Nerve Conduction Studies, Endocrinology and Audiology results. SJS fatigue

[MAT51]

I've had an exhausting few days down visiting my prospective long term home. First our car blew up while driving down here! Had to buy a new car to replace it as it was good only for scrap  Then I had to organise back to back appointments all over the place in the run up to my rheumatology appointment this Monday.

The nerve conduction studies this morning showed not only that nothing was amiss, but that everything was really good?! So why are my arms and hands so tired and weak all the time now - same for legs, although not as severely affected as arms? The young neurophysiologist didn't know. She said they don't run other tests here and rolled her eyes when I said that they do at John Hopkins and Mayo and also in London. She commented "yes but there are so many variables with these newer tests and no treatments for PoTS, gangliopathy or autonomic neuropathy anyway? And how would you benefit from galvanic or sweat/ thermostatic tests if abnormal when they are non diagnostic and inconclusive?"

Then I saw my endocrinologist in the same hospital and he disagreed and said they used to run tilt tests and others up until a year ago and the previous neuro physiologist had the equipment and expertise to assess for patients with autonomic issues.

This endo feels all my symptoms I've listed for rheumatologist on Monday are most probably related to Sjogrens Autonomic Neuropathy, from sweats to constipation, swallowing issues and freezing extremities. He agreed there was overlap with my Hypothyroidism as both autoimmine diseases are completely entwined. However he's happy with my TSH since he increased my Levothyroxine so feels my SFN and mild ganglionopathy relate entirely to my Sjogrens so he's passing me back to primary care for thyroid matters now. He did wonder if my arm weakness was something they should run a muscle biopsy of next. But if it didn't show up in very thorough NCS which included sticking a needle into various points by muscles to assess muscle strength - there's surely not much to be gained from further tests? He did say that his colleagues in rheumatology have all been trying to work out what causes the extremely prevalent and severe Sjogrens fatigue. Any thoughts on this as apparently it's the rheumatic disease most closely associated with CFS?

Then I had to drive north for a few hours for an audiology appointment -largely comprising of tests to see if my awful tinnitus relates to hearing loss. It definitely doesn't the audiologist explained. My hearing is fine bilaterally. He knew nothing about Sjogrens at all but agreed that I am suffering from hypoacusis from my description of how many loud sounds make me feel.

He has referred me to ENT now to check that I don't have inner ear problems that might be causing Menieres or Accoustic Neuroma or a vestibular condition to explain my disequilibrium. I feel like I'm running out of evidence to back up my sensory or autonomic issues otherwise. I know now that I won't be offered further immunesuppressants and perhaps this is a good thing after all. I'm feeling quite ambivalent about this since these three appointments.

[irish]

Just because your TSH is normal doesn't mean that your Hashimotos isn't active. Hashimotos has a ton of symptoms that are related because of the autoimmune attack on the body. Have you had the thyroperoxidase blood work done. I have forgotten the total words in this blood test. This is what measures the number of antibodies in your body because of the Hashimotos.

My oldest son got really sick about 3-4 years ago and continued to get worse. I finally got him into  my immunologist and this blood work was done. Normal is around 0-35 sons was 16,000 and ended up at 23,000. My immunologist was flabbergasted at how high his numbers were and all the symptoms he had. Many of them were central nervous system involvement. He is now on 25 mgm of Methotrexate a week for several years and has improved but still not back to the normal.

I would pursue this part of the disease process. People do not realize how sick one can become from thyroid. If I hadn't gotten my son to the doctor and gotten him treated I think he would have been dead in about 5 years or less. He was just dwindling. He is still working but it is hard and he loves to work but has to rest a lot more.

Also, have you ever been to a neurologist and checked for Myasthenia gravis. This is a neurological muscle disease that involved weakness in arms, legs, diaphragm and can result in drooping of eyelids, problems with swallowing, etc. The blood work needed is acetylcholine levels and anti striated muscle antibodies. There is such a thing as seronegative myasthenia gravis so don't let the doctors push you on the negative blood work thingy.

