Have Axonal damage?

Hi, I am brand new here, I am a 60 yo male. I began with numb toes on both feet about 18 months ago and it spread to tingling up my legs, on patches on my forearms and on my face, the face and arms were intermittent but the feet became pretty numb. My first nuero suggested it was my bad back but eventually sent me to a large teaching hospital, my first EMG was labeled a “borderline” and my symptoms more or less stabilized until last July when my hands and forearms really started bothering me and I began losing muscle in my thighs and calf’s. Four weeks ago I had a LP and from that point forward my symptoms worsened dramatically, not only did I have the spinal fluid leak but my hands and arms went weak. I was hospitalized where I was given IVIG over a three days with the only response I could tell was that I had regained some of my reflexes. A few days later, I was not getting any better and the headaches continued from the LP, I had a blood patch and another neuro came in and saw that I was only able to walk with assistance, did more blood work and found I had inflammation and put me on steroids for a week, this improved my walking but I am still on a cane for balance and my muscles are weak. The spinal tap revealed elevated proteins. I had an another EMG today (14 months after the first, different facility and dr) and the Dr told me I had axonal damage and will start me on another round of IVIG. My question seems to be could the spinal tap made this worse? Also the Dr told me today that he thinks this is possibly related to Sjorgrens or some type of vasculitis but he is calling it CIDP. Either way, I am pretty concerned today as I have zero co-morbidities and have previously lived a very active lifestyle. So I thought I would ask people with experience and I appreciate very much allowing me to join.

I have Sjogren syndrome as well and it is associated with CIDP. Everyone thinks that Sjogren syndrome is only dry eyes and dry mouth, however, there are cardiac, pulmonary, and neurological manifestations of this disease. If you do have Sjogren and CIDP, you may need more than just one run of IVIG. I have been on IVIG for ten years and it has truly been a life saver. No I do not have the same strength I did but I certainly function fairly well. I need to know when to stop. I hope you get an answer from the new neurologist.

Thank you for your reply, I will also be following up with a Rheumatologist as well. What I am finding concerning in my case is that when I went to the big teaching hospital 14 months ago and they did an EMG/NCS test, they found no evidence of nerve damage, I plugged along until yesterday and had another test and no I have axonal nerve damage, my lower legs are dead numb and my hands barely work. Since I am new to this, will the IVIG actually improve these functions? I did have one round in the hospital Like I mentioned but there were no noticeably dramatic improvements. I guess I never thought it would get to this point as I was asking for IVIG all through this but until I got the positive spinal tap know one seemed to care. Oh well, again I appreciate the feedback and the chance just to talk to someone.

Bradola, your experience is so close to my husband’s it is frightening. He has not had the LP though. But his numbness and damage progressed almost the same as yours. He tried IVIG with no improvement at all, and is now having Rituximab every 3 months, with no improvement yet. He will have his 4th dose this month. Agreed, no one ever thought he would get this bad. He is completely disabled now because he has no functioning of his hands at all. If you don’t feel confidence in your neurologist, keep pushing for answers and help. I will keep you in my thoughts and prayers.

Hi Positve_outlook
I did want to ask you that having been diagnosed with Sjogrens as well, is there any differences in treatment other than the IVIG? For example was there any improvement in the other symptoms related to Sjogren or is your only symptoms been the nephropathy issues? I do have dry eyes but I had a Lasik procedure years ago that it was attributed to, other than drops at night I really do not bother with it. And I guess one more question is how do they determine that, as my first Neurologist did a “Sjogren’s Panel” that was negative a year ago however in the hospital they ran an ANA test and it was strongly positive, this is what the new neurologist is basing this on. Anyway, thank all of you again for your replies I feel I am in good company…

Your Sjogren’s titer can be negative if you just got IVIG. A positive ANA can be a false positive. The test is nonspecific. I should tell you that I am in the healthcare field. Sjogren syndrome is more than just dry eyes and a dry mouth. I would definitely confirm your diagnosis with an ophthalmologist with an interest in Sjogrens.

Brad - many of these neuropathies are closely related and are on a sort of continuum. Hard to know where one leaves off and the next one begins. I have been diagnosed with CIDP and 9 years in, I can only walk with a cane (barely) or a walker. But I don’t have ALL the classic CIDP symptoms such as chronic pain. Had ivig for 8 years and I continued to deteriorate. It never helped me. I have extensive axonal damage now and ivig does not help with that according my neuro. So, as you can tell if you spend a fair amount of time on this forum, there aren’t always definitive answers for those of us with these neuropathies. I’m not considering steroids unless someone can tell me they might bring about a long term benefit. Otherwise, not worth the side effects for temporary benefit. You can’t take them forever. Just hang in there and make the most of every day. I hope you find a therapy that works for you. If you do, I would love to hear about it.
Best,
Mike

