Thursday, June 13, 2013

My Story of Cranial Neuropathies and Sjogren’s

I have put off writing this blog post a number of times.  To be honest, it's not something that I like to think about, a luxury I sometimes have when my symptoms are not particularly severe. But it is a subject too important for me to continue to put off.  So here goes.

I am one of the unlucky Sjogren’s patients who suffers from significant neuropathies; in my case multiple cranial neuropathies are the particular nemeses. In fact, for me, cranial neuropathies are perhaps the most difficult part of contending with Sjogren's syndrome. They can be exceedingly painful and are one the reasons SS has been so debilitating for me over the last several years.

While I know I am not alone in having these cranial nerve issues, I have also Iearned that, in some respects, I am the exception to the rule. For starters, depending on the study, approximately 30 percent of Sjogren's patients have neurological involvements of some kind. That broad group includes all neuropathies that impact the central, peripheral, and autonomic nervous systems. Multiple cranial neuropathies in particular are a relatively uncommon subset. One estimate places them at just 5% percent of those with neuropathies, which would mean less than 2% of the overall SS population.  (I’m sure others will have seen other numbers, but the point is multiple cranial neuropathies are considered fairly rare with Sjogren’s.)  

However, since at least I know I am not the only one out there with Sjogren's, Lupus, or other autoimmune diseases contending with cranial neuropathies, I thought I would start with some of the basics I have learned over the last few years.  For starters, there are actually 12 cranial nerves. Making things more complicated, some belong to the peripheral nervous system (PNS) and others belong to the central nervous system (CNS). The short version is that these are many of the nerves that are involved in your face, head, eye, and ear. (If you’re interested, greater details on the specific functions of the twelve cranial nerves can be found here.)

With Sjogren’s (and other autoimmune diseases such as MS) the 5th cranial nerve or the trigeminal nerve is one of the most commonly involved in the disease process.  This nerve has three main branches V1, V2, and V3 impacting the upper, middle, and lower sections of the face as shown below:

Dermatone of Trigeminal Nerve
By Madhero88 via Wikimedia Commons

Patients with trigeminal nerve involvement or trigeminal neuralgia often have severe pain running down the side of their face, lack of feeling, sensations of numbness or general abnormal feeling in their face.  I realize that pain and numbness may sound contradictory, but it will make sense to anyone who has experienced it. Symptoms can be made worse by simple everyday activities that most people don't think about twice such as eating, being outside in the cold or wind, or even just touching the face. Unfortunately, trigeminal neuralgia is an extremely painful condition and can be difficult to control. Doctors recognize it as a condition of its own and often believe it to be untreatable; a terrible thing to hear for those of us who have it.

For me, the trigeminal nerve was the first of my cranial nerves to be affected by the disease.   It started with a change in sensation on the right side of my face such that touching it became uncomfortable. It was as if my face had gone numb and progressively painful at the same time.  Not a good feeling at all.

But that was just the start for me. After the trigeminal nerve, I had other cranial nerves become involved too.  I had problems with the 7th cranial nerve, which impacted my facial movement and caused hemi-facial spasms.  As a result of its involvement, at times I was unable to move my face or open my eye.  My 8th cranial nerve, which is responsible for hearing and balance, also joined the dysfunctional nerve party.  I experienced (and at times still do have) extreme ear pain, sensitivity to any sound and vibration, and balance issues.  I was even unable to be in a car or train because the vibrations and bumps of the road caused so much pain.  I was in constant unremitting pain and unable to do basic everyday tasks.  It really left me at my wits end… I could go on, but I am sure you get the idea that my immune system had mounted a full-fledged attack on my cranial nerves.

Now, back to the autoimmune aspect of it, which I hope may help some other patients. All of the nerve/pain issues described occurred before any of the more twenty doctors that I saw during this time diagnosed me with autoimmune disease.  If anything, the pain threw them off. I was told I had a pain syndrome and doctors put me on pain meds. All they did was make me slow-witted and, while they masked the pain at times, they did not make it go away and I kept getting worse. The doctor's response?  You must have a "bad pain syndrome. Take more drugs." This was from some of the most prominent medical centers in the country. 

