Hi! ~ new here

[lovelily]

Hi Rose!

Sorry you're going through this ordeal. :sad: I wanted to ask you about your appointment with Doctor 'S' in New York...Did you like him?I live in New Jersey and actually had been sent to see another neurologist in the Mount Sinai group because of a CVA and atrophy of the elbow and weakness of a particular leg. I had already had one biopsy that showed 'myopathic changes. His name was Dr. Stanley Turhim.

He sent me off for a second biopsy (thigh) and that came back acute and chronic denervation with collateral sprouting. I went to see the doctor you saw and explained some of the problems I was having with weakness in my hands and some muscle movement. He chucked a pencil across the table and told me to pick it up. When I did, he told me not to worry.

To say the least I was not happy inside. I didn't go back to him.

Dr. Turhim sent me to his wife who does EMG and she did NCS which was normal and the opposing leg (EMG) and that was not normal. I had no atrophy in my legs at that time ( I do now) but did have a small disc herniation that he blamed the abnormal muscle biopsy and EMG. Now I have one hand that has atrophy.

The NY doctor said come back if things get worse, but right now I am staying with my local neurologist until we can see what we can see.

The best to you as you continue your search for a diagnoses.

 

[rose]

Hi Rose!

Sorry you're going through this ordeal. :sad: I wanted to ask you about your appointment with Doctor 'S' in New York...Did you like him?I live in New Jersey and actually had been sent to see another neurologist in the Mount Sinai group because of a CVA and atrophy of the elbow and weakness of a particular leg. I had already had one biopsy that showed 'myopathic changes. His name was Dr. Stanley Turhim.

He sent me off for a second biopsy (thigh) and that came back acute and chronic denervation with collateral sprouting. I went to see the doctor you saw and explained some of the problems I was having with weakness in my hands and some muscle movement. He chucked a pencil across the table and told me to pick it up. When I did, he told me not to worry.

To say the least I was not happy inside. I didn't go back to him.

Dr. Turhim sent me to his wife who does EMG and she did NCS which was normal and the opposing leg (EMG) and that was not normal. I had no atrophy in my legs at that time ( I do now) but did have a small disc herniation that he blamed the abnormal muscle biopsy and EMG. Now I have one hand that has atrophy.

The NY doctor said come back if things get worse, but right now I am staying with my local neurologist until we can see what we can see.

The best to you as you continue your search for a diagnoses.

Hi Lovelily, I'm sorry to learn of your struggles!

Dr Sivak is a great guy as far as I know at least. I went to him because my Laryngologist works with him one day a month conducting the EMG on vocal cords. I went back to him for the EMG on my extremities, which he said looked "pretty good". I'm not that sure what he meant, he was aware that my right hand has some problems off and on, although the focus is of course on my throat etc. All of this was at the end of last year though, and I've had lots of changes since then. He was the one that insisted it was not Myasthenia, and sent me to another rheumatologist (Harry Spiera) to see what that doctor thought about Sjogren's being the cause.

It was my regular rheumatologist that wanted me to go down to Johns Hopkins, because he felt that I was seeing quality doctors in this area (he himself is the chief of rheumatology @ Hackensack Med Ctr) and we didn't seem to be getting anywhere. So, I guess because that was his own field of specialty, I ended up at JH through the rheumatology clinic. ~ and that doctor is sending me on to Dr Sumner, who is a neurologist at JH. So it wasn't that I had a problem with Dr Sivak, that I've gone on to JH.

However, I would like to say that he (Sivak) is a very busy man. My laryngologist has sent him emails concerning me, because she feels there is an urgency with my situation, and he has not responded back to her (I'd left a message with his secretary as well) . He teaches and also does a good bit of duty at the VA, so I don't know how much actual time he spends seeing patients through the clinic at Mt Sinai. But, as my laryngologist pointed out, no matter how brilliant he is, he's not doing me that much good if he can't be consulted with.

His manner with me when I've seen him has been fine though, he wasn't impatient or anything like that, but his personality is much more what one would expect of academia or research. He was very thorough when I went back in to see him after the vocal cord EMG. He spent his time and took a detailed history.