The EMG testing doesn't prove to be very good in diagnosing this disease. The symptoms are pretty straight forward in some ways but others can't see the suffering that we have.  I used to swallow food up into my nose because my swallowing muscles weren't working right. This also explained why I had such a time blowing up ResusiAnnie for my nursing continuing education. Who would have thunk. Good luck in your search and don't give up. Have you seen an immunologist?? They are good at searching for autoimmune diseases usually. Irish

[MAT51]

Hi Irish. Many thanks for your thought provoking reply. The answer is that I think it would be impossible to separate my Hashis to my SJS. So both are acknowleded as AI diseases which I have and my TPO antibodies were quite high when privately tested a few years ago. But they won't even test my free T3 or T4 here now so it's TSH only these days with the labs in many NHS UK areas. He says he's happy to leave me at the currently reasonably high dose because I'm not suffering from a Hashis flare. I don't know how I can tell - but after 15 or more years of Hashis I somehow know the difference between being undermeducated or over now and I think this is my perfect dose -125mcg Levothyroxine.

No my neuro probably hasn't tested me for MG. Maybe that will be next step? But I thought this and also Polymyositis would show up in nerve conduction tests. My neuro is very focussed on the Sjogrens now and so is my rheum - ever since lip biopsy results came back so positive four moths ago. They seem to feel they need look in further? 

I take B12 sublingually but my B12 levels have always been good to date -even in mind the controversy over ranges.
Many thanks

[jazzlover]

You might want to check out the Facebook group "Thyroid Advocates." Really good info there .. Levo doesn't help most patients. Their tests may look better, but THEIR SYMPTOMS are not.

[irish]

Jazzlover, You are very correct. There are several sites that go into deep detail about Hashimotos symptoms and they do not not improve from Levothyroxin. The levothyroxine helps the thyroid because it treats the too high or too low level of hormone. However, the Hashimotos is entirely different because it is the antibodies that are attacking the thyroid gland. The Hashimotos is so complicated and the average doctors are not up to par on all these issues---this has been my experience.

My son went to one specialist-endocrinologist who ignored his high TPO and went into spasms when my son asked for his lab values to send to a new doctor for a second opinion. It was like this doc ignored his illness until he was afraid he might be sued. I was really mad about this as my son did decline because his TPO wasn't taken seriously nor were his symptoms. Thanks. Irish 

[jazzlover]

I know. It's really terrible how little is known about the thyroid...after all these years.

My GP is treating my "low normal" thyroid. Without him, I would be left to an endo who knows nothing.

[MAT51]

I did go and see a private endo earlier this year and he felt I might be having trouble converting T4 into T3. So he added T3 to the mix. I felt a bit less stiff for six weeks but then my GP practice had a wobbly because my TSH was through the floor and so the endo dropped my Levothyroxine by 25mcg every other day from 100mcg. That was dire - I felt so ill and wiped out I could barely function. And since starting the T3 I'd had heart palipiations. Finally GP referred me to an NHS endo who advised me to drop T3 as it was starting to cause Atrial Fibrillation. He said I could take more Levothyroxine -go back up to 125mcg which was my dose five years ago before they started dropping it for low TSH. He is happy to leave my TSH at 0.11. My FT4 and FT3 are always so low in range that I'm never going to be Hyper I'm sure. I definitely can tell when my symptoms are Hashis and I find Levothyroxine works fine for me as long as I'm getting enough of it. I do use Thyroid UK HU where many will say that levo is awful. I know the politics around this are dire in the U.K as the medical profession is at loggerheads with a handful of mavericks. But for me I do okay on Levo so I don't need any more forums etc. Sjogren's is definitely my main disease though and because I had allergies to three antirheumatic drugs I'm not allowed to try more, perhaps this is for the best as I've no organ involvement yet.

The rest is Sjogrens -which my doctors here won't treat at all so I'm having to manage alone drug free with just topical meds only and supplements.

[irish]

When my TSH is 0.11 I get shaky and hyper. The number is low but in the medical world this means that a person is more on the hyperthyroid end of things. When the TSH is higher as in a higher number it means that we are hypothyroid and need more medication. It is so darned confusing because a lay person refers to this one way and the doctors refer to it another.