I know that I am probably not alone in the autoimmune disease community of having had something like this happen.  What I have learned since then is that many doctors, even rheumatologists, are not aware of the possible neurological complications of Sjogren's Syndrome.  And the big and important question of course is why is this the case?  So that is what I’ll try to tackle in a bit more detail in an upcoming post.  Stay tuned.

I realize it's a very tough subject but I would be very interested to hear if any of you have neuropathies related to autoimmune disease. If so, how have they affected your life?




















Sources
http://brain.oxfordjournals.org/content/128/11/2518.short
http://www.ncbi.nlm.nih.gov/pubmed/20827117
http://www.hindawi.com/journals/ad/2012/645967/




22 comments:

  1. Thank you so much for this post. I was recently diagnosed with occipital neuralgia. When I asked the Neurologist is this was brought on by Sjogren's, he seemed not willing to confirm that it is. He did state that it can happen, although it is very rare.

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    1. Thank you so much for writing. I have a suspicion that there are more of us than doctors realize with neuro Sjogren's complications. And that many of us are diagnosed with something else or our neurological symptoms are considered separate from our autoimmune disease when they actually go together. There are some sources at the bottom of the post that could be helpful if your doctor is willing to look at them.

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  2. I have quite a bit of cranial neuropathy. My doctor chalks it up to fibro, but I really think Sjogren's is to blame. I often get dizzy when the attacks come, I don't know why. Vitamin B12 complex has helped to lessen the power of the episodes. It is really quite scary, at least to me it is. Thanks for your post, it's good to know I'm not alone with this stuff!!

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    1. That's very interesting about taking B12 and that it seems to help with these neuropathy episodes. Something to definitely discuss with my doc to see if it might help. Thanks for sharing and it's also good to know that I"m not alone too.

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  3. I am sorry because the pain from your neuropathies sounds very bad. I have many neuropathies but quite different to yours. Mine include autonomic cardiovascular dysfunction causing extremely labile blood pressure. A neuropathy that results in a paroxysmal cough like whooping cough that often lasts till I vomit. It comes without warning and is either more or about as embarrassing as passing out in public from low blood pressure. Numbness and tingling around mouth and nose and extremities. Often problems picking things up or holding things. Weird little tiny spikes of pain across the skin of my thighs when I sit down and relax in the evenings that often cause me to cry out and jump and wriggle but only last for 10 mins or so then disappear till the next evening. A cramp that strikes me at intervals in the instep of my left foot may be a neuropathy. All very odd and I wish they would go. It's like they have stolen my body.

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    1. These episodes sound so difficult and very unexpected and I'm sorry you have had to go through them. I am glad that you took the time and had the courage to write about what happens to you so that patients with Sjogren's and autoimmune disease can read this and not only better understand autonomic neuropathy, but also bring it up with their doctors if their symptoms seem similar. I know for me it can be tough to write about the most difficult health aspects of living with autoimmune disease so thank you for sharing your experience.

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  4. I am interested because I have been recently diagnosed with sjogrens and have dizziness often and anxiety along with strange facial and eye spasms sometimes and just a feeling of numbness like the size of a hair across my face or something..

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  5. I am very happy you wrote this blog post. I am sorry you experience all this pain. I have good news for you. I had extreme trigeminal nerve pain and was told a variety of things to do but only one thing actually worked for me. Using energy medicine they were able to desensitize me and I am now pain free. .
    I also suffered with various autoimmune diseases so I know the frustration firsthand.
    Feel free to contact me for more information.
    Www.mariaelenahealthcoach.com

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  6. Reading these comments not only inspires me, but reminds me how brave my autoimmune and Sjogren's friends are everyday in the face of such health obstacles.

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  7. So glad I found your page also. I have not been formally diagnosed with Sjogren's but the Neurologist said he thinks I have it. I have peripheral neuropathy, dry, burning mouth, dry eyes, etc. Now my face has started burning and I am noticing a numbness on the right side of my face. No pain on my face yet anyway, just in my mouth and my throat. My arms and face feel like they are sunburned all of the time. A lot of numbness and tingling and burning.

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  8. Thank you so much for writing this....I have battled this for 12 years. Have yet to find a doctor that knows how to handle it since I lost my insurance coverage. No insurance now so I just maintain as best as possible. But this makes me feel better for some ODD reason. But so very sorry you have to go through this. But thank you so very much for sharing this. BE BLESSED MY DEAR.

