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 5^th 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 7^th 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 8^th 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/

Sometimes You Need (Gluten & Corn Free) Pancakes

This past weekend I was madly craving pancakes.  This sort of thing usually happens when I am taking post infusion steroids and my desire for carbohydrates goes up exponentially.  All I could think about  eating are those delicious butter and maple syrup topped treats.  Healthy, maybe not, but splurging is allowed sometimes.

While I certainly wouldn't mind heading out to a local diner and enjoying pancakes without any cooking involved, this is not feasible with my corn allergy and gluten sensitivity.  If I want pancakes, I have to make them myself and find, or at least adapt, a recipe that fits my allergy specifications.  Luckily, I found a simple recipe on allrecipes.com for rice based pancakes that calls for few ingredients all of which were in my pantry.   For me, less ingredients is my motto.  Less ingredients = less likely to have an allergic reaction and less room to make a catastrophic cooking mistake.   

The pancakes were super easy to make and tasted good too.  My father, who was in town visiting and also cannot have gluten, joined me in eating them.  He hadn't had pancakes for quite some time and definitely enjoyed this rare treat.  My husband who is lucky not to have any allergy restrictions also had some.  I figure it is always a good sign when someone who eats gluten has two helpings of a dish that is gluten-free.  It means it must pass the wheat-eaters taste test.

I topped my pancakes with plenty of butter and maple syrup to satisfy my increased appetite from steroids.  Maybe next time I'll have to add my corn-free and gluten-free chocolate chips to the recipe!

Rice Flour Pancakes. 

Adapted from Allrecipes.com

Ingredients:

• 3 eggs
• 1/2 cup milk
• 1 tablespoon vegetable oil 
• 1/2 teaspoon salt
• 1 cup rice flour
• 1/2 Teaspoon Baking Soda * (Added for additional fluffiness)

Directions

1. Beat the eggs in a mixing bowl with a whisk or hand mixer.  Add the milk, vegetable oil, and salt into the same bowl. Add and stir in the rice flour until no lumps remain.

2. Cover the bowl and let stand at room temperature for 1 hour or in the refrigerator overnight.

3. Heat a lightly-oiled/buttered griddle or fry-pan over medium-high heat. Drop batter by large spoonfuls onto the griddle.  Add fruit or chocolate chips to pancake batter if desired.  Cook until bubbles form and the edges are dry. Flip and cook until browned on the other side.

  

Autoimmune Fatigue: Reaching The Tipping Point

While the details are different, this is probably a story familiar to other readers.  I've been officially diagnosed with AI disease for a few years now and you might think that I would not let an increase in my energy levels lead me to overdo it.  But it is an inexact science to gauge my tipping point or that magic cut off between feeling good to having overwhelming  fatigue.

Image Source

At the beginning of the week, I had energy.  I was a very excited to welcome this old friend  that makes rare appearances back into my life. I was able to walk the city streets at the same pace as others. Dare I say there might have even been a bounce to my step. My future seemed to  have a few more possibilities than usual--would working if even part-time be an option?  Maybe  I would be able to get to some of those house projects that have been piling up?

My feeling good also corresponded to an upcoming work event that my husband had to attend. I wasn't initially planning to go, but I decided why not attend now that I was feeling  up to it.  I realized that I hadn't been out in so long that I needed to go shopping for something to wear.  But I soon found that going to stores and the actual act of trying on clothes is extremely tiring.  That creeping fatigue and pain started to set in the day before the dinner.

I rested beforehand as has become my routine whenever I have  plans.  Still I wasn't sure if I could make it though the evening, but I was determined to be there.  I had made a decision and I wanted to stick to it!  Yes, some have called me stubborn before.  In the end,  I managed to attend and was proud of myself for just getting there.  I even made  it through the evening and although my energy had started to seriously dwindle before I arrived,  I was buoyed in the moment by socializing and meeting new people.  I rose to the occasion.

Now, the next day was certainly a different story. I had pushed myself too far.  My fatigue was complete and overwhelming and my entire body ached.  My neurological symptoms were also  rearing their ugly head. I had crossed the tipping point of overdoing it and I crashed.  It took me a two day couch-rest to recover and begin to recoup some of my energy. 

Was it  worth it? Most definitely. I won't make a habit of overdoing it and using my energy sparingly  is critical, but there are occasions when the benefits outweigh the after effects.  The  trick is choosing these rare occasions very wisely and not too close together.  I do wish there were a monitor that would alert me each time a rest is needed before the fatigue sets in.