I wish you the best of luck in getting to the bottom of what is causing your problems. Honestly, it seems like this particular area of medical specialty seems hard to deal with, and I don't get it, because I think that most neurologists have psychiatry training and or degree's as well, so you'd think that they (as a generalization) would be more empathetic and strive to answer questions to put our minds at ease, and it seems like its almost the reverse of that. I went to a neurologist in Englewood NJ after the vocal cord EMG, mainly just to get the blood workup ordered for myasthenia, and I didn't want to have to drive into the city just for that, and the guy didn't even bother returning my calls asking about results. The tests he'd ordered were very specialized and I knew that they would take a while to get back, and he'd assured me that he would let me know the results either way, whether anything was found or not, and I left numerous messages without one call back! - then, when I saw Dr Sivak, he was like, "I don't need to see the blood labs, I can tell you just by my exam that you don't have myasthenia" (so maybe he does have a little bit of an ego too LOL)

So anyway, I can relate to your frustration, it seems like often we have to pique a doctor's interest ~ maybe any doctor, not necessarily a neurologist's ~ but have someone pushing for answers. I hope you get those answers soon. take care, Diane

 

[lovelily]

Thank you Diane, for your kind response. You are correct in your thoughts about neurologists being psychiatrists first.

Do you have rheumatological issues, or are those matters still in the air? My doctor's are totally confused at my presentation. My grandfather died from ALS and keeps a worry in my mind. I have a positive ANA, but rheumatologist says it is not the cause of the atrophy, that the biopsy would have been very specific if I had something like 'polymyositis'.

I thought I had been tested for the familial gene for ALS, but it turned out to be for something different called MELAS.

My doctor wants me to go back to my neurologist to re-evaluate my progression and then take it from there.

 

[LindaB]

Diane,








Diane,
Were you ever prescribed any medication for Myastenia, such as Mestinon, or Cell Cept, or IVIG? Did you ever have the Tensilon test? When I did have the tensilon test it showed that I did have Myasthenia, so I wonder why I suddenly developed all the sympton of ALS. Now, my neurologist says he suspected ALS from the beginning. I am sure that I did not have fasciculations when I had my first EMG . Now I have them. I am still on the Mestinon. The neurolgist at Columbia told me to stay on the Mestinon. I wonder why. Everyone seems so confused. I am getting worse every day.
I hope you get some answers soon,
Linda

 

[rose]

Thank you Diane, for your kind response. You are correct in your thoughts about neurologists being psychiatrists first.

Do you have rheumatological issues, or are those matters still in the air? My doctor's are totally confused at my presentation. My grandfather died from ALS and keeps a worry in my mind. I have a positive ANA, but rheumatologist says it is not the cause of the atrophy, that the biopsy would have been very specific if I had something like 'polymyositis'.

I thought I had been tested for the familial gene for ALS, but it turned out to be for something different called MELAS.

My doctor wants me to go back to my neurologist to re-evaluate my progression and then take it from there.

Who knew there were so many possibilities out there, right? You sound like you have so many avenues that the doctors could explore. What's funny (not ha-ha funny) is parallel in our stories with the familial medical history. No ALS in mine that I know of, but when all of this first started with me, it was this sensation in my chest/back when I breathe, and my dad passed away from a rare form of emphysema that can be inherited, so the first thing tested for me was the test for that (negative).

To answer your question, I did have a history of rheumatological issues, which may or may not have muddied the waters, depending on what this turns out to be. I have had Reynaud's for many years, and had dry eye and mouth, which could be Sjogren's Syndrome. But no elevated ANA to anything. The test for dry eye (Schirmer's test) I qualify for dry enough eyes (anything at or below "5", mine are "2" and "5") but no inflamation. Dry mouth, one sole biopsy showed mild focal chronic inflammation, but it was not diagnostic in itself of Sjogren's, as everyone might have a few salivary glands that were inflamed if enough were tested. I'd still had a "possible" diagnosis of Sjogren's though, based on just this. So when the nerve involvement became so much more prominent, I was treated with immunosuppressants and prednisone for the Sjogren's.

It didn't really help anything, not even the dry eye or mouth.... fast forward to December, when my doctors collectively decided that if it were Sjogren's infiltrating my nervous system, that a "super" dose of prednisone should cause some improvement, as Sjogren's is an inflammatory disease. It did not help anything (and probably gave me the lesions on my retina). The rheumatologists feel that if my bulbar problems originated from Sjogren's, then my Sjogren's symptoms ~ the dryness ~ would be more severe, that, added to the prednisone not helping (in addition to the trial dose in December, I'd taken it in smaller doses since April of last year along with other meds) is what makes them agree that my problems are not autoimmune (inflammatory) .