I have had to argue with doctors about my dosage at times. I always do better with my TSH around 2.0 or so and the doctors seem just bent on keeping that number below 1.0. It makes me mad. The thing is that with thyroid we are all different in how we respond to meds and the doctors need to listen to us.

And yes, the symptoms of Hashimotos and Sjogrens can be confusing. I had a period of time where my joints in hips and knees were so bad I could hardly walk. I asked my neuro for a script for a walker with a seat. Didn't get it and ended up having ny 6 month TSH ad it was almost 10.0. I had a medication increase and in 3 weeks my pain was gone. I was surprised as I did not know that it was my thyroid issues but I sure did find out. Irish

[anita]

Hi MAT,

Sorry to say, I know very little about the thyroid, but I can definitely say that the neurophysiologist should just stick to running the test and not comment on diagnostics or medicine in general.  I say this, because she is absolutely wrong about no treatments for POTS, Ganglionopathy and autonomic neuropathy!!!  Of course there are treatments...various medications for many of the symptoms and of course treating the underlining cause (if know, in some cases).  And why on earth would they even have sweat testing if they weren't diagnostic and conclusive??  She is so wrong it just blows my mind to hear such things.  What does she think...that those of us with these conditions just curl up and die??  There are several ways to approach treatments...many of which you & I have already discussed.  But you need to be tested first to know exactly what is not working properly in your autonomic system.

You would greatly benefit from autonomic testing...as we have discussed before.  Doctors don't really need a tilt table to perform this test.  It's just a table to lifts you from laying to standing.  Yes, it is better with this specific table, but can be done crudely from doing orthostatic BP's and HR just laying down, then sitting up and then standing.  My neuro does this at every visit in his office.  It may not be perfect, but if you have any autonomic problems with your BP and HR it WILL show up doing it this way.

I think that your arms/hands being heavy and weak feeling might be related to vascular issues.  Didn't you say you had livedo reticularis?  This won't cause that type of symptom, but it tells me that you have autonomic problems affecting your blood vessels.  Livedo reticularis IS an autonomic neuropathy condition...it is spasms in the medium sized blood vessels in the skin.  If you have this in the skin, then you may have other spasms in blood vessels elsewhere.  I have vasospasms in the cardiac arteries and often experienced exactly what you describe in your arm/hands.  My (autonomic neuropathy) vasospasms are well controlled now with medication---(that the uneducated lady just told you wasn't treatable!!) and I no longer have the weakness in arms/hands.

I personally don't think you need a muscle biopsy at this point, but I will say that just because a NCS is normal...it doesn't mean the biopsy won't show anything.  it is very possible that a muscle biopsy can/will show abnormalities that NCS (or even a MRI doesn't pick up).  This was just discussed on Neurotalk about muscle atrophy.  I had normal NCS/EMG early in my testing (back in 1996), yet my muscle biopsy did, in fact, show denervation atrophy (where the atrophy is cause by nerves that severe from the muscle).  It also showed where some areas had re-innervation of previously denervated fibers.  So don't rule out the muscle biopsy just because your NCS was fine.  But I would explore the vascular side first...especially if you have livedo reticularis (can't remember if you told me you had this).  Does the hand/arm weakness come on suddenly and go away just the same? 

[MAT51]

Thanks Annita - your response has cheered me up. I so value your wealth of knowledge based on experience.

I tested my BP myself, sending a lupus friend who has a PoTS photos of the results on my BP monitor. My heart rate did not increase or decrease significantly enough to meet the criteria for orthostatic intolerance. One GP and a hospital consultant ran this test on me last year so I assume I don't have PoTS. Is it possible to have autonomic neuropathy without OT? My BP is usually quite high although it does occasionally drop through the floor. I'm on an ARB for hypertension  - Losartan. My heart rate has been checked many times, by monitor and as an inpatient last year.

The endocrinologist said that it is -just as the neurologist said that she was diagnosing non length dependent small fibre neuropathy despite negative skin biopsy last year. The neurophysiologist is new to this hospital and I sense she is not liked by the relevant consultants. She did stick pins into my skin by the muscles on my arms, legs and fingers and I guess the neuro will think this is sufficient?

