My epic eagle journey

I’ve been reading these forums for awhile, and I finally have the courage to tell my own story. It’s a little long, so bear with me.

Like many people on these forums, I have had a long and complicated medical journey, replete with bizarre conditions and neurological problems that no one has ever been able to explain. In fact I have always said that “weird stuff happens to me”. In my mid-20s, I began experiencing random bouts of gastroparesis (although I didn’t know the name for it). Since it wasn’t constant, I just chalked it up to digestive issues, which were becoming worse and worse. I was too young to research it and advocate for myself, I now know it is a classic sign of Vagus nerve dysfunction/compression. In my mid-30s my gall bladder stopped working. It wasn’t diseased, I had no gallstones, it just didn’t work (dyskinesia they call it, I eventually had it removed in my late 30s). Also another classic sign of nerve dysfunction, but no one ever said anything.

About 5 years ago I started having cognitive problems – brain fog, trouble concentrating, memory problems, and problems with executive functioning. My children were still home at the time and would get incredibly frustrated with me – I could not remember anything. When they would tell me something I started having them write it down because I just couldn’t remember.

Then the heart palpitations started – I would have random bouts of tachycardia/racing heartrate when lying down (every doc tried to tell me it was anxiety…but I had no anxiety whatsoever, it was just my heart fluttering and beating fast). In 2017, I went to the ER with severe esophageal pain – I was beginning to have random attacks of esophageal spasms and it was excruciating. I was given a shot of Zofran (anti-nausea medicine), within 2 minutes I went into V-fib and coded right there in the ER from drug-induced long QT syndrome (basically an arrhythmia). After getting my chest pounded and shocked a couple of times - I was tested and everything came back fine. There is nothing wrong with my heart – in fact the cardiologist said I had the heart of a 17 year old (I am an endurance cyclist and at the time I was training for a 100 mile bicycle race - Two days before I “died”, I had ridden 80 miles in 100 deg heat in Oklahoma). Like I said, weird stuff happens to me…I can’t make this stuff up.

Then the vision problems started, I noticed snow-like disturbances and after-images. Then in 2018 I got out of my car one morning at work and suddenly couldn’t walk. My right side was weak and partially paralyzed, I couldn’t talk or see out of my right eye. Everyone thought I was having a stroke and I was taken by ambulance to the big stroke hospital in the state. All CT’s and MRIs came back clear – the neurologist was incredibly rude and condescending. He diagnosed me with convergence disorder – it was basically in my head and in my chart he wrote that I had severe psychological problems. I was so frustrated that I never went to another neuro again until this past year – even though I would have random “attacks” of hemiparesis a few times a year. I just chalked it up as crazy migraines (weird things happen to me after all).

The visual disturbances continued, and the cognitive issues just kept getting worse. I started having headaches with a lot of pressure, I felt like my eyes were going to pop out of my head. Then in all hit the fan in 2020 when I had a bicycle accident. I went down very hard over a set of railroad tracks going about 20mph, I landed on my left side, separated my left shoulder, got a mild concussion and a mild case of whiplash. It rapidly went downhill from there. Within a couple of months I was basically disabled: headaches and pressure, pulsatile tinnitus, heart arrhythmias and chest pain, my body just started going haywire. Over the next year I had to take leave from work and couldn’t function: I bounced from specialist to specialist with a bizarre set of nonspecific symptoms. I was diagnosed with rheumatoid arthritis, then Lupus, then they were convinced I had MS (which was terrifying), then we were back to rheumatoid arthritis. It has been exhausting, I have almost lost my job and my relationship, and have been on the brink of suicide several times. And all of that time – no one thought to look at my neck.

Being the nerd that I am (I’m an engineering professor), I poured through every MRI and CT that I had. I researched day and night, chasing dead end after dead end (this drove my S.O. completely nuts). Then one day I noticed it – one of my post-contrast MRI’s showed a significant “flattening” of what I knew were my internal jugular veins. I wondered if they were supposed to look like pancakes, I mean, none of the radiology reports said anything about it. I began researching what might cause that and my whole world changed. I read article after article about extrinsic compression of the internal jugular vein and its symptoms – they might as well have been talking about me! After learning about elongated styloid processes, I poured over my CT scans, and sure enough! Both styloid processes are about 35mm and are directly compressing the carotid sheath against my C1 process. (The bicycle accident and whiplash most likely caused cervical instability that made the compression worse.) I have had symptoms of carotid artery compression, jugular compression and vagus nerve compression. Looking back through pictures, I noticed a significant change in the the way I looked over the last couple of years - my face is red and I noticed that my left eyelid would tend to droop a little.