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  9. Hello there
    You are not alone...one of my first symptoms before being diagnosed with PSORIATIC ARTHRITIS was a burning face...I am stil waiting to find out if I have secondary Sjøgrens. I believe it will happen soon. I just had some test done.
    I can recommend one thing...try to distract yourself when the symptoms are there..I am on Pregabilin/Lyrica and Sirdalud/Tizanidin besides my MTX but careful ...it all has side effects- So if anything else helps....don´t take too much medicine for it.
    Do not worry about Fibromyalgia or no Fibromyalgia ...I believe that people with years of pain have a changed system. Hope you understand what I mean..I come from a country where we speak a very different language. Good luck all...and thank you for your info.;-)

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  10. I had pain in my trigeminal nerve that started abruptly. Drugs took the pain away for the most part, but left me a total zombie in the brain fog part, or so totally fatigued I could barely function. At the end of September I weaned off the last drug. I have had only two short "pounder" pains, and seem to only have what I describe as "sparks" occasionally around my mouth and chin. I often feel like the left hand side of my face is "bigger" or puffier, or maybe has a sense of fullness but nothing that is visible. I also have occasional tingling of the back of my scalp on that side. About once a month I get a pain I call a headache, but it actually hurts behind my left eye and deep in my left ear. My neurologist ordered an MRI both with and without contrast, but they could not get an IV started (??) so I was not able to get the one with contrast, but the doc said everything was "normal." I beg to differ - neuropathy is not normal!

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  11. I have the pain from head to toe always one sided, either right or left at every one time and occurs on a daily basis. Been to a neurologist who says its migraine as I get really dizzy with the pain. But what you've said exactly describes my symptoms. I've been called crazy for imagining it as the GP has never seen such a symptom before where the face feels numb/pain. I don't even know how to begin describe the sensations I feel in the face and head. Its so frustrating. Has there been a diet that has helped you? I've eaten really clean in the past where I just boil/steam everything I eat with only salt or soy sauce for flavouring and it has not helped.

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  13. Hi - I am glad I found this blog. I am female, 44 dx with lupus and sjogrens. I have had 24/7 tinnitus in my right ear for 2 years. I have the occipital nerve pain and have such bad cervical spine pain that I wear various neck braces. If it weren't for the neuro issues, I could live with the other associated symptoms that these diseases bring. I have been getting nerve blocks and botox but they don't really help. Ice, Naproxen and pain meds and muscle relaxers help. I also have documented cognitive disfunction and severe depression. I am planning to start the paper work with my rheumy to go to Johns Hopkins Sjogrens clinic. I also am on benlysta infusions - about to have my 21st infusion and they aren't helping. Each day is a struggle. All I want to be able to do is go on long walks in the late afternoon with my dog and garden, but I am getting ON flares from those simple tasks. I am also getting an epidural soon.

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    1. Hello! I am just now finding this blog. It is March 2017. I'm planning a trip to Johns Hopkins. Can you tell me about your experience there?

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  14. Hey thanks for the learning point! Im a young physician and stumbled on your article! Get well soon! You are a blessing!

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  15. Hey thanks for the learning point! Im a young physician and stumbled on your article! Get well soon! You are a blessing!

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  16. I have to thank you for posting this. I get peripheral/ poly neuropathies that come and go with flares of Sjogrens on my left side. My entire left side becomes affected including my face. It is painful and uncomfortable and painful. I take prednisone which helps to alieviate it after several days. The neurologist
    Keeps insisting there is no way the side of my body and my face can be related. And must be migraine related. Though the medicine he tried to put me on did not alleviate it. At this point looking for answers...?

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  17. Hi! I started `reading`your blog almost 1,5 years ago when i was dg with pSS. I was then hoping that i will not get as many symptoms as you have been describing on the blog. Unfortunatelly, Sjögren has other plans for me. I have had from the very beginning trigeminal neuralgia, but it was more this sharp acute pain that would come now and then. However, for the last 6 months i have been having pain in my left ear, so strong that i can no longer use headphones on that side and have been having problems with my balance. The balance problems are really scary and debilitating. What has helped you against balance problems?
    PS: Hope you are doing well and will continue posting.

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  18. Have been trying to figure out what my trigeminal issue has to do with my undifferentiated connective tissue disorder. Will be sharing this with my rheumatologist next week

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