Ganglionopathy/Sjogrens
 

Hello fellow neuro talkers,

I come to you in desperate need of help and guidance due to a mix of perplexing symptoms over the last 8 months in-particular (However I do feel like this is something than began around 5 years ago, but symptoms settled for some time).

My initial set of symptoms began in 2011, with the onset of the following symptoms: burning dry mouth, numbness and tingling in arms, burning pain in shoulder, severe lower back pain, rashes & onset of extreme depression overnight. I was tested auto-immune due the number of conditions in my family (Lupus, Neuro Bechets & RA), but the settled on a diagnosis of depression and after around three months the symptoms settled and I continued life as normal (numbness and tingling continued periodically but didn’t affect my life as such).

Fast forward to 2016 and the symptoms seem to return and then some. All of the symptoms above returned but with some more nasty symptoms, including drying of all visible passages throughout body, swelling of cheeks in face, numbness on right side of face, loss of taste, sexual dysfunction, extreme coldness in hands and feet, sudden onset of a lot of eye floaters facial flushing, waking in the middle of the night with my heart rate going through the roof, extreme sweating through the night, electric bolts throughout my body, constant itching, intolerance to alcohol (and the list goes on). To accompany the symptoms I once again came down with severe depression overnight, it’s like the level of something changes in my body and I turn into a person I do not recognise (outside of these two occasions I am a very positive person and it’s never been a demon I’ve struggled with).

Once again the medical investigations began, firstly with the doctor suggesting the symptoms were caused by a sinus infection, secondly referring me to a rheumatologist (who did blood tests for Lupus & Sjogrens) and said there was categorically no sign of auto-immune and wanted me neurologically tested. Prior to seeing the neurologist I underwent Nerve conduction studies and an MRI (both clear). The neurologist has ruled no sign of any neurological damage and has suggested something has upset my nerves which has resulted in my depression & anxiety. I sought a second neurologist opinion as by this point I had looked at symptoms online prior to the appointment and felt quite convinced that the cause behind this was sjogrens, in particular neurological damage by sjogrens (my reading online always leads me back to Ganglionopathy, as the symptoms described seem to fit). The second neurologist disagreed with this assumption and said I showed no symptoms of autonomic dysfunction he would expect to see (issues with heart rate), and came to the conclusion of a “brain stem mediated central pain hypersensitivity syndrome” and prescribed anti-depressants. Four months after beginning the tablets I am still in the same position in terms of symptoms, I am managing to work every day but my quality of life is not very good. There is no other options for me in terms of medical assistance as the NHS has referred me both to a rheumatologist and neurologist. The only option I can consider if going private to a rheumatologist and requesting a lip biopsy but it is not guaranteed they will give this to me, especially as two doctors have suggested it’s depression.

I understand that a lot of the symptoms I have explained can link to depression but I believe 100% that there is something further causing this. Has anyone had any experience with the symptoms listed above in relation to Ganglionopathy? And also experienced depression alongside it?

Thanks so much for your time in advance,

Warm wishes,

Kirsty

Hi Kirsty

Welcome to NeuroTalk.

I am in remission from Major Depressive Disorder. When I was not in remission I experienced some of the things that you have described. I do not have any signs of ganglionopathy or similar.

I was on an anti-depressant for some years. After a while I decided to discontinue, with approval and monitoring from my psychiatrist. This was uneventful.

Another option which you could consider is seeing a clinical psychologist with experience of using Cognitive Behavioural Therapy (CBT) with his/her clients. CBT takes time and practise to learn but there is good evidence that it can be highly effective for people with Major Depressive Disorder and other mood disorders.

Using CBT methods, which my clinical psychologist taught me, has been invaluable in my staying in remission.

All the best.

Did your symptoms return during a seasonal short day length time? (shorter sunny days of winter?)

If so you might investigate light therapy designed for SAD--seasonal affective disorder.

When I was still working long hours before retirement, I often went to work in the dark and came home in the dark (long days) and started to really feel awful at those times. The light therapy worked very well for me and I still do it each autumn...but I don't need it for as long as I used it at the beginning.

This is the visor I use:
FeelBrightLight.com – The World's smallest, most convenient light therapy device. – Feel Bright Light Deluxe

I notice that the price has come down quite a bit since I bought mine. I keep mine on a trickle charge and it still works after several years! I have avoided antidepressant drugs using this light source only 20 minutes in the morning, on the lower setting.