I’m sorry for your situation. The onset of your CIDP sounds like mine with tingling in tops of big toes spreading to alll foot areas and lower legs into left thigh. I was diagnosed within 9 months of onset by great neurologist who started me on IVIG which I continued to receive weekly for 4 years. My CIDP went into remission some years later which allowed me to regain previously lost strength.
My CIDP always was painful and 11 years later still is. As a large man, 6’ 7” 260 lbs I took a jumbo size of IVIG always with Benadryl to prevent headaches. I’d encourage you to go to a CIDP Center of Excellence as recommended by CIDP/GBS International Society as this is no time for learning hospitals to figure out this rare disease. Get into chronic disease support group because you can’t talk about everything that is happening to your spouse, friends and family. They love and care but only want to go so deep with your fears. I will tell you that you might regain lost strength, but it’s 50/50. I did get physically better and still work, but dealing with chronic pain is very, very hard. Find or rely on faith. Keep looking for answers and be your own advocate demanding better treatment. Call on me anytime for help. I’d give my phone and email but I think it’s against website protocol.
Do not give up hope of reclaiming your lost strength!!! You can get better, but I think my rebound is the exception, not the rule. Be thankful you don’t have pain! My CIDP was later confirmed by nerve biopsy at insistence of pain management doctor. I understand why neurologist didn’t insist on it because it’s barbaric. He just diagnosed and started treating.
This isn’t the end of life but the start of a life greatly altered from before. It could be worse like my friend with MS, but it’s hard. Too hard to stand against it alone. Get help, get support, dust off your bible, and fill your mind with positive ness and inspiration! I don’t know you, but we share a common bond of life forever changed, but still life. Get a fashionable cane, that you’re not embarrassed to be seen using. Get handicap parking, SS Disability, pain management doctor, take care of yourself. Every day is a challenge! Are you ready? Go!

Hi

Thank you for the thoughtful reply, I appreciate it very much. I think unfortunately we are still in the finding out stage as the Drs have called it CIDP but are suspicious that it is related to Sjorgrens Syndrome or some other form of auto immune disease. Either way I have been really struggling with the neuropathy as well as the muscle loss in my calf’s and ankles, thighs and hands. I started my second round of IVIG today (5 day course), my first was three weeks ago, I can say the only response I had to the first one was that my reflexes went from nonexistent to near normal (or it seemed to me) a week later although I am still symptomatic. I see the neuro-muscular specialist at Loma Linda this week again and will see what he says. Otherwise I am still on prednisone as well and am hoping for some small improvement at least, this stuff really sucks the life out of you but I know the good work was already done, I am speaking it into reality every day, I am calling for my healing and stand ready to receive it!

Doesnt the positive result of IVIG and Prednisone (the reflex improvement) show that CIDP is the more likely culprit? I thought spinal tap protein testing would yield a definitive diagnosis? It must be frustrating to have gone through with the testing they wanted you to do, and still not be sure. But the spreading and rapid progression of reflex loss and recovery and atrophy sounds so much like what I experienced that it seems like we are experiencing the same disease. But I’m no doctor. Usually cidp is treated with IVIG or steroids, but not both at the same time. Whatever has caused your reflexes to return is a big step forward for you! Congratulations on getting some improvement. Have you had any decrease in pain to go along with the improvement?
Wishing you continued success, healing and answers to your medical mystery. In this world you will have trials, but don’t fear for He has overcome the world. Speak of and claim your renewal! God bless! Bill

I decided to stop treatment after 3 years of IVIG and 1 year of steroids. IVIG stopped reducing pain and steroids stopped me from feeling human. I stopped treatment which increased pain but progression of disease stayed at same level. It’s never easy to know when to stop treatment but for me it was the right move.
I wish you well in your decision to stop or continue treatment.

Hi Bill,
I just got home from my third of five treatments today, I did ask the Dr about the steroids and I was told it is OK to do both at the same time and I am beginning to taper as of yesterday again to 30 per day for a week. I will see the Dr tomorrow and ask about the exact Dx because as of right now I think to get the IVIG started it is considered CIDP straight out, however the one Dr I saw while in the hospital had me tested for ANA titers and one came back strongly positive for Sjorgrens Syndrome which carries a different set of complications as well but IVIG is still used for that as well. Interestingly though I had all these tests right along and they were always negative and as another poster mentioned to me above that they can have false positives but that is sending me to a rheumatologist next week. I think the one thing the DR is concerned about is the fact that I have already demonstrated some axonal nerve damage and I do not know if this is consistent with CIDP or something more sinister as I thought CIDP was usually demyalating only. As for right now, I am experiencing greater weakness in one leg from my hip down and I have marked muscle wasting in all four extremities but particularly in my lower legs and thighs. I have also developed a lot of trembling in my hands, I really hate this but I am thinking it might be partially related to the prednisone. I am able to walk without a cane but I keep it for balance but after a mile my legs really hurt. I am not sure when or what I should expect from the IVIG as I see different folks in the infusion suit who seem to have symptoms like mine but they all seem like they are doing pretty good compared to me, or at least that is what it seems. Anyway, I will hopefully get some more answers tomorrow and some improvement with the treatments…staying positive!