So now, especially after reading Linda's post, I wonder if (just like she fears she may have Myasthenia and ALS) if I might have Sjogren's and some sort of motor neuron problem. One of the reasons that Dr Sivak discounted any sort of ALS diagnosis last fall was because of my dry mouth, he'd said that people with bulbar onset of ALS had problems forming the words, not with their voice itself, and that they had a problem with too much saliva, not too little. But, if I do have Sjogren's,then that explains the dry mouth. And, unfortunately since the time I saw him in the autumn, I've begun having some problems with speech, swallowing, etc that weren't there at the time of my appointment with him. My loss of ability to taste some things bothers me though, because that is a sensory thing, not motor, and it can be caused by Sjogren's. But I'd think that would be with someone that has extreme dry mouth, (which I don't) and an altogether more severe case of it ~ and I don't even have the elevated ANA's!

Lovelily, the neuro that I'm seeing at JH has a specialized field of interest in neruo-muscular diseases that are inherited. Here is the link to her "bio". It sounds like to me, that your profile is one that would be a good fit for her.
http://www.hopkinsneuro.org/muscular_dystrophy/doc.cfm/expert/Charlotte_Sumner

I will let you know what I think of her when I get back from my appointment.

Here's hoping you get some answers! Diane

 

[rose]

Diane,








Diane,
Were you ever prescribed any medication for Myastenia, such as Mestinon, or Cell Cept, or IVIG? Did you ever have the Tensilon test? When I did have the tensilon test it showed that I did have Myasthenia, so I wonder why I suddenly developed all the sympton of ALS. Now, my neurologist says he suspected ALS from the beginning. I am sure that I did not have fasciculations when I had my first EMG . Now I have them. I am still on the Mestinon. The neurolgist at Columbia told me to stay on the Mestinon. I wonder why. Everyone seems so confused. I am getting worse every day.
I hope you get some answers soon,
Linda

Linda, no, I've not taken or had these medications. I took Plaquenil (which is a quinine) for almost a year, and took Methotrexate for a number of months. Both of these can be used for Myasthenia, the Methotrexate more so than the Plaquenil. I also was on Prenisone for about 10 months. None of them helped me at all.

My laryngologist had expected Dr Sivak to do the tensilon test at the time of the EMG on my vocal cords, but when the EMG was finished, and all of the damage was found, he did not feel the need to do it. I think it was because what he saw on the EMG was a different type of damage than is caused by Myasthenia. I'm not positive about that. I remember that my laryngologist was disappointed that he didn't want to do it, as we were all right there and it would have been easy enough to go on with it.

For it to be Myasthenia, he (Sivak) also felt I should have some type of optical involvement (I don't). This was when he was saying he still thought it was the Sjogren's, and called Dr Spiera himself while I was there, to get me in to see this rheumatologist for another opinion. This doctor, Harry Spiera, is renowned in the field of rheumatology, and he is the first to be assertive that in the opinion I did not have an autoimmune cause for my problems. BUT when I saw Dr Alan Baer, (Chief of Rheumatology at Johns Hopkins) he felt that Myasthenia still needed to be completely ruled out.

I think the fasciculations come at the end (not of our lives, of that particular nerve group) so it would make sense that you were not having them when you were seen before. From what I've read ~ and that is not nearly as much as many on here ~ the twitching/ cramps come first, and then the fasciculations come after the damage is done, when the nerve can no longer communicate with the muscle.

Maybe your doctor wants you to stay on the Mestinon because until you have a complete diagnosis, it is safer, rather than taking you off of it, and risking sending you into a flare if it is the Myasthenia. With me, when they decided it probably wasn't Sjogren's, they still didn't want me off of all the meds all at once, in case they were helping more than anyone realized. And now I wonder myself, because I am worse than when I was on them, but then, I was getting worse anyway. So how does one know?

I did want to ask you if you have any eye involvement? Apparently, there is a rare form of Myasthenia (Musk positive, or sometimes called Serri Negative) that does not get bother the eyes, but I'm thinking you said that you did have positive ANA (?)

As discouraging as this has to be for you, just don't give up, there have got to be answers, and someone is out there that can give them to you!

 

[rose]

Thank you Diane, for your kind response. You are correct in your thoughts about neurologists being psychiatrists first.

Do you have rheumatological issues, or are those matters still in the air? My doctor's are totally confused at my presentation. My grandfather died from ALS and keeps a worry in my mind. I have a positive ANA, but rheumatologist says it is not the cause of the atrophy, that the biopsy would have been very specific if I had something like 'polymyositis'.

I thought I had been tested for the familial gene for ALS, but it turned out to be for something different called MELAS.

My doctor wants me to go back to my neurologist to re-evaluate my progression and then take it from there.

Lovelilly, I answered this, but (maybe because I included a hyper-link ?) I got a message that it needed to be reviewed by a moderator before it posted. I'd sent you the link to the doctor I'm seeing at Johns Hopkins, as her area of research involves inherited neuro-muscular diseases. Hopefully it won't take to long for my response to show up, but if you want to look her up by googling her name, put in Charlotte Sumner Johns Hopkins, and it should come up for you.