I'm despairing a bit that my doctors will assume I'm imagining or exaggerating the weakness -which also affects my legs and face too -but has affected my arms for the longest and the most severely.

Yes I do have Livedo R on my knees and feet when exposed to air, particularly over the winter months. But it doesn't affect my arms and the weakness doesn't come and go -it's a permanent state. I'm not sure how to explain it but when I'm resting it's as if my arms and legs become paralysed so I can't sleep for this feeling of profound fatigue in my peripheries - like a car that's lost power and can only crawl along. Even my bowels and pelvis feel this way now. I don't really sweat and feel my breathing isn't always right now - and get palpitations Often, especially after eating. But my oxygen levels have been okay when tested. The endocrinologist says this is automoic and probably relates to Sjogrens.

I wonder also about a Mast Cell Disorder perhaps as I have such a historic allergic disposition?

It is as if my arms and legs are leaden and it takes a mental leap to snap myself out of a resting position. Yet I can and do walk quite long distances if I walk in a straight line on an even surface in strong daylight. If I turn my head I feel I will fall and sometimes do. If I have to stand and talk or wait...well this is very hard, often impossible. Not easy to explain to my husband or cope with in supermarkets or art previews with no seating!

And it is increasing so that I find it hard to raise them for any length of time. I also have occasionally just had to sit down on the ground wherever I am, because of the weakness in my lower body and feeling of imbalance / disequilibrium /vertigo.

Perhaps this is what people mean when they say they have chronic fatigue? But I just see it as limb weakness rather than fatigue because I don't always need to sleep when this happens and I don't have brain fog as such, although I'm often a bit disorientated and bewildered these days when I'm walking about, and often get lost in interior spaces such as hospitals, fail to observe signage etc.

I also wonder if this could be early Parkinson's? Which is something my neuro would identify I think as it's her area of expertise. Maybe nothing to do with Sjogrens or autoimmunity although very coincidental if so.

Does this explanation/clarification help and do you think it's worth emphasising over other things with my rheumatologist on Monday? These appointments are usually very time pressured. 

I have met a group of people with Sjogrens here in Scotland today and none seem to take any serious medications - all just seem to feel it's untreatable apart from topically or with anticonvulsants? Maybe this is different and Scotland is significantly behind other places for Sjogren's? RA seems to warrant much more attention and urgency it seems.

[jazzlover]

MAT .. Have you found any relief by eating a low histamine diet?

[MAT51]

What does an antihistamine diet consist of Jazzlover? I try to avoid anything with additives/ refined foods or too much gluten and very little dairy.

[anita]

MST,

I wrote a detailed reply to your last comment and somehow it didn't make it to the board.  I must have hit something but I swear it was 'post'.  Not sure what happened, but I'm sorry.

It doesn't really make a difference at this point, since your appointment was this morning.  Please update and let me know how it went and what was her conclusion.  I certainly hope she was more receptive this time and considered your arm weakness as a genuine problem that needed to be addressed.

BTW, POTS does not just involve the heart rate.  Yes, many people just have tachy high rate when standing, but a drop in BP is more common for orthostatic intolerance.  You didn't mention even checking your BP during the laying, sitting, standing tests...what happens to your BP??

As for the NLD SFN...it is impossible to properly diagnose this condition unless you've had a skin biopsy where more than one site has been checked.  That is how they determine length dependency...if the distal site has lower fiber density, then it is length dependent.  If the site are equally low, then it is non-length dependent.   But you have to compare TWO sites...and you only had one.  Not to mention your biopsy was questionable in transport and the doctor performing (according to you).  Although I would likely agree that you have NLD SFN, it is wrong to guess at a diagnosis like this.  IVIG will likely never be offered if not confirmed to be SFN.  It's hard enough to get it in your location, much less with guessing the Dx.