Some of your symptoms suggest to me low thyroid functioning. So if you haven't had thyroid testing, I'd get that done too.

There are light panels that will sit on a desk or table, and some of them today are quite good. Just Google "light therapy for SAD".

Hello Kiwi33 & Mrs D,

Thanks so much for taking the time to reply, very much appreciated.

First to answer you Kiwi33, do you really think that depression can cause such physical symptoms? I’m not foolish enough to not consider that having a couple of specialists saying similar things makes the possibility of depression causing this, but I find it hard to believe it can leave such physical, long-lasting symptoms? I think having seen many family members deal with the weird and no-so wonderful effects of auto-immune disease I am very aware and very quick to link back strange symptoms to it. For instance the dry eyes, nose, mouth initially led me down the sjogrens route, and then with the neurological symptoms led me down the ganglionthraphy route. It’s so difficult having these symptoms coupled with the depression onset as even-though I know that the symptoms were very real it makes me doubt myself.

I have in the hope of helping myself signed up for some CBT sessions which begin next week. I will try anything to help me ease these symptoms and make life better.

Mrs D these symptoms did being in September so the nights were getting darker, and I have had my thyroid tested but everything is fine there. Will look into your suggestion for sure.

Thanks,

Kirsty

Kirsty, the physical symptoms associated with my clinical depression were waking up at night, extreme sweating and panic attacks. These have largely resolved through using CBT and deep breathing exercises. They may have exacerbated by self-medication with alcohol (I am also in remission from alcohol abuse).

As far as your possible Central Pain Syndrome Dx is concerned as far as I can see, based on https://rarediseases.org/rare-diseas...pain-syndrome/, this most commonly arises from physical brain injury.

I don't know if that is part of your history but the information in the link might help you in discussing this with your health care team.

You could have more than one thing going on with you.

I went to a medical seminar when Prozac first came out given by
Eli Lilly. There was a psychiatrist giving the talk. He made a statement that was very illustrative of how dismissive doctors can be of their patients. He said when confronted with a patient giving a long list of symptoms, (he called this the "organ recital")
just prescribe an antidepressant, because that is all that is needed.Then began the discussion of how tricyclics are so much more toxic than SSRIs and to switch patients to Prozac now.

I have never forgotten that day...I lost much of my respect for doctors then.

Dry eyes, nose mouth etc can be from OTC drugs, RX drugs and certain vitamin deficiencies. There are papers on PubMed about dry mouth responding to lipoic acid, and biotin. There is a poster here who had painful dry mouth and found relief from
a dopamine agonist used for Parkinson's!

You can get dry mouth from extreme fear or anxiety, which is an adrenalin response. But that usually is temporary.

I think your symptoms are very mixed. To blame "depression" is lazy and perhaps not helpful.

Hi Kiwi & MrsD,

Again thank you for your time!

I hadn’t had any physical brain trauma prior and the neurologist made it seem like it really wasn’t anything to worry about – so maybe something slightly different to that? How he explained it was that my brain is letting into much noise, which is making me very aware of everything.

Yes Mrs D, I very much feel like me presenting with such an onset of symptoms made them put me right in that category. From my GP’s perspective it was a little more gradual as I was presenting to here with the different symptoms as they began – but when the referral appointment happened it was six months later and I had a list the length of my arm.

When this first happened five/six years ago I had recently lost someone very close to me so I found it easier to accept the depression diagnosis, but it was still incredibly out of character and I still had this gut feeling that there was something more behind it. When it struck this time I couldn’t have been any happier, life was great, I think the pain in my face & drying of nasal passages slowly got to me over a couple of month previous, but nothing else.

The thing that unnerved me this time and made me think it was definitely something neurological was that I was very aware of each part of my body it was affecting at any given time (My mum tells me I’m crazy when I tell her this and I didn’t really explain it to the neurologist as I was worried he would think the same) – but for a two/three day period when the symptoms peaked around Christmas I had very weird things going on in my body. This ranges from me having the strangest cough (not like anything I had experienced before), which lasted for around half an hour and literally took my breath away, to having the feeling that my genital area was aroused for quite some time (without me being in that mood whatsoever) – post this the sexual dysfunction began and it, which has resulted in my body not responding in sexual situations whatsoever, to having the feeling that you get when you’re on a theme park ride (adrenalin rush I guess) while just sitting on the sofa - to feeling jolts of electricity, which resulted it lots of bruises on my legs (where I was feeling the jolt), to waking up each morning at exactly the same time (3am), sweating around my waste, it really was a strange couple of days and it has scared me to be honest.

My GP has exhausted all blood tests possible (including vitamins) at this moment and nothing showed up, obviously she has to take the professional recommendations on board too, I’m just scared that I could be allowing something that is already causing me such issues to progress. Another thing that worries me is that around a month before these symptoms began I used Aldra cream on a wart, I thought nothing of it at the time, but if I did have some immune issues underlying and I revved up my immune system – it wouldn’t have helped what so-ever.

I am currently using eye drops, coconut oil & Xyimelts for the dryness but I have read a lot about lipoic acid so I may look into this too!

Sorry to be so late in reply. I haven't been on the board much lately.

Your symptoms certainly sound like Sjogren's and I would suggest you request a lip biopsy. The labs for Sjogren's are often inconclusive and 40% of those with Sjogren's have negative labs anyway.

The lip biopsy would confirm/deny the Sjogren's. I don't see much sign of neurologic involvement in your symptoms listed. Your doctor is right, typical symptoms of autonomic dysfunction would include BP and heart rate changes...especially when standing. The other autonomic symptoms would include temperature intolerance (not just night sweats), urination problems (difficulty starting to void and also complete emptying), GI involvement like gastroparesis, vision changes (double vision), livedo reticularis, as well as a few other not so common symptoms.

They certainly haven't done a thorough work-up for you (at least by your reporting) and you should immediately ask for a lip biopsy to confirm the Sjogren's. Most of your symptoms can be explained by the Sjogren's alone...even the swelling in the cheeks (swollen parotid glands is common in Sjogren's). You would definitely benefit a proper Dx so you can be treated accordingly.

Hi En Bloc,

Thanks so much for your reply. I have read through many of your posts and feel you are far more knowledgeable on the Sjogrens topic than any Doctor I have met as yet, so was hoping you would reply.

I have made a private appointment with a specialist Sjogrens rheumatologist for next week and I am going to beg for the lip biopsy. My GP who I visited again this week stated that I wouldn’t be treated any differently if I was diagnosed as it’s just a symptomatically treated disease, just the answer you want went not feeling top dollar.

I guess at the moment the two main neurological symptoms for me are the constant numbness in upper mouth/face, the lack of normal feeling in other places, so more sensory. However I do think I possibly suffer for gastro issues in terms of a weird constipation, it’s not overly painful it just feels like its stuck a lot of the time & I am pretty much always full, no matter how little I eat.

When I was going through what I would probably class as a bad flare over Christmas/January, I did have a lot more of the autonomic symptoms, including the dizziness upon standing, struggling to deal with the very cold temperatures, increase in frequency of weeing – but it was mainly the sexual dysfunction & dryness that led me to the autonomic symptom route. I guess it would be very unlikely that it would permanently damage one part of my ANS and the others return to near normal when the flare is over?

The lack of understanding of Sjogrens in this day and age and the lack of drive for specialists to thing outside of their very rigid box really does annoy. I also find it ironic that I spent around 3 months back and for the doctors with severe dryness issues and sensory issues and did not do any of my own research – yet the moment I arm myself with information they get annoyed and tell you what you have read is incorrect. If this anything more annoying that being unwell – it is having to pretend to not have a clue what’s going on to massage an ignorant person’s ego. Sorry – rant over, this process is turning me into not the best version of myself – it will change, I hope :0.

Thanks,

Kirsty

Kirsty,

Not sure where you are located, but there are many Sjogren's specialist in the U.S.

Wait until you see the Sjogren's specialist before believing that treatment is just symptomatic...it's not. There are many options for treatment...from DMARDs to steroids, to biologics.

It is quite common for autonomic symptoms to wax and wane. So if you are having these symptoms, then by all means bring it to the rheumy's attention and try to document when these happen (by taking BP and heart rate upon standing and journal results). Make notes so you can better present your case to the doctor. Showing a journal of symptoms makes things much clearer for them to see the entire picture.

Hope this information is helpful. Let me know if you have any other questions or ways that I can help you understand this disease process or how to present your case to the doctor and get their attention.

Hi en bloc,

I am based in London in the UK - apparently there is one rehumy who has a special interest in Sjogrens, so I have my appointment with here.

I have a collection of notes dating from when this first began in 2011, I will organise and take along with me. I hope as I am paying for this privately that she will allow me to have the lip biopsy regardless of the lack of evidence in any blood results.

Another random question, which I seem to have flagged as a pattern, do hormones play a part in this illness? I without doubt notice a flare around the time of my period, is that common?

Thanks,

Kirsty

Many people with Sjogren's find this to be true. But I don't know if it has been confirmed medically, or what the mechanism of action would be causing the flare.

Kristy I haven't had a chance to read all that you posted but did either of the neurologist do a skin biopsy for small fiber neuropathy. This will not show on an EMG. I had a lot of your symptoms when I first was diagnosed with SFN. I did not get depression right away though. I think that came later after being in so much pain.

Hello all!

Some positive news, I met with a rheumatologist who specialises in Sjorens. Her first words were you can't discount Sjogrens based on the blood test alone (music to my ears). She carried out a schirmer test, which showed I was below normal in both, one much more than the other. She also did the saliva test which showed I was under.

I have been booked in for an ultra sound on my glands in two weeks and then we go from there.

@Hopeful, I raised the concern of the small fibre neuropathy with both neurologists but they said I did not show burning pain so it was unlikely. I know there are def some neurological things going but my hope is if I get the sjogrens diagnosis the neurologists won't just palm me off as a depressed young girl. (When I say young I am 30, but in these appointments with doctors they make me feel about 12, dumb and 12 to be precise).

Also En Bloc can I please ask you another sjogrens related question.

A big issue of mine is the dry nose. I have gone from someone being full of snot (due to allergies), to having pretty much nothing (which makes it very uncomfortable now my allergies have kicked in). Do you suffer with that?

Thinking about it logically my body has gone from receiving lots of mucus from my nose, mouth. Is there something you take to help with dryness of the digestive tract? Are there any substitutes I should be taking for what my body is missing from my saliva (digestive enzymes etc.?)

Thanks so much,

Kirsty

I do have extreme burning with small fiber. I happy for you that she is testing further. You are young but that does not mean they have the right to make you feel dumb. Do your research and you in there sounding like you are well informed. If they make you feel dumb nicely request the speak to you in laymans terms. I've worked with doctors who do that sometimes they need to be called out on it.
Good luck with the future testing!

Your sinuses absolutely suffer when Sjogren's is present. And the sinuses don't drain properly, causing more frequent bouts of sinusitis, etc.

There are many products to help keep the nose comfortable...saline sprays (make sure they are just saline, and not medicated. Also Ayr gel/ointment helps keep the inside of the nose moist, cutting down on nose bleeds from dry air and dry skin inside the nose.

There really isn't much you can do to replace saliva in your GI tract. All organs can suffer from lack of moisture. Just make sure to drink plenty of fluids. If you have GI problems, you really need to address them with a GI doctor for specific instructions. Don't add digestive enzymes unless you been tested and found they are deficient. Just focus on a balanced/healthy diet, fluids and if any problems arise, then get them checked out.

I also take a generic form of Mucinex, twice a day. This help to thin my mucus, which is very thick due to my severe dryness.

Regards, ElaineD

Thanks both! Am fully stocked up on products to help now - you have to be very organised with this illness hey!

Some further developments on my search for answer, the ultra sound actually showed severe shrinkage of my lacrimal glands. The partroid glands apparently look normal sized at the moment, so it seems to have attacked my eyes quite heavily first. Next steps will be lip biopsy which the rheumy says correlates with this.

I actually got a call from my doctor this week who said they had tested my blood for Lyme disease and it has come back positive, but I need another test to confirm this. I have read somewhere that this can test positive sometimes in people with auto immune disease, so we'll see what that brings too.

Just very happy to be moving forward with this rather than just being looked at like I was crazy. Hopefully if they can get to the bottom of it they can actually help me address the neurological issues :).

Thanks for listening!

Kirsty