 

[lovelily]

Lovelilly, I answered this, but (maybe because I included a hyper-link ?) I got a message that it needed to be reviewed by a moderator before it posted. I'd sent you the link to the doctor I'm seeing at Johns Hopkins, as her area of research involves inherited neuro-muscular diseases. Hopefully it won't take to long for my response to show up, but if you want to look her up by googling her name, put in Charlotte Sumner Johns Hopkins, and it should come up for you.

Thanks so much! I will look it up.

Take care!

 

[lovelily]

Hi Rose...Have you been to J/H to see Dr. Sumner? Her picture looks kind. And this is in Maryland? It's down the coast for me. It would be easy to get a referral. I think that is the best way to go.

 

[rose]

Thanks so much! I will look it up.

Take care!

Hi Lovelily, (I see my other post to you with the link to Dr Sumner is up on the site now) Yes, I think she would be a good avenue for you to try. It is a very doable drive. I'm a flight attendant, so one would think I'd fly down, but I feel its just easier to drive it. It took about 3 hours when I went down a couple of week ago for the rheumatology appointments. (I live in Bergen County - just hopped on the turnpike) I see her on Monday, and will give you some feedback about her afterward. take care, Diane

 

[rose]

Hi Rose!

Sorry you're going through this ordeal. :sad: I wanted to ask you about your appointment with Doctor 'S' in New York...Did you like him?I live in New Jersey and actually had been sent to see another neurologist in the Mount Sinai group because of a CVA and atrophy of the elbow and weakness of a particular leg. I had already had one biopsy that showed 'myopathic changes. His name was Dr. Stanley Turhim.

He sent me off for a second biopsy (thigh) and that came back acute and chronic denervation with collateral sprouting. I went to see the doctor you saw and explained some of the problems I was having with weakness in my hands and some muscle movement. He chucked a pencil across the table and told me to pick it up. When I did, he told me not to worry.

To say the least I was not happy inside. I didn't go back to him.

Dr. Turhim sent me to his wife who does EMG and she did NCS which was normal and the opposing leg (EMG) and that was not normal. I had no atrophy in my legs at that time ( I do now) but did have a small disc herniation that he blamed the abnormal muscle biopsy and EMG. Now I have one hand that has atrophy.

The NY doctor said come back if things get worse, but right now I am staying with my local neurologist until we can see what we can see.

The best to you as you continue your search for a diagnoses.

Hi, just just caught that it was Sivak you saw at Mt Sinai, I'm very sorry he treated you this way! Like I'd already said, his personality was more of an educator or scientist, I didn't get a warm fuzzy feeling with him, but he was ok ~ didn't toss any pencils around. But it did bother me that he dismissed Myasthenia so readily. Its probably because he understands so much more about it than I can even imagine, but now its a loose end that needs to be addressed, when it could have already been looked into last autumn. - And it also bothers me that he is usually so difficult to get ahold of. - but then on the other hand, the beginning of February when I was coming off of the very high dosage of Prednisone, I got a migraine that was just about unbearable, had already taken more of my migraine meds than is safe really, I knew my rheumatologist was out of the country, so I called Sivak's office on a Saturday, expecting to get an answering service, just frantic for someone with enough knowledge about my particular situation to help, and he answered the phone himself, spent a long while with me, emphasized that I couldn't take any more Axert, and why I did need to get something else for the pain. But because that was NY and I'm in Jersey, he said he couldn't call something in, and insisted I call my rheumatologist, that someone had to be covering for him in his absence. He could have just said to go to the emergency room and left it at that. So, I guess my take on him is that he has not been unkindly to me, but he does seem to be unavailable a lot. I'll be thinking about you when I'm talking with Dr Sumner next week! take care, Diane

 

[lovelily]

Hi, just just caught that it was Sivak you saw at Mt Sinai, I'm very sorry he treated you this way! Like I'd already said, his personality was more of an educator or scientist, I didn't get a warm fuzzy feeling with him, but he was ok ~ didn't toss any pencils around. But it did bother me that he dismissed Myasthenia so readily. Its probably because he understands so much more about it than I can even imagine, but now its a loose end that needs to be addressed, when it could have already been looked into last autumn. - And it also bothers me that he is usually so difficult to get ahold of. - but then on the other hand, the beginning of February when I was coming off of the very high dosage of Prednisone, I got a migraine that was just about unbearable, had already taken more of my migraine meds than is safe really, I knew my rheumatologist was out of the country, so I called Sivak's office on a Saturday, expecting to get an answering service, just frantic for someone with enough knowledge about my particular situation to help, and he answered the phone himself, spent a long while with me, emphasized that I couldn't take any more Axert, and why I did need to get something else for the pain. But because that was NY and I'm in Jersey, he said he couldn't call something in, and insisted I call my rheumatologist, that someone had to be covering for him in his absence. He could have just said to go to the emergency room and left it at that. So, I guess my take on him is that he has not been unkindly to me, but he does seem to be unavailable a lot. I'll be thinking about you when I'm talking with Dr Sumner next week! take care, Diane

