to see many more authors supporting that claim by data because that really does fly in the face of conventional wisdom which has held since the earliest days of description of ALS and the other since then, recognized forms of MND.

Whew....well, that was a marathon, wasn't it.....hope it made sense. I corrected all the grammar and orthography I could along the way but I just want to get this off to you so I can get to the hospital now and deal with some other issues (vets to be sure!)....take care as always Kind Sir.....and let me know if there's anything more I can do for you between now and whenever.

Cheers!

This query has thus far required 255 min. to read, research, analyze, and prepare response.

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Brief Answer:
Recall our indepth discussion on your fasciculations and Klonopin

Detailed Answer:
I do believe that I have provided you with as much LONG DISTANCE education and reassurance that one can given your circumstance.

You'll also recall our indepth discussion on the use of medications such as benzodiazepines which can cause this sort of "body toxic" picture. To be honest, I believe I was under the impression that you had stopped taking the benzodiazepines after we'd talked about their possible implication in some of your symptoms. Definitely, as a final recommendation I believe it would help your current status of fasciculations to see how things are if you can stop any and all medications, nutritional supplements, OTC types of supplements for a variety of things, etc. to see what the baseline of all this activity may be with a solid washout period of several weeks.

Of course, in the case of benzodiazepines I do NOT RECOMMEND cold turkeying...nor do I recommend that you make the attempt to retire or wean off any benzo or other substance that runs the risk of causing dependency on your own accord. Benzodiazepines can be very tricky for some people to withdraw precisely for the reasons you mentioned of suffering WITHDRAWAL EFFECTS. I highly recommend that you see the VA specialists at the VARC for how to retire these drugs.

As a substitute to using benzodiazepine drugs for reducing stress/anxiety, etc. I would consider (as we've always emphasized) some type of holistic approach to understanding and confronting those issues which are most likely at the root of your anxious state. Is it based in some of military experience that happened which you have not yet addressed yet? Is it based upon some type of seriously traumatic event that happened in your NON-MILITARY life, etc? I believe there are many worthy substitutes for medications of this nature such as acupuncture, deep breathing and visualization exercises, BIOFEEDBACK, and of course, my all time favorite activity to calm the mind......GOOD HARD EXERCISE of an aerobic nature for the most part.

I also do not recommend you increase the benzodiazepine dosing as a way to trial or test an idea you have on the metabolic side effects. I think those decisions should be left to a doctor and one who is familiar and experienced in both administering as well as RETIRING patients from this type of medication.

Also, recall that I believe I pointed out that your VITAMIN D levels were much lower than what we recommend which are bloodstream levels of at least 60-80 ng/dl . My recollection that your numbers are in the vicinity of 26-30 or thereabouts. Vitamin D has on more than one occasion been a game changer for my patients when the levels are correctly boosted and maintained. I think that in order to have ruled out ALL METABOLIC issues as contributing to your symptoms you need to get on a SOLID REPLACEMENT THERAPY regimen which would be 8 weekly doses of 50,000 INTERNATIONAL UNITS of Cholecalciferol (Vit. D3) followed by maintenance of either 2000 or 2400 international units before doing a reassessment. With some luck maybe some of your neuropathic symptoms will go away.

I wish you all the best in your EMG/NCV and any other tests or examinations that your neurologist has planned for you to complete. Please do let me receive some updates from you and let's see how things pan out down the road?

This query has thus far required 276 min. to read, research, analyze, and prepare a response.

Cheers....Kind Sir!

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Brief Answer:
Recall our indepth discussion on your fasciculations and Klonopin

Detailed Answer:
I do believe that I have provided you with as much LONG DISTANCE education and reassurance that one can given your circumstance.

You'll also recall our indepth discussion on the use of medications such as benzodiazepines which can cause this sort of "body toxic" picture. To be honest, I believe I was under the impression that you had stopped taking the benzodiazepines after we'd talked about their possible implication in some of your symptoms. Definitely, as a final recommendation I believe it would help your current status of fasciculations to see how things are if you can stop any and all medications, nutritional supplements, OTC types of supplements for a variety of things, etc. to see what the baseline of all this activity may be with a solid washout period of several weeks.

Of course, in the case of benzodiazepines I do NOT RECOMMEND cold turkeying...nor do I recommend that you make the attempt to retire or wean off any benzo or other substance that runs the risk of causing dependency on your own accord. Benzodiazepines can be very tricky for some people to withdraw precisely for the reasons you mentioned of suffering WITHDRAWAL EFFECTS. I highly recommend that you see the VA specialists at the VARC for how to retire these drugs.

As a substitute to using benzodiazepine drugs for reducing stress/anxiety, etc. I would consider (as we've always emphasized) some type of holistic approach to understanding and confronting those issues which are most likely at the root of your anxious state. Is it based in some of military experience that happened which you have not yet addressed yet? Is it based upon some type of seriously traumatic event that happened in your NON-MILITARY life, etc? I believe there are many worthy substitutes for medications of this nature such as acupuncture, deep breathing and visualization exercises, BIOFEEDBACK, and of course, my all time favorite activity to calm the mind......GOOD HARD EXERCISE of an aerobic nature for the most part.

I also do not recommend you increase the benzodiazepine dosing as a way to trial or test an idea you have on the metabolic side effects. I think those decisions should be left to a doctor and one who is familiar and experienced in both administering as well as RETIRING patients from this type of medication.

Also, recall that I believe I pointed out that your VITAMIN D levels were much lower than what we recommend which are bloodstream levels of at least 60-80 ng/dl . My recollection that your numbers are in the vicinity of 26-30 or thereabouts. Vitamin D has on more than one occasion been a game changer for my patients when the levels are correctly boosted and maintained. I think that in order to have ruled out ALL METABOLIC issues as contributing to your symptoms you need to get on a SOLID REPLACEMENT THERAPY regimen which would be 8 weekly doses of 50,000 INTERNATIONAL UNITS of Cholecalciferol (Vit. D3) followed by maintenance of either 2000 or 2400 international units before doing a reassessment. With some luck maybe some of your neuropathic symptoms will go away.

I wish you all the best in your EMG/NCV and any other tests or examinations that your neurologist has planned for you to complete. Please do let me receive some updates from you and let's see how things pan out down the road?

This query has thus far required 276 min. to read, research, analyze, and prepare a response.

Cheers....Kind Sir!

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