Hi everyone,
This forum is a godsend.
I have the novel jugular variant of Eagle’s syndrome. It seems though that the dust hasn’t fully settled on what to call this condition. A few other names I’ve picked up on in the literature are “Styloid Jugular Nutcracker”, which is fun to say to people and seems to be preferred in Italy, “Styloidogenic Cervical Spondylotic Internal Jugular Venous Compression Syndrome”, which is harder to say to people, and “(Moderate Cervical) Cerebral Venous Congestion”, which seems to be bringing together a few disparate teams—research looks to be progressing with a newly developed rat model! It seems to me that “Eagle Syndrome” best retains the potential cranial nerve involvement in the disease, but I’m not a nosologist.
[2021 November, CT-E; Volume reconstruction using Aliza MS on Linux]
(Edit: Here are a couple gifs with reduced resolution so as not to overload your servers)
[A screencast of some panning between the two sides in Aliza]
[A lightly annotated scroll through the same CT series with Weasis]
The most authoritative record I have yet for this diagnosis, albeit scant, is from a neuroradiologist’s report on February 8, 2024, following an MR angiogram. The MRA (with venous phase) was done to rule out vertebral artery dissection pursuant to a CTA for evaluating extent of cervical spine degeneration, which itself was following up on a doppler ultrasound for vertebral artery stenosis that instead indicated vertebral artery occlusion. The neuroradiologist’s report states that my “right jugular vein demonstrates focal stenosis just inferior to the skull base.”
This is in some tension against an ER consult with a neuroradiology fellow a month earlier on January 6, 2024 that “did not think that there is any significant stenosis in the jugular vein and no other findings suggesting raised intracranial hypertension.” The neurosurgeon, content to concur with the neuroradiology fellow on what is and is not “significant stenosis”—with nary a glance at the dozen or so studies I brought him—concludes on February 22, 2024 that “the MRI has been reported normal. There has [sic] no signs of venous hypertension.”
He was appreciably late to the appointment coming from an earlier surgery. I got the sense from his distinctly serene—almost glowing—countenance that the earlier surgery went well, perhaps after some hiccup. After he took a beat or two to silently review my file, I started the appointment by characterizing the stack of whitepapers I brought him as all seeming to say that my condition is underestimated. He countered that the authors are probably just trying to get published. So rebuffed on a perusal of any single one of the papers I had, I quickly tried to rebut the assertion that my jugular stenosis was insignificant by flashing him two images I had printed out, one of a spiral-shaped collateral vein just above my hyoid, and another of some subtle periventricular white-matter hyperintensities. He cautiously acknowledged my research efforts and clarified that he’s not denying the reality of my symptoms. He cordially invited me to follow-up for dynamic DSA from advice elsewhere and closed the door on surgery from himself. “We’re not the ones that do this surgery,” he said. Seeming to expect recommendation for stenting by the referring vascular surgeon, he rather vaguely seemed to advise against it. In retrospect, it seems he was not aware of styloidectomy as a desirable course. I probably could have brought it up then and there, but what with the risks of the procedure, I want to speak to someone who knows more than me about the condition, and have them bring it up independently.
For background, I grew up in Toronto, finished university in 2008, worked for a while, sojourned in Korea from 2015 to February 2023, and am now back in Toronto… sojourning still.
My sense of well-being began deteriorating in 2016. After a particularly obsessive stint at my keyboard, I found myself with a sort of nagging back/shoulder irritation that wasn’t really going away. I had the bright idea of stretching my muscles, knowing exactly nothing about anatomy then, and being accustomed to being an invincible 20-something, despite my 30th birthday having passed. I found a stretch that seemed to alleviate the irritation—head-and-neck forward-left flexion. Ambitious and naive, I held the position for many many minutes, very slowly and occasionally intensifying the stretch. It felt pretty good the whole time, until it very suddenly—with a loud pop—did not. I was bed-ridden for 3-4 days, though able to get up for food and toilet. I don’t think I’ll ever be certain, but I believe I may have dissected my right vertebral artery right then, with reconstitutive remodeling ever since.
I think just the CT scans I’ve had over the years sketch out the rest of the story. I’ve had five CT scans of my head. First in 2010 through psychiatry at NYGH (CT1), one through neurology in 2019 in Korea (CT2), two in 2021 through otolaryngology (CT3/4), and one more in 2023 through neurosurgery at Sunnybrook after I came back to Toronto (CT5).
The three scans in Korea, CT2-4, were kind of frivolous in retrospect. They just offered to do one the same day without really challenging me on why I was there. This is, of course, really nice if you know what you’re doing, but I certainly did not. They were each around $400, and consultations were around $40 each. At the time, I had next to no understanding of how healthcare works, nor what a primary care physician is, so I cut my teeth on trying to figure things out myself. This was very difficult to do alone and that with the very symptoms being investigated. But I had material support from my family to lean on, so I had nothing but time and nothing better I could do. The learning process was all very slow, very sporadic, and very disorganized. Gratifying though… I’ve since gathered a pretty robust ‘tool chain’ of essential textbooks, websites, and software to answer questions for myself, though not without being simultaneously very certain and very wrong plenty of times. I like to think of being medically wrong as a kind of rite of passage, so long as it doesn’t harm anyone else.
My Core Tool Chain for the Medical Amateur
Excellent and Breezy Introductions
- Overview: Ch4’s Anatomy for Beginners with Dr. Gunther von Hagens
- A graphic but very visceral and riveting introduction in just four fifty-minute dissections. I’d advise a more passive watch at first, revisiting later for the minutiae that whiz by.