I have made some ergonomic changes that have made life bearable, and allowed me to work full-time again. I just don’t look down - I have to keep my head in a very neutral, upright position. Anytime spent looking down causes a wave of nausea, dizziness, headache, head pressure, heart palpitations, brain fog and the visual disturbances and tinnitus get significantly worse. I finally got in to see an ENT - I went “armed” – and was so nervous. I tried to be incredibly polite and humble. I brought my laptop with the appropriate views of my MRIs and CTs up. She had never seen eagle syndrome personally, and had not heard of the jugular variant. She was actually wonderful and said that she would consult with her colleagues. I sent her two journal articles and she actually read them, talked with the others in her practice, and finally came back that it was a plausible theory and diagnosed me (tentatively) with the jugular variant of Eagle syndrome. They referred me to the ONLY doctor in the state that treats Eagle syndrome (Dr. Greg Krempl in Oklahoma City)

I am so relieved, and yet so angry. I have spent so many years trying to get someone to listen to me - I knew something was wrong and I was dismissed over and over. Even my family were to the point of diagnosing me as a hypochondriac and that it was all psychosomatic. To know that this is a real thing, and that there is an actual treatment fills me with a sense of relief that only others who have gone through the same thing can understand.

I see Dr. Krempl on Dec 20th, and am incredibly nervous as my experience with specialist has not been the best. I know this has been a long ramble, so thanks for reading through, this is the first time I’ve put all of this together and written it down. If anyone has had experience with this Dr. (he’s on the list posted on this site), please let me know!

9 Likes

Hi Rather_b_hiking!

Welcome & thank you for your detailed story. What a horrendous journey you’ve been on, but you have a confirmed diagnosis now. Seeing Dr. Krempl in Dec. is an excellent next step, but I’m not sure he does ES surgery based on what our member @Hadassa posted in 2018. However, things could have changed since then. New Eagles Diagnosis in Detroit - #7 by Hadassa

Since you have serious vascular symptoms, the doctor you should see for surgery is Dr. Hepworth in Denver, CO. He is the VES specialist on our doctors list. The wait time to see him is long, but his front ofc staff does keep a cancellation list, & several of our members have had their surgeries bumped to significantly closer dates than originally scheduled. That is to say, if you decide you want to see him, make an appt. ASAP.

I have to say that your fitness level is likely what’s kept you so strong through all of this. I was a long distance runner when my ES was diagnosed, & I believe that was helpful in getting me through my ES surgeries.

It’s also interesting to note that we have another member who made the same comment about needing to hold her head in neutral to avoid debilitating symptoms. The head position that causes jugular compression can vary significantly from one person to another so having two of you note the same thing on the same day is such a coincident.

BTW - I had a cycling accident between my ES surgeries w/ a head injury. It left me w/ Meniere’s Disease/hearing loss in my left ear. I can sympathize w/ the damage yours did to you. That doggone pavement needs to be more forgiving!!

Please know that we’re here for you & totally understand how diverse & destructive ES symptoms can be. You’ll get compassion & support from our members, I promise. :hugs:

4 Likes

@Rather_b_hiking wow your journey is bit similar to mine but I could not have it together the story as concise and eloquently as you did. I am glad that your persistence and patience has paid off. You are indeed now vindicated by your research.

Given your symptoms, it appears that you either have severely compressed IJV on both sides or if you have dominant IJV, you have your dominant IJV severely compressed. This is my assumption in the absence of any imaging evidence but based on my experience, if you have hemiparesis along with typical IIH symptoms, your upper spinal cord might be affected by venous hypertension resulting from dilated collaterals along the vertebral venous plexus.

Your journey to getting proper care begins now and I would not only rely on ENT. Get neurovascular or interventional neuro-radiologist to do Catheter angiogram/Venogram to proper assess your carotid & jugular vein blood flow. After removing the Styloid, you might need endovascular procedure for ballooning the jugulars if they don’t open up. This won’t be done by ENT. Also you also need your carotid artery blood flow assessed as the compression of the carotid itself can potentially cause arrhythmias since carotid has baroreceptor sinus which influence the autonomic nervous system and increase or decrease heart rates much like vagus nerve. So if the Styloid is messing with it, it could induce heart palpitations.

Anyways I am glad that you are finally getting the help you need. I wish you the best in your upcoming ENT appointment.

