Mononeuritis multiplex and Vasculitis (Am I overdoing this?)

[maya]

I was seeing a neuro for about 6 months (after an EMG and an MRI couldn't diagnose anything useful, finally gave up on me and asked me to visit again only "if necessary"). A few weeks back, I happened to go to my home country and met another neuro to seek second opinion. He did 2 things that were not done here earlier - ENMG (he told it's different from EMG) and Nerve biopsy.

Here is the result from each report:

ENMG: Mononeuritis multiplex axonal.
Nerve biopsy: Inflammatory neuropathy with non-uniform chroinc axonopathy, consistent with vasculitis; left sural nerve biopsy.

He says I've Vasculitis. He also believes very strongly that I've Sjogren's (blood work -ve so far but I've all usual symptoms) and that Vasculitis is Sjogren's related.

I'm back to US now. Of course, want to change my neuro here (he has brushed me aside anyway). My GP wrote me a prescription to see doctors in Georgetown hospital. She told me that there are really good neuros there but appointment will take weeks to months!

Of course, I'm anxious and stressed about this. Is there anyone else who has been diagnosed with Vasculitis with Sjogren's. I don't have any specific questions to ask but general googling of Vasculitis has scared the crap out of me. Neuro (the second one) told me that generally, Vasculitis with Sjogren's is not as bad as other systemic Vasculitis.

Again, no questions. If you can share your (similar) experiences, or maybe some resources that throw more light, or just maybe some words of encouragement to keep me going until my next neuro appt - will all help a lot.

BTW - have been told that I'll lose the job. Don't know what I'll do w/o an insurance. Right now stressed +++.

[Shell]

Hi Maya,

I am sorry for the scare you are going through.  I completely understand the anxiety related to vasculitis.  I am not diagnosed with Sjogren's or any other AI, just have a collection of symptoms, the most troubling of which are neurological.  When my GP sent me for rheumatologic evaluation, vasculitis was one of the conditions she suspected, so I've done my share of reading about it.   I know in the past there were a few others on this board who'd posted their experiences with vasculitis related to Sjogren's, so hopefully they will come along to offer more insight.   

It sounds as if your GP is agreeable/helpful since she has made the referral to Georgetown neurology.   With your recent test results, I personally would ask for (truthfully, I would probably demand it  ) an evaluation by a rheumatologist as well, especially considering that neuro #2 believes you likely do have Sjogren's.   You have access to Georgetown, GW, and Johns Hopkins, all with rheumatology departments.  I live in the area, too.  We're lucky to have major medical institutions nearby.  I have heard good things about Georgetown (personal recommendations about 2 of the docs there) and see a great doc at Hopkins.  I don't know much about GW.   Your GP, if willing, should be able to refer you.   In the past, Georgetown rheum dept required that you (your doc) send your info/records for evaluation prior to scheduling an appt.  Not sure if that is still the process, but my recollection from my conversation with them was that once your records were evaluated and you were "accepted" as a patient, that you could be seen fairly quickly (within a few weeks, not months).  That is what I was told early last year, at least.  I got lucky with Hopkins--my ENT is there and he suggested a second opinion by rheumatology and got me in quickly. 

I am seen in the neuro dept at Georgetown currently and like my doc.  Feel free to PM me if you would like to discuss further.  Not sure what your neuro symptoms are--for me, paresthesias, hx stabbing pains, optic nerve issue, headaches/maybe migraines, pulsatile tinnitus, brain fog, hot/cold sensations.  MRI, EMG, NCV haven't shown much.  Just sensory deficit/neuropathy on exam. 

I wanted to share the following website that might be helpful to you:

http://www.neuropathy.org/site/PageServer

I hope you are able to get things sorted out promptly.  Again, feel free to PM me if you would like to discuss offline. 

Take care,
Shell

[Linda196]

Sorry you got such a scare, and now have to wait so long to have your fears addressed. Did the neuro who made the DX offer any suggestions for treatment, or suggest what areas are affected by the neuritis/vasculitis?

Vasculitis is a name given to a collection of diseases that cause inflammation of blood vessels...what type it is, and how serious it can become, depend on the areas involved. Each form of vasculitis has its own characteristic pattern of symptoms, much of which depends on what particular organs are affected.

Because your biopsy was of the sural nerve I'm guessing that any vascular involvement is in the skin of the lower leg, where a very common response is a skin rash, and if that's as far as the involvement goes, most of what you read about vasculitis won't apply. It is a scary subject, and needs adequate follow-up, but ultimately its quite manageable.

Good luck with the new rheumy consult, and let us know how things go, please.

[maya]

Linda,

Neuro suggested to give me pulse methyl prednisolone monthly and if no response, to put me on other immunomodulators. There are no rashes on the skin. I've tingling, and numbness on my foot / hands. Sometimes cold hands / feet but no color change. Also - numbness in the throat area (not on the surface but within), numbness on the left cheek just below my lips, difficulty moving my jaws, pain in the jaw etc.