Hello Rose! It sounds like Dr Sivak was very understanding with you. I was only at the beginning of figuring out some of the things that were wrong with me.

Like you, I think I may have more than one thing going on? My father's side had the nerve/muscle disease, and my mom's side (grandmother) had a rare lupus disorder. I think I got stuck with a bad set of genes from both sides.

My neurologist works w/ the rheumatologist to try to combat the muscle atrophy. I take methotrexate because of the low positive ANA, and a low dose of prednisone every other day. My last appointment she stated, I was still progressing.

My PCP contacted my neuro about my progression so he wants to see me again and they wanted to send me to UMDNJ in Newark if necessary.

I wish you the best with your appointment and hope everything works out, and that you can get your answers, and that anything you have is manageable.

 

[DavidGL]

David, let me say that you express yourself very well :wink: Its a shame that its under these circumstances that we get to know one another. I'm glad that nothing else has developed in the bulbar area for you, at least yet. Please let me know what you find out. Good luck tomorrow.

Thank you for your compliment, Diane. And I can surely the same about you!

I will be filing my disability claim on Friday as my very understanding neuro agrees that the time is right. She is puzzled by the vocal cord atrophy--she says it is not to be expected in an exclusively UMN involvement situation. We are calling this aspect a mystery, which will no doubt resolve itself in time. Her formal diagnosis for the disability claim is ALS variant, though she stresses that we should not think of it as full-fledged ALS. She prefers to label it PLS but the ALS wording will ensure that the claim is honored promptly--though that ALS sword will be hanging over my head for a long time to come! When asked for a general prognosis she gave me six to eight years before I find myself in a wheelchair--recognizing that I am experiencing very slow progression.

I must say that the tenor of this thread is quite refreshing, as there seems to be some recognition of the fact that these diseases are very difficult to diagnose. Neurologists, I think, are quite overwhelmed these days as the baby boomer generation ages. It seems as if one pretty much has to get into the hands of an MND specialist if one is to have any chance of finding a fruitful path towards diagnosis.

What can we do but educate ourselves and ask good questions? Then learn to accept, and live with, what has been thrown our way?

~David GL

 

[rose]

I must say that the tenor of this thread is quite refreshing, as there seems to be some recognition of the fact that these diseases are very difficult to diagnose. Neurologists, I think, are quite overwhelmed these days as the baby boomer generation ages. It seems as if one pretty much has to get into the hands of an MND specialist if one is to have any chance of finding a fruitful path towards diagnosis.

What can we do but educate ourselves and ask good questions? Then learn to accept, and live with, what has been thrown our way?

~David GL

I agree. and I think this forum has so much combined experience, not only with the physical health problems being we come up against, but with the problems many if not most encounter finding diagnosis, prognosis, and treatment! ... I was also thinking that maybe the good that comes from this, is being forced to to examine what we've wanted to do in our lives, and have good reason not to put those things off. So much of my life I've gotten by on having what others consider a pretty face. It sounds shallow, and truly it would be, except that our priorities in today's society don't make it seem that way. And now I think, what else is it that I have done? My kids are by far my greatest accomplishment. I could write volumes about how thankful I am to have had them, and to continue having them in my life! Before I embarked on the glamorous life (JOKE - so not true! ) of a flight attendant, I painted, and also did some applied arts ~ my avatar is a painting I did years ago ~ So now, I keep thinking that I need to get painting again, because my right hand does twitch and who knows if its going to "go" next! ~ but I don't (paint) because I'm so bogged down in this, and am tired much of the time, etc etc. I feel like I'm stuck in limbo, not that anyone has a promise of tomorrow, but once I get that prognosis it will give me the incentive to do the things that matter most . Whether I'm given an estimate of a normal lifespan, and normal lifestyle or not. this waiting and wondering if nothing else has stopped me from taking life as I've known it for granted.

Well, enough angst for tonight! Good luck tomorrow when you go to officially file your disability claim. What is/was your occupation, if I may ask? take care,