New member with questions on Atypical Facial Pain

1

Hi,

New member here. I’d like to run my history by the good folks here for thoughts, feedback, speculation, wild theories, or whatever.

I have Facepain of Obscure Etiology (the ailment formerly known as Atypical Facial Pain). Of course, no doctor I’ve ever consulted has a clue as to what’s causing it, or how to treat it. Here’s a brief history and description.

In 1991, at age 37, I woke up one night with bilateral tinnitus (I had never had this before). Right around that same time (either a couple of days later, or before – can’t recall) I developed an intermittent sensation of what felt like my hard palate going into strong contraction. Over the next few months, the episodes increased in frequency and intensity, and began to spread to my nose and the area behind it. Within a few months, the sensation became chronic – with a mild-to-moderate baseline, and strong exacerbations, usually brought on by a light touch of my nose or the area above my upper lip. The exacerbations would often last a few days, but at times, even when they got very strong, a good night’s sleep would eliminate it and return it to its lower baseline.

The pain is not the sharp pain of TN (I’ve never experience that, thank God, so it’s not Type 2 TN) – it’s a very uncomfortable sensation of strong contraction, tightness, and a deep aching. The pain is ALWAYS bilateral and completely symmetrical.

Naturally, no doctor I’ve ever consulted has a clue as to what’s causing it, and can only take wild guesses at what might relieve it. I alternated between doing alternative therapies (3 rounds of TMJ treatments (but I don’t actually have TMJ problems), acupuncture, chiropractic, myofascial therapies, deep massage of all head and facial muscles, etc etc), and a few medicinal trials (Nortripyline 75 mg/day, Ergonovine (possible migraine equivalent), Neurontin 900 mg/day, Topomax 100 mg/day) – none of which helped. Mostly I just lived with it.

I have another undiagnosed neurological ailment that also arose around the same time (hard to describe – it’s like having many of the hyperarousal somatic symptoms of an anxiety disorder, but zero anxiety – I’m not a worrier at all. But it’s like having a baseline of uncomfortable systemic tension, which gets strongly exacerbated whenever I get into a state of arousal – excitedly telling a good joke, getting frustrated and arguing with an incompetent service rep, etc. This can set off 24 hours of unpleasant systemic symptoms.)

For THAT, I’ve also tried a number of medications, none of which ever helped, but none of which caused problems.

Then in 2005, a few doctors strongly recommended a trial of Valium. It was the first med that seemed to help the systemic problem I just described. As I look back on it, it was also around this time that my Atypical Facial Pain seemed to become less of a problem (but I’m not sure of an exact correlation – the ATP might have eased a year of so later).

After about a year on the Valium, its effect on the systemic problem seemed to have waned, so I tried to taper off. I hit some very strong withdrawal symptoms, and was only able to get halfway off (from 30 mg/day to 15 mg/day). These withdrawal symptoms have persisted ever since (the main one being a profound startle reaction – unexpected noises evoke hours of systemic discomfort), but oddly, during this whole time, the Atypical Facial Pain seemed to have almost completely disappeared.

Until a few weeks ago. I awoke during sleep, and before I was really awake, my nose was itching, and I furiously rubbed the bottom of my nose. It instantly set off my first episode of the same old strong facial pain in the same area – the hard palate and the nose - just like the old days. It’s now back, constantly, just as it was before, and getting set off more easily than before.

I wanted to treat it aggressively this time, hoping that quickly easing it with medication might prevent it from sensitizing and becoming a chronic problem again. My internist’s first recommendation was Baclofen, and I’ve just done a trial of titrating up to 30 mg/day, with no real benefit. So I’m tapering off of that now and considering my next trial (likely Neurontin, but perhaps at a higher dose than my 900 mg/day trial 12 years ago, which did nothing). I’ll be seeing my neurologist on Monday, but he’s never had a clue as to what this facial pain is all about, so I suspect his only recommendation will be trials of Neurontin, followed by Lyrica if that fails.

But I’m curious to hear any ideas, thoughts, or feedback from people here as to possible causes or other treatments that could be considered. (Although I’m very, very leery of updosing the Valium – I seem to grow tolerant to that quickly, and for me, it’s a monster to try to get off of – updosing might be a last option, but it would likely result in me having to be on it for life, and updosing it regularly, and hoping I never develop a bad side effect that would require me to come off). I’d prefer other medications that don’t have the same degree of tolerance and dependence.

Thanks in advance!

2

Hi

Have you had any MRIs at all?

I am not sure it wil help but google Tornwaldt cyst.

The reason I say this is that I had one and it was removed 2010.

I was diagnosed with ATN in 2005 and they had no idea what was going on but with imagery They found a vessel over my TN and I had an MVD. this made a huge difference for me in 2006'

I then started having facial issues in 2010 and they found a tornwaldt cyst in my synus top of throught that sat on a branch of the Trigeminal nerve. when this was operated on and removed I now have normal every day TN.