3 Likes

Well done you for not giving up, for persevering to get a diagnosis & to proving all those who disbelieved you that you do have a genuine condition! And for finding techniques to make your life bearable…
I used to love cycling too & was building up to do a longer charity ride, but the neck positioning caused a prolapsed disc, & I believe this possibly altered the styloids enough to compress my IJVs starting vascular symptoms, never as bad as yours although I was starting to feel really grim before my first surgery. Having the worst side done helped those symptoms enormously and quickly after surgery, but that doesn’t always happen- if there’s been compression for quite a while sometimes the veins don’t spring back, so might need ballooning or stenting. So be prepared for that, it may be that you need someone with even more experience than Dr Krempl as @Isaiah_40_31 says, and consider getting your name on Dr Hepworth’s list in the mean time.
Best wishes!

2 Likes

@KoolDude - Thank you for filling in the finer details of what’s needed for a full blood flow assessment & potential vascular follow-up after ES surgery as well as possible vascular causes for symptoms. I really appreciate your input. I have a hard time keeping track of those types of details since I didn’t have vascular ES. Your research & experience have paid off very well.

3 Likes

Thanks so much @Isaiah_40_31 , I will call and set up setting with Dr. Hepworth today - I plan on keeping my appointment with Krempl, as there’s really no harm in it. I appreciate the insight and guidance, as I have felt very alone in wading through all of this.

The neutral head position has been a life saver, the interesting thing is that it is definitely the worst looking down - but it also happens looking up (probably pushing my C1 forward) or turning my head to the side. So I’m sort of stuck in a military stance lol.

2 Likes

thanks so much @KoolDude, I have attached a couple of images from MRI’s - I’m curious what you think - I really haven’t had anyone look beyond the radiology reports (except for that one ENT) I have noticed enlarged collateral (I think) at the C1 level on the left side. And I had never thought about cord compression causing the hemiparesis.

sagittal T1 post-contrast, 5mm consecutive slices on left side:

Axial Vibe fat-suppressed - 1.5mm slices

1 Like

thanks @Jules - I have wondered if the thousands of miles I’ve spent in that cycling position has contributed to the problem…I’m hoping that the surgery does it and I don’t have to have any stents - but this has been going on so long I’m kinda worried it’s done permanent damage to the ijv

2 Likes

@Rather_b_hiking you must be really good at reading MRIs if you can spot the styloid/c1 compressions on MRI images because MRIs are good at showing soft tissues but hard to decipher bones in the images. It takes a knowledge of the anatomy to understand & locate bones in MRIs. So my conclusion on these limited images are, that your left Jugular Vein (Blue arrow) is being severely compressed by Styloid (Cyan arrow) and C1 (Red arrow). Styloid compresses it first starting with the rightmost image and then as you move from right to left in the images below, you see C1 compression coming into play. Your right Jugular Vein is also compressed by C1 primarily and the styloid to some extent but does not appear to be as bad as the left one. You can also see that collateral dilation (Yellow arrow) is worse on the left side compared to the right indicating impaired Jugular veins. I can also see the venous plexus (Orange Arrows) at the skull base being dilated on the left more as well. I could be wrong as I did not examine the whole MRI in this case but you do have the tale tale sign of Jugular Vein Compressions by Styloid & C1. CT scan would definitely show the bony compression better.

Do you feel the symptoms more on the left side ?

Styloid (cyan arrow - appears black on MRI), C1 (Red arrow), Jugular Vein (Blue arrow), Collaterals (Yellow arrow), Suboccipital Cavernous Sinus (SOCs) (Orange Arrows) and Vertebral Artery (Green Arrows).

As far as the Sagittal images go, I can see the C1 (Red arrow) and the Jugular Vein (Blue arrow) narrowing better. I also see the dilated collaterals (Yellow arrow) in the second image but because this is an MRI, the interaction between blood vessels and the bones are not very clear. So can’t much more than that

As far as spinal cord involvement is concerned, not many literature exists but few hypothesize venous hypertension/compression as cause of hemiparesis/quadraparesis. See this article (Dilated Collaterals Cause Progressive Weakness on All Limbs as result of Bilateral Jugular Vein Occlusion - Interesting IJV Bypass Treatment)

Also in this image you can see the dilated Internal Vertebral Venous Plexus (IVVP) (Red circles) on the left side more than the right side. This could be, in theory, causing venous hypertension at the upper cord or exerting pressure on the spinal dura. Do you feel body tightness or weaknesses when the IIH symptoms are on steriod? I do from time to time.

2 Likes

I have vascular eagle syndrome.

