MS or sjogrens...waiting for MRI again

[trc1962]

After 12 years we are back to going through all the MS stuff again to rule it out. I am trying to educate myself so I can ask good questions in regards to what is seen on the MRI. I have a "probable" sjogrens diagnosis secondary to celiac disease based on a positive schiermers test, a suspect lip biopsy and other connective tissue stuff...raynauds, livedo recticularies, hair loss, facial and scalp sun sensitivity, raised CRP often but no positive blood markers that typically point to sjogrens. I developed a vascular type neuropathy 12 years ago that was painful but in time responded to low doses of aziathiaprine and stayed fairly quiet for many years. I had facial nerve involvement with that as well and it really bothered my left quad muscle. Now I am looking for good info that might help me prepare. Can you have a lesion on the spine or areas of inflammation and have it be sjogrens? My rheumy doesn't seem to have a lot of experience with sjogrens and he just says the radiologist can decide what he sees...makes me kind of nervous. Input appreciated, even just a link to look for. My MRI isn't until Sunday night, which is better than 3 weeks ahead they wanted to book me for, but still a ways to wait if this is a CNS type flare.

[Jasper]

I nee to post this in two posts due to the limit on the number of characters per post: So this is post no. 1:

"CNS or Central Nervous System complications of Sjogren's syndrome, Dr. Julius Birnbaum, Johns Hopkins:"

"Myelitis"

"Patients with Sjogren's syndrome may have 'myelitis,' which is inflammation of the spinal cord. Myelitis can cause weakness, numbness, and difficulty with urination and/or defacation. Myelitis can present quickly (i.e. within hours). However, symptoms due to inflammation of the spinal cord may evolve more slowly, progressing over weeks or months. This slower progression may be difficult to distinguish from Multiple Sclerosis (MS). Unfortunately, patients with myelitis and Sjogren's syndrome can be misdiagnosed with MS. Appropriate tests, which may include spinal tap and MRI of the brain/spinal cord, can lead to diagnostic clarity. The importance of accurately distinguishing between Multiple Sclerosis and Sjogren's syndrome is of paramount importance. MS and Sjogren's have completely different treatments. The approved treatments for MS are ineffective for Sjogren's syndrome, and may actually lead to ?flares? or worsening of Sjogren's syndrome.

Patients with Sjogren's syndrome may have another autoimmune disorder called 'Devic's syndrome,' or 'Neuromyelitis Optica.' Neuromyelitis Optica (NMO) causes inflammation of the nerves connecting the eye to the brain, or 'optic neuritis,' as well as myelitis. The pattern of myelitis which can occur in Neuromyelitis Optica is much different than Multiple Sclerosis. Typically, the myelitis in Neuromyelitis Optica is more severe, causing severe weakness, and may cause future relapses.

Again, the treatment for Neuromyelitis Optica is much different than MS. Patients with Sjogren's syndrome, who are diagnosed with MS, need to be skeptical about receiving these dual diagnoses. Below, I offer some helpful hints, which may especially cast doubt on the diagnosis of MS.

Red flags for a diagnosis of Neuromyelitis Optica

Answering 'yes' to the below questions should especially raise consideration for the diagnosis of Neuromyelitis Optica.
(1)  Have episodes of myelitis been especially severe. (i.e. associated with inability to lift the arms or legs in the air)?
(2)  Have there been multiple relapses?
(3)  Have episodes of myelitis been associated with 'optic neuritis,' or inflammation of the nerves connecting the eye to the brain?
(4)  Have MRIs of the spine shown inflammation which extends beyond three 'back bones' or vertebral bodies?

Any patient with Sjogren's syndrome, a history of myelitis, and answering 'Yes' to the above questions should have a blood test which may assist in the diagnosis of Neuromyelitis Optica. The name of this blood test is the NMO-IgG antibody, and is performed at the Mayo Clinic. It detects Neuromyelitis Optica in 70 percent of cases. This means that it is not a perfect blood test and will be negative in 30 percent of cases. In such cases, consultation with a neurologist with training in neuroimmunological disease is crucial."

[Jasper]

Post no. 2, more of the article by Dr. Birnbaum:

"Red flags against a diagnosis of Primary-Progressive Multiple Sclerosis"

"Multiple Sclerosis is not a single disease, but has different presentations, with different tempos. The most common type of MS is called 'relapsing-remitting' MS, and is punctuated by episodes of unequivocal deterioration (called 'flares'), versus periods of clear improvement and quiescent disease. However, a different type of MS may be confused with Sj'gren's syndrome. This pattern is called 'primary-progressive' MS. In such cases, the crescendos of worsening and improvement typical of 'relapsing-remitting' MS are not evident. Instead, there may be a slower and more gradual period of deterioration, reflecting inflammation and damage in the spine (i.e. progressive myelitis).

Patients with Sjogren's syndrome who receive a dual diagnosis of primary-progressive MS need to be especially vigilant about diagnostic misimpression. Specifically, there is no effective treatment for primary-progressive MS. Any patient with Sjogren's who receives a diagnosis of primary-progressive MS deserves a trial of immunosuppressant medication which may be helpful if progressive myelitis is occurring because of Sjogren's syndrome.

It is often difficult to distinguish between primary-progressive MS and Sjogren's disease. Patients with Sjogren's syndrome may have less brain disease on MRI compared to patients with primary-progressive MS. In addition, patients with Sjogren's syndrome may have lesser amounts of protein in the spinal fluid, called 'oligoclonal' bands. Therefore, any Sjogren's patients with a diagnosis of primary-progressive MS needs to have brain MRIs, as well as a spinal tap.

Conclusions
The most common CNS complications of Sjogrens syndrome include myelitis and optic neuritis, which are syndromes that can occur in MS. Because of this symptomatic overlap, patients with Sjogren's syndrome may erroneously receive a diagnosis of MS. Because the treatments for MS are not only ineffective for Sjogren's syndrome, but may actually precipitate flares of Sjogren's disease, accurate distinction between MS and Sjogren's syndrome is crucial. When the myelitis is particularly severe, associated with optic neuritis, or involves extensive regions of the spine, then the diagnosis of Neuromyelitis Optica should be considered. Spinal taps and brain MRIs may help in the distinction between primary-progressive MS and Sjogren's syndrome. All patients with myelitis in the context of Sjogren's syndrome deserve a trial of immunosuppressant therapy."

http://www.hopkinssjogrens.org/disease-information/sjogrens-syndrome/neurologic-complications/

[Jasper]

Here is a link to the slides from a presentation by Dr. Julius Birnbaum. The section on Sjogren's starts at slide 18.

https://www.ncrheum.org/wp-content/uploads/2015/03/Neurological-Complications-of-Lupus-and-Sjogrens-Syndrome-Birnbaum.pdf

[trc1962]

Much good information to go through - thanks very much!

[jazzlover]

an MS diagnosis is one of exclusion. There is no definitive test.

Be sure to also consider Lyme disease as a possibility. Igenex Lab is the best. The lesions on the brain can look the SAME in Lyme and in MS.

www.everydayhealth.com/multiple-sclerosis/symptoms/conditions-commonly-mistaken-multiple-sclerosis/

www.dailymail.co.uk/health/article-2343062/Mother-diagnosed-MS-facing-life-wheelchair-cured--discovered-symptoms-TICK-BITE.html

[trc1962]

I suffered many tick bites as a child so I will remind my rheumy of that. I had a brain MRI in March to check for an acoustic neuroma (due to the ear ringing) and nothing had changed since 2003 2 small white area that are considered UBO's. Now they are checking the spine but that was clear in 2003 as well. I am just hoping they can do something for the discomfort as my lips are evening burning today. To think on June 1st I felt really normal and it has changed slowly since about the 10th. Life is crazy - I am trying to feel positive and get this turned around. Many thanks to all.

[jazzlover]

A rheumy will ignore it. If they even take it seriously, they will run a test through whatever lab they use. It likely will not pick it up .. but you COULD get lucky.

[trc1962]

I have found  that the rheummy's I have seen don't take Lyme's disease very seriously as a whole, but it seems like they sure should as that is an actual toxin. Anyways, after feeling a lot of pain today, especially in my legs the rheumy says that if the MRI is negative then he suspects small fiber neuropathy and time for another skin biopsy. He was pretty insistent that sjogren's is mainly dryness and the nerve stuff is almost non existent and idiopathic- not what I have heard on here though. It is weird to have most of a day when you hurt and feel so bad and then it shifts so it is much better...and I know I am not crazy. I found out today there is another health and fitness teacher in the district that has had sjogren's for many years and she has some crazy symptoms that come and go as well.

[gurs]

Another thing about Lymes...a few years ago I had a doctor send my labs to Igenex for Lyme testing. Several bands came back positive. After talking with about 20 specialist, they all concluded that Autoimmune diseases can interfere with the lyme testing and actually make these markers positive. I didn't recall being bit by a tick? very confusing?

I think Sjogrens and my hormone issues from menopause causing most of my issues. Most of my major issues started
shortly after a total hysterectomy and instant menopause. You would be surprised how many things hormones, primarily menopause can effect, including the CNS system.

Gursie

[jazzlover]

Gurs... doctors do NOT want to find Lyme because they do NOT want to treat it. You have to find a doctor who specifically treats Lyme.

Other doctors will find reasons to tell you the test results are "false positives."

Not everyone remembers or ever sees the tiny tick .. the size of a period in this sentence.

[SJSman]

Hello,

I went through all the tests and procedures that are being discussed here, because my Sjogren's presented with MS symptoms and I was almost sure I had MS.  Here are a few observations - I hope this helps.

--When you have a head/brain MRI on a suspicion of MS, they typically take a lumbar puncture/spinal tap at the same time.

--There is a standard suite of tests that are performed on spinal fluid samples.  As I recall, about ten or fifteen diseases are tested for, including syphilis which surprisingly can lay dormant for up to 50 years before becoming active!  Back to the subject, lyme disease is on the standard suite of tests.  People obsessed with Lyme disease may question the accuracy of the test, however, the point I want to make here is that, if you have a spinal tap, you are automatically being tested for lyme disease.

--The suite of standard tests that are typically performed on spinal tap samples makes this a great procedure if someone is searching for a diagnosis like I was for years.  I had to fight hard to get it against huge resistance from an evil team of cost-minimization HMO doctors. 

As it turned out, this combo procedure was the Golden Key I sought so desperately for so many years, as it provided critical information that convinced doctors that I was in fact extremely ill as I had been saying for years, and indirectly but quickly caused me to be correctly diagnosed with Sjogren's.  If you are seeking a diagnosis and have symptoms like MS or auto-immune, the MRI lumbar puncture (spinal tap) combination can be invaluable.  Remember, negative results are almost as valuable as positive ones in seeking a diagnosis, because you can rule things out and move on.

--MS is a disease where the myelin sheath is eroded from the outside of nerves, causing then to short circuit.  This can be potentially be picked up on emg tests, depending on where the myelin is missing.

--In MS, there are frequently white-matter hyperintensities that are readily visible on the FLAIR Series of the MRI.  In MS, these are typically grouped into a specific area on the rim of the corpus callosum.  If white-matter hyperintensities are grouped here, then that is almost certainly caused by Multiple Sclerosis.  MS is not a disease of exclusion - there is plenty of clinical evidence in a positive diagnosis, there is just no "one and done" test for it.

--In repeat checkup MRIs at 6 month or year intervals, MS lesions may disappear because the myelin can heal, although new lesions may form at the same time.  So in MS, the MRIs over time provide a definitive diagnosis of MS, due to disappearing lesions, and grouping of lesions along the rim of the corpus callosum.

--In Sjogren's, white-matter hyperintensities may, or may not, be present on the FLAIR series of the MRI.  If they are present, instead of being grouped along the corpus callosum, they are typically diffusely distributed throughout the white matter.  Unlike MS grouped and vanishing lesions, Sjogren's lesions are non-specific, because identical lesions are seen in all four major types of dementia, and also everyone gets some lesions in their white-matter as they age anyway.

--Sjogren's lesions never vanish - they are dead tissue instead of missing myelin. 

--I have been though all of this, and more, including an eye problem Odyessy.  I am diagnosed with Primary Sjogren's with Secondary Central Nervous System Vasculitis.   I did visit John Hopkins while on my horrendous Mystery Diagnosis Odyssey, but that is a small part of a very long story.

--MS is absolutely diagnosable, however, it is one of those illnesses with non-specific symptoms that takes years for a team of doctors to narrow down the suspected diagnosis to MS, so that you start getting the definitive suite of tests that diagnose MS.  It is most certainly not a diagnosis of exclusion. 

One enormous problem is that one has to have continuous health insurance for many years to chase a MS diagnosis to conclusion.  That together with the fact that the tests for MS, such as repeated MRIs and spinal taps, and repeated emgs, are expensive and time-consuming for the ordering doctor to manage, contributes to the fact that, in my experience, I estimate that only half of the people who say they have MS for years or decades have ever had it conclusively diagnosed.   

If anyone is interested, I could continue commenting.  Please let me know, and I am quite willing to share additional comments.  I don't like writing long replies that no real person is interested in reading.   

[quietdynamics]

SJSman just gave an excellent post.
I can sympathize with the frustration of trying to ascertain the cause of so many symptoms; they leave some Dr. scratching their heads ( which makes us even more baffled, concerned). Hey, I want my life back.. it wasn't perfect, but it was mine!

At first Drs. considered MS due to symptoms, had lyme done, etc. On and on..
Worked with students and was having a great deal of difficulty due to muscle weakness, overwhelming fatigue, cognitive dysfunction, constant nausea,bouncing off walls in the morning,  etc. Would go home and crash.

Two years ago, after many years of treatment (plaquinel and pilocarpine/ Rheum, other meds for Fibro pain and then from Neurologist) went to Sjogrens Center and new protocol.  No symptom mentioned was overtly dismissed, some discussed, some new to me that had never been noticed.. such as
livedo recticularies, other tests done (D and B defiecient.. theraputic doses helped with bone/muscle pain)
Methotrexate was new treatment protocol and for me I have found that while not gone the ringing in my ears is less ( goes up a pre-flare.. so I back down) and have been able to lower doses of other medications. We are discussing option of injections.
Sjogrens can show white brain lesion in brain (google images)

During the course of this journey (10years) I have gone to the ER 3 times.. and they have always been for " stroke-like" symptoms. Which can be similar to MS. Paralysis of limb (left leg), lose of control of eye movement, room going dark and tilted, stopping at red light in town and not knowing where I am, just crazy, bizarre stuff. Stuff which if it were not happening to me, I might not believe. During a severe flare even the sound of someone coughing felt like highly unpleasant electric waves going through my brain.. so overall, systemic inflammation 

So far with the new protocol things are holding.. not the old me.. but good. The hair loss stopped and some is growing in.

I am wishing you well with the MRI.
I would consider sending labs and MRI to a Sjogrens Center for review.. can't hurt.
Even though I was originally seeing the Chief of Rheum @ a teaching institution I went for a second opinion and I am so much better for it and the Chief Rheum learned from it as well. The Chief Rheum I know was being conservative in treatment, but did do reseach after seeing the improvements and remarked that some pts. present with Neuro symptoms first, which was my situation. Or at least they were more pressing.

Effects of low dose methotrexate on relapsing-remitting multiple sclerosis in comparison to Interferon ?-1?: A randomized controlled trial
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3214348/

[trc1962]

My white matter abnormalities are considered to be consistent with celiac disease (not off gluten in early 2000's) and sjogrens are I have had them looked at by many specialists and non say MS. That said, I am relieved to have it all being checked out as I want to match up the treatment with the problem - and the diagnosis has never totally made itself clear. I won't do a spinal tap unless it is really necessary as I have a chiari malformation and the pokes leak spinal fluid even with a couple of blood patches and recovery is awful. The dr. originally wanted to put me on methotrexate and I asked for Cellcept because of the hair loss from methotrexate. I would now consider the methotrexate even with the hair loss as having my function is bigger than everything else. I don't have any weird neuro symptoms besides the ear ringing and that seems to worsen when I consume salt or citric acid (sounds crazy I know) and I have mast cell issues of some type as well and try to eat low histamine. The last 12 years there has been no change on my MRI's, so hoping for the same tomorrow and then maybe a direction to go in. I so appreciate the great information on here and how autoiimune effects everyone so different. My TSH has been high (over 10) for over a year and I am reading that could cause issues. I am told they can't treat it until I am off the prednisone which I wonder could not help my flair. I will update and ask more questions. Dr.'s don't always like lots of questions (although my rhuemmy is patient and answers well) the neuro not so much, but this is my life and I won't put my head in the sand. Hoping everyone has a good Saturday.

[jazzlover]

Approximately 20% of spinal taps will show active Lyme. (even when it's present)