The point is the Tornwaldt Cyst was mucking up my diagnosis. whats more it sits in an area that can cause "Some"of what you describe.

Google it and see. The thing is if you have had any imagry it will show. It started very small for me and they missed it for 10 years. size of a pea. when they removed it it was the size of a plum.

The fact that you have bilateral issues and the sensitivity of the palate.

I doubt that this is your answer as Tornwalt cysts are very rare. If they get large they cause huge issues

Hope you get an answer to your issues

Simon

3

I've had several MRI's over the years, most recently a 3T MRI in 2009 - nothing abnormal shows up that would account for the facial pain. But I'll Google your idea. Thanks!

4

The "perfectly bilateral" nature of your pain leads me to suspect that you may be dealing with some sort of systemic problem like migraine or a cephalgia (headache). I've read in the past of tinnitus sometimes being associated with vascular compressions of a ganglion in the nerve which control auditory sensitivity and the balance interpretations generated from the inner ear. However, both bilateral tinnitis and the pain triggers you report for the symmetrical pain in the hard palate region are unlike anything I've ever heard of or read about.

My instinct here (and I will not justify it with more credibility than just that -- an "intuition", if you will) is that it may make sense to taper you up on Neurontin to well above the 900 mg/day dose you've reported from years ago. Maximum recommended dose in that medication is on the order of 3600 mg/day. My wife has been on 2700 mg/day with minimal side effects for over 10 years for bilateral TN/ATN pain. Your neurologist might also want to put you on a trial of Neurontin with a "booster" drug like Baclofen or one of the muscle relaxants such as Flexeril. Another option in the same family is Trileptal (Oxcarbamazepine), and a third and different approach would be Lyrica.

Your hyper-alert reactions seem to suggest that some other mechanism is also acting that might not be directly related to neurological pain. I don't wish to suggest in ANY way that your pain is psychogenic in origin, because I see an abundant lack of evidence for that proposition and I am all too aware of the abuse which many patients have suffered when they are labeled "head cases" by doctors who don't understand what's wrong with them. But that said, I still wonder if it wouldn't be smart medicine to separately evaluate you for both anxiety disorder and bipolar disorder. I must also observe that your reaction to Valium withdrawal is one that I don't believe I've heard before --though this is not one of the medications I have studied in serious depth.

I wish you well in your discussions with your neurologist. I might also suggest that one goal of that discussion would be to arrive at a more definitive diagnosis than "atypical face pain", which has implications of pain originating as psychogenic. It's basically a fanciful way of saying "be damned if I know what this patient has." From your own description of symptoms, I suspect that a more accurate interim label would be "idiopathic bilateral neurological face pain of unknown origins."

Go in Peace and Power

R.A."Red" Lawhern, Ph.D.

Resident Research Analyst, LwTN

5

hi, Red,

Thanks for the quick reply. Yeah, I stump everyone - including you! 8-)

None of my doctors or neurologists ever diagnosed me with "atypical facial pain" - I came up with that "diagnosis" on my own, as it was the only bilateral facial pain syndrome of the hard palate/nose region that I could find. My doctors' diagnoses HAVE always been "I have no idea what this is", although a few neurolgists suggested migraine equivalent (hence the trials of Ergonovine, Nortiptyline, Neurontin, and Topomax, all fairly low dose, with no benefit). None have ever labeled it psychogenic.

I've had the odd experience of neurologists saying, "Maybe it's a very atypical anxiety disorder - like, one without actual anxiety, but just the somatic symptoms". So I've seen psychologists who specialize in anxiety disorders, and after a few sessions of getting to know me, they say "No, you do NOT have an anxiety disorder - you have a very easy-going attitude - y0u need to find a better neurologist who can figure this out." Psychiatrists are a different breed - a quick session, they shrug and say "Who knows, maybe it's an anxiety disorder - do you want a benzo or an SNRI for that?" I've done a number of trials of those, with no benefit (and some moderate withdrawal syndromes from Effexor and Lexapro - and one heck of a withdrawal syndrome from Valium (not uncommon, but mine is longer-lasting by far than most, raising a question of whether the withdrawal symptoms (mainly the highly exaggerated startle response) simply "kindled" or neurally sensitized, to the point that it just established itself - as a side note, one w/d symptom was abdominal spasms, and I was prescribed Dicyclomine, which I later learned has the adverse effect of exaggerated startle, so I had two things - the Valium withdrawal AND the Diclyclomine which can both cause this). In my most recent workup with a highly regarded psychiatrist, in which he insisted on a couple of sessions to get a deeper look at me, his conclusion was "There is NO psychiatric problem here. Not an anxiety disorder, not depression - not anything in my field."