I will see how it goes from here.

Shell,

Thanks a lot for your post. Will PM you to discuss further.

[Chickpea]

Hi Maya

It's understandable that you're feeling scared.  When you first check out the details of vasculitis it seems all encompassing, and troubling.  I think Linda's very wise: everything I've read about vasculitis leads me to believe that it's manageable.  It would be good if your GP could get you an appointment with a neuro/rheumy combination as Shell suggests.  Even better a pair of doctors who have experience with SjS!

I've been along some of the same road as you: an initial diagnosis of Primary Progressive MS, and then years in limboland before a diagnosis of 'SjS with cns involvement'.  My neuro isn't convinced that all my cns symptoms are due to SjS and vasculitis is still on the agenda.  But they're treating me anyway.

It sounds to me as though the neuro you saw was both thorough in his testing, and aggressive in his treatment suggestions.  Prednisolone can help with inflammation, and if your neuro symptoms are caused by inflammation in the central nervous system or by vasculitis you should get some relief.  Immunosuppressants work at the source of the problem by tackling the auto immune system.  I'm currently on the immunosuppressant CellCept and a few other people here have found it helpful.  Others are on Rituxan or Methotrexate.

Do you get headaches and/or migraines?  I've found some correlation between severe headaches and onset of new neuro symptoms, particularly numbness and tingling in my face, throat and tongue.  Has your speech been affected?  I'm still trying to figure out the mystery of SjS cns issues - it sounds as though you're on the same journey.  Welcome!

Post lots and tell us how things are going with you.

Thinking of you - Chickpea

[maya]

Chickpea,

Thanks for the post. I agree with you when you say the neuro was both thorough in his testing, and aggressive in his treatment suggestions. He is young, eager to gain experience, and needless to say inexperienced - which probably explains his approach / suggestions.

I don't know how I'm going to react to Prednisolone and what will be the side effects. I do have fungal infection, which is not good.

And yes - speech is affected (mildly). No headaches actually. Numbness / uncomfortable feeling in throat, anal region, and left foot - bothers me a lot. All three are related. They wax and wane together; stress elevates them all together. In addition, worsening tingling, cold hands / feet, lightness in parts of limbs etc.

Things have been just getting worse since a year (when it all just began to add up). To cope with this all, I started meditation - but well, anxiety / stress are so dominant right now that I'm not able to focus on my meditation routine.

[dbaratta]

Hi Maya:  I too am on this long/exhaustive journey into the neuro complications of SJS.  Dr. Birnbaum from Johns Hopkins posted here to me.  He suggested I talk to my Rheumy, Dr. Vivino in Phila. about mononeuritis multiplex and told me to come to his clinic in Baltimore for evaluation with Dr. Vivino's permission.  I looked up Mononeuritis multiplex and it scared me too. I had two previous EMG/nerve conduction studies over two years both showing neuopathies and radiculopathies.  I had also had a drop foot for which a new nerve root grew.  But recently (for a few months)  have been having deep nerve pain (especially when trying to sleep) in my neck, arms, wrists, low back, hips legs, ankles, toes and severe weakness all over.  I've suddenly having alot of cervical spine pain resulting in arms/shoulders/wrists.  Headaches are getting really bad too. My Rheumy referred me to Dr. Birnbaum's clinic and they finally got me an appointment for the end of September -- there was a note that they would make every effort to get me in sooner but frankly, I'm not sure I can wait 4 more months.  Also having problems with autonomic systems, severely cold, severely hot, bladder, bowels, etc.  This thing is mushrooming into a true nightmare.  Oh well, no choice but to stay with the program--maybe someone will cancel and I'll get to see them sooner at JH.  Just wanted you to know that there are others of us out there with Sjogren's resulting in neuro problems/pains.  Thanks to the others for your valuable insight and I'm going to use one of the links right now.  Hang in there.  I will too.  Diane

[Chickpea]

Hi Maya

Good to hear that you've finally got a diagnosis, and a recommendation for a good neuro.  That's two more steps forward, although it probably doesn't feel like it right now!

Waiting is so hard, especially when you have new symptoms developing.  I can understand why you want to get an appointment as soon as you can, but don't be too disappointed if you don't get much further with the first neuro.  It's good to get lots of viewpoints, but not all doctors are open to the idea that you don't view them as omnipotent!

Have you considered seeing a speech therapist?  I saw one for a few months after I first started having speech difficulties and numbness in my mouth, tongue, throat and parts of my face.  There was no complete answer but it was good to talk through the issues with someone experienced.  I've had to accept that when I'm tired my voice fails, or I produce odd sounds.  I'm learning to avoid busy social situations and my listening skills have developed apace.  That's no bad thing!

Thinking of you - Chickpea

[Dolly Dimples]

 Dear Maya & Diane, how awful it must be for you both in this waiting game ...

I pray that you's will soon get to see someone that can help.

    It is scary to think that one complaint can take one down so many roads..

  Please let us know how both of you go..  bless you's. Hugs Dolly.x