I’m sorry for that @HannahM. I hope you’re able to travel to one of the surgeons on our list who takes care of vascular ES. :heart:

Hello,
When I got to your 6th paragraph, I think it was that, I said that’s me. Hell it took me 2 years, 13 doctors, and this wonderful web site to figure this out. I’m approaching 6 months. Much better, but have a few relapses every once in awhile. You are on the right track keep thinking forward. Force yourself to do thinks, no pity party as my daughter says.
This forum and will help you out. Good luck!

4 Likes

That’s do things, hobbies, activities. Regular hard cardio, get those endorphins up.

2 Likes

Thank you for the great kudos to our forum @NH48! It was here for me as well, & I got answers to many unanswered questions & support when I needed it. That’s why I’m still here. :blush:

3 Likes

@Rather_b_hiking So sorry to hear what a long awful experience this has been for you. I wondered if you’ve been assessed for connective tissue disorders or ehlers danlos syndrome because people who have those seem to suffer a wide and confusing array of symptoms. It seems likely you’ve already covered that, you seem to have a thorough approach, but thought I’d mention it.

Please do let us know how you get on. I agree with others here that it would be worth seeking out a specialist who is well versed in eagles and vascular eagles, though as you say it can’t hurt to keep your current appointment too.

1 Like

thanks @NH48 !

For quite awhile I did kinda flounder - laid on the couch a lot (which actually made it worse because my neck would be in such a bad position. Then finally something snaps inside you, you know? Enough, I’m done, I have to get back on the bike/go to the gym. And no doctor is going to figure this out - they don’t have the time or energy for a complex diagnosis and can’t seem to see outside of their little area of specialty. I just reached the point of realizing I had to take matters into my own hands - as you did!

2 Likes

@IJVCompression - Thanks for the response, I have had extensive workups for autoimmune/connective tissue disorders. All bloodwork has been perfect - no C-reactive protein, rheumatoid factor, ANA negative, the whole 9 yards. I was diagnosed by a rheumatologist with RA based on my hand Xrays - it does run in the family so I wasn’t too surprised. We chased that rabbit for a good 18 months until it became apparent that my symptoms are not those that would be caused by RA.

(edit - it is in the realm of possibility that it could be RA, but it is highly improbable according to a second rheumatologist opinion, and he did not believe it was RA at all. So I don’t know at this point)

2 Likes

@KoolDude - Thanks so much! you’ve pretty much confirmed what I was thinking - but I didn’t catch the dilated internal venous plexus and sub-occipital cavernous sinus. Thanks for pointing that out and for the articles as well. I appreciate you looking at it so thoroughly. I do have CT’s, but just of my head. I do think there’s a lot of C1 involvement due to instability, based on how much it got worse after that accident. I have attached a couple of shots of CT’s, you can see the styloid process pinching off the left jugular against the C1. As you said, the right isn’t as bad. The CT doesn’t go down far enough to see the whole length of the styloids - unfortunately. From the axial window, I counted how many frames you can see the styloids in and multiplied by the slice thickness - it came out to be between 32-35 depending on what slice you started the “count”. (edit - these are soft tissue windows rather than bone - I found it easier to see the vascular structures with this view).



And it took a long time and a lot of reading to be able to read MRIs. If you had asked me 2 yrs ago I wouldn’t have been able to tell you what hyperintense signals or flow voids are!

2 Likes

@Isaiah_40_31 - you know those bicycle accidents can be pretty violent, it took awhile for me to be able to ride the bike again, I think I had some PTSD! And concussions really suck… I also have severe tinnitus in my left ear and about a 50% hearing loss - I don’t know if it’s from the concussion or from the condition…

1 Like

@Rather_b_hiking I can clearly see the severe compression on the left side with the CT scans you posted. I believe they are non-contrast head CT. I think years of extensive academic studies (takes many years of studying & teaching to become a professor) has helped you with your research & knowledge acquisition skills in learning how to read MRIs without formal training. Most of us here had to play armature radiologist since we have been let down by the medical establishment. I really do not give blind trust to doctors anymore without proper verification of their diagnoses.

I am glad you are now on the right path to address it and removing Styloid alone might open up the left vein a bit to reduce the IIH symptoms.

BTW, I have a bilateral sensorineural hearing loss (SNHL). Hearing loss is the fourth or fifth (can’t remember the rank well) most common symptom if you have bilateral IJVS (which you do). So it is highly likely that your left side hearing loss is also related to vascular ES (No-one knows the underlying mechanism but it is thought to stem from blood flow disturbances via venous congestion from the studies I read).

3 Likes