The only reason I push you to get this done is so you can possibly be considered for other treatment options...like IVIG.  You have read many of Dr. Birnbaum's papers...if not treated quickly, then nerve damage (especially autonomic) will likely end up permanent.  What a shame this would be when you have options.  Just as here in the U.S., you have to be your own advocate...don't expect the doctors to hand out treatments on a silver platter.  You have to fight most of the time...which means having proof.  They are not living with these symptoms...you are.

Again, sorry my post ended up in cyber space and not on your thread.  I'll be looking for your update on your appointment.

[MAT51]

Not your fault about cyberspace Anita. I often don't get E-alerts to your replies either but this one came through so here I am!

My rheum apt was most unexpected. I got same registrar I saw the first time. He confounded me by running through my potted history and then talking about Rituximab -handing me Arthritis Research UK info and saying his clinical team meeting is tomorrow but this is what the newly retired consultant had  planned for me.

I had to think quickly because my sense told me that he was on the back foot with results of my nerve conduction tests not yet through. So I asked about MMF/ Cellcept as an alternative and explained that I've read that in trials people with SFN of primary Sjogrens responded significantly better to MMF than they did to Rituximab.

He didn't mind at all that I'd read up. Said that primary Sjogrens is rare and neuropathy even rarer so they don't yet have established treatment protocol for it.  But I know that actually a very recent trial from Barts hospital U.K showed Rituximab to be innefective in the treatment of Sjogrens sicca  symptoms and fatigue too.

He said he would put this to the senior rheumatology staff on Wednesday and then, if a trial of Cellcept is agreed, he will write to my GP and let me know so I can get started on it.

I know that I'm in a hiding to nowhere trying to get my neurologist to recommend IViG and now I've met the neurophysiologist I'm even more certain that I won't be able to get the tests for autonomic neuropathy or skin biopsies here. If I find that the Cellcept does nothing or I'm allergic to it then I will galvinise myself to fight and insist on being sent to Newcastle in the north of England for specialist tests -this is one of two UK hospitals that specialise in Sjogrens and they test for Dysautonomia there. By then I'll be living down here properly so it will be easier to fight for proper testing. I hope all this makes sense as I do agree that it's better to get incontrovertible evidence that can give us the best possible choices where possible.

But my instincts tell me, based on what I've read and learned here and elsewhere, and from you - that MMF/ Cellcept will be a good start and I don't need to prove that I have SFN or dysautonima to try it. X

[Jasper]

MAT51 .....

It is encouraging that your Rheumatologist is thinking about trying some of these drugs. Maybe you will finally find something that works for you.

I know you have done a lot of research and reading about various treatment for Sjogren's mediated neuropathy.

I did want to mention that I am on Rituximab infusions and have experienced marked imrprovement in most of my Sjogren's symptoms. They are as follows:
Marked improvement in fatigue and energy levels (I was not functioning at all prior to Rituximab. Now I am able to function, be active, and enjoy life.)
No flares since starting Rituximab (I was on Prednisone tapers 5 times in 2015 due to flares)
Marked improvement in cognitive function
Improved balance
Increased saliva
No edema of lower extremities since starting Rituximab
Decreased joint pain and stiffness
Marked improvement in peripheral neuropathy symptoms (burning, electric shocks, wadded up cotton sensation around toes and feet, throbbing pain R toes and forefoot, sensation of vibration in arms, hands, legs)

My Rheumatologist is using Rituximab on her Sjogren's patients much more frequently than previously and she is seeing excellent results. She is getting people off of daily prednisone by using Rituximab infusions and she is seeing marked improvement in symptoms.

I realize your neuropathy symptoms are different than mine and you also have autonomic dysfunction. I am not saying you will respond the same way I have responded. I just wanted to mention my experience with Rituximab (and my Rheumatologist's experience) to let you know that some people with Sjogren's do respond very well to Rituximab.

In addition, I have included a link to a 120 week  Rituximab infusion study which showed that Sjogren's patients did improve markedly with Rituximab infusions. Another study shows improvement with neuropathy in Sjogren's (but I cannot find that article right now).

http://arthritis-research.biomedcentral.com/articles/10.1186/ar4359

I hope whatever you are prescribed works well for you.