What's most curious about the facial pain is how it's triggered by light touch of my nose or the area above my upper lip. It's so similar to what TN patients report - feeling fine, lightly touch a trigger zone, and WHAM - the pain is on. I've never heard of that with Migraine (I've also never heard of Migraine lingering 24/7, exacerbated for days by light touch). That's why, now that it's returned after a long absence, my research led me to looking more closely at Atypical TN. The pain seems to radiate through the 2nd branch of the trigeminal nerve, but yes, the perfect symmetry of the pain would seem to rule out the main causes of TN (nerve compression or demylenation - it would be pretty weird to have two perfectly matching lesions on both sides). As I study Atypical TN, I see that ATN can cross the midline, but normally the pain differs on both sides. This all leads me to a few follow up questions

1 - what could cause a milder, chronic, symmetrical irritation of both sides of the trigeminal nerve?

2 - are there any tests that might shed light on the facial pain which I haven't yet had (main tests have been the MRI's and MRA's, which revealed nothing that jumped out.) side note - I also did an ABR when my tinnitus altered dramatically a few years ago while doing a trial of a beta blocker to ease the startle reaction - looking for a vascular compression on the auditory nerve - results seemed to rule that out). Since the facial pain eased for years and has just now resurfaced, I'm wondering if any new tests have come on line, or if there were existing ones which I should have done back then, and should consider doing now.

Any thoughts you have on this would be appreciated, as are your "intuitive" thoughts on possible medications.

thanks!

6

I wonder if anybody has tried to check you out for auto-immune reactions, Alex. Lupus can be a strange beast at times, and it certainly attacks multiple body systems in areas other than the face. But some face pain patients also have Lupus as a complicating factor. The Tornwaldt Cyst hypothesis suggested by Simon should also be evaluated. And in the same class, I would wonder if you have any evidence of distributed small calcium deposits or other fluid-filled cysts in gray matter of the brain. I'd think MS would be a serious long-shot as so many of your symptoms are restricted to the head and face and they are so atypical of things that MS commonly does in early stages.

At this point, I think I am "reaching" for low-probability answers to your questions, Alex. One thing I can say, though -- I don't know of any recently emerging definitive tests for characterizing causes of face pain, other than medication trials. You had a long term remission, but it's not at all clear why you did or why the pain has now returned. I don't think testing is going to shed much light on that question.

Sorry I can't be of more help. This is something for a very skilled neuro team to look into. I suspect you might be better served by a regional medical center like Mayo, Johns Hopkins, Cedar Sinai or Mass General Hospital, than you will be by an individual neurologist in local practice.

Regards, Red

7

hi, Red,

Your thoughts are quite helpful. They give me a few more ideas to grill my neurologists on. And with your extensive study of TN syndromes, since you haven't encountered my particular type of facial pain before, that probably saves me from barking up a wrong tree of ATN - although the symptomatic medication treatments of ATN or FOE seem pretty similar to those of Migraine, or of "I don't know what this is, but here are some meds you can consider for symptomatic relief" - mainly anti-convulsants, muscle relaxants, TCA's or a combo.... I'm just finishing up a brief trial of 30 mg/day of Baclofen for the facial pain, no benefit. I'm not sure if that was a minor dose or not.

I've had a consistently mild positive ANA for years, but years ago had a highly regarded rheumatologist do whatever follow up testing he thought was appropriate (I'm not very versed in auto-immune diseases) - he said that all follow up tests were unremarkable, and that when one has a mildly positive ANA, in 95% of cases, it means nothing of significance. But I can re-push that avenue.

I've got a few cavernous angiomas on MRI, but mailed my history and MRI to a renowned angioma doc in Phoenix, who said that none of them would be causing any of my reported symptoms. I can have my neurologist recheck my MRI for calcium deposits or other cysts in gray matter tomorrow.

A regional center might be a good idea. I tried to get into the Rochester Mayo in 2007, but since I live in California, they wouldn't take me, and insisted I could only go to the Arizona Mayo, which I did. They're not any better than the docs here in San Francisco. When the neurologist I got there said "I have no idea what this is", I assumed that, like Rochester, the entire neuro staff convenes regularly to discuss undiagnosed cases. Wrong. The most I could get was "Ok, I'll ask a colleague if I run into one in the hallway." I can look into the others you suggested.

Thanks again!

8

Alex /Red

Reading into this the reason I metioned the Tornwald cyst was that the one I had sat mid line in the back of my throat upper sinuses. I also was partialy blocking my Eustachian tube. It sat right over the TN for the left hand side and probably the right hand side.

IE its central affects both ears and causes tinutus and affects the TN on both sides.

a Tornwaldt cyst is hard to pick up on imaging until it gets quite large.

Now Im not saying that this is the cause of what you are going through but a cyst type issue back there somewhere in the grey matter may play havoc with all types of things (As i found out the hard way with all sorts of unfortunate drooling and sluring of words etc)

IE if you have ATN and someting is back there and happens to get inflamed etc and is sitting on both the left and right TN then it could account for what you are going through.

But there again scans should be picking this stuff up. The cyst I had did increase in size very quickly though.

Good luck with it mate!