- A&P101: The Way We Work by David Macaulay
- A severely underrated gem. Just the first chapter on cells is worth seeking this book out at your local library. The author covers the material for an entire standard A&P course, but has put a great deal of thought into what to omit and what to include so as to make as gentle an introduction as possible. The style of the illustrations—very impressive and original, mind you—tends to give the impression that the book is for children, but it’s not.
Robust Reference Texts
- A&P Reference: Human Anatomy & Physiology by Marieb & Hoehn
- Any standard A&P book would do. I’ve yet to summon the energy to work through much of it, but it’s handy whenever I want to get a rigorous gist of a specific bodily process as a starting point for further investigation.
- Regional Anatomy Atlas: Atlas of Anatomy from Thieme
- I started with Netter’s Atlas of Human Anatomy, but the one from Thieme (atleast in the 2020 edition by Gilroy) is very… ‘clean’ and the progression of illustrations is a little more predictable.
- Sectional Anatomy Atlas: Pocket Atlas of Sectional Anatomy from Thieme
- This seems to be the rare sectional anatomy atlas that shows and actually annotates each of the slices in a full series. The thoracic series is confusing for the parascapular muscles though, because the arms are raised. The authors have another sectional anatomy atlas for ‘The Musculoskeletal System’ that basically doubles the slices in the arms and legs, and is a bit better for the parascapular muscles.
- Anatomy Reference: Gray’s Anatomy
- The writing’s very dense, but the payoff for patience is always there. When I start to have more detailed questions about a particular body part, I end up here as a launching pad for further investigation.
- Muscle Pain Analysis: Travell’s Myofascial Pain And Dysfunction
- This has been like my bible ever since I learned about myofascial trigger points. I refer back to it when I encounter some new pain or irritation for a differential that doctors often don’t know about. I’ve collated the pain referral patterns, printed them out, and have them taped to my wall, because who wants to flip through a book or a screen when you’ve got a muscle that’s bothering you?
- Any Dictionary of Medical Terms
- For a painless, private search
Websites
- PubMed, Google Scholar, et. al.
- There’s certainly a learning curve with parsing through any dense research paper, but going past just the abstracts, the introduction and discussion sections are often worth working through for accessible and invaluable background.
- Radiopaedia.org
- It’s for professionals, and I found it a bit disorienting for the longest time, but it’s got case examples when you want to check your own images.
Software
- Weasis
- Again, it took me the longest time to learn to do anything beyond just opening and scrolling through an imaging series, but now that I can appreciate it a little more, it’s got a pretty solid feature set. No volume reconstructions though.
- Aliza MS
- I think Windows probably has a better offering for radiology software generally. This seems to be a rare Linux offering for volume reconstructions.
Just Some Casual Reading
- Found a book called “How Doctors Think” whose Introduction and Epilogue were insightful enough for me to let sit on my shortlist for a couple months now
- Also, the title and backcover blurb for “The Rise & Fall of Modern Medicine” caught my eye enough to let sit on my shortlist for a couple months now too
CT5 was the aforementioned angiogram, ie with contrast, and includes the neck. This was pursuant to the suggestion I made to my referring family doctor for a diagnosis of vertebral artery stenosis. I was convinced of the diagnosis after poring over a sparse MRI of just my cervical intervertebral discs for a few years. The MRI was taken at an orthopedic clinic in Korea in 2019. It cost around $200—apparently a promotional offer.
CT3 was to check on my paranasal sinuses. I went back a few months later and asked about my parotid glands, and so CT4 was done to check for a “parotid mass” with a contrast agent that incidentally lit up the cervical arteries and veins very clearly and also included my neck. After CT4, and after marinating a bit in the imaging results, I went back to the ENT and actually suggested Eagle’s Syndrome at one point. The latest edition of Dr. Janet G. Travell’s “Myofascial Pain and Dysfunction” has Eagle’s Syndrome as a differential for pain/dysfunction involving the anterior musculature of the neck. However, I was pointing to the styloid process on his screen and calling it the stylohyoid ligament and pointing to the sheath of the styloid process and calling that the styloid process. Annoyed, he corrected me in belittling tone essentially pointing out that what I was calling the stylohyoid ligament was in fact the styloid process. Unsure of myself, the conversation moved on from there. I think seeing that what I had initially correctly took to be the styloid process was roughly under 30 mm, I had fooled myself into thinking that rather than having an elongated styloid process, I had a calcified stylohyoid ligament. (Though based on the sudden angulation in the middle of the styloid, I may not have been so wrong.)
Eliminating both above-standard styloid process length and calcified stylohyoid ligament as possiblities, I moved on from Eagle Syndrome as a possible diagnosis. Only then, after continuing to haphazardly look for any asymmetries in my images and spotting some in what I would then learn to be my vertebral arteries, did I start to care about what blood vessels looked like in sectional imaging.
CT2 was the most frivolous scan. I went into a hospital panicked and sleepless after noticing a bony bump on my head asking if it was some kind of bone cancer. Months later I would remember having bumped into the corner of a cupboard door not long before the scan.
CT1 was done during an involuntary admission and so I was not even aware of its existence. It was done alongside an MRI of the same region. I retrieved it from NYGH just a couple days after CT5 expecting just an MRI, and was pleasantly surprised to see it. Though I was skeptical about whether these were worth retrieving, a couple months later in early January, when I first found out about Eagle Jugular Syndrome, I was able to see that my internal jugular veins were stenosed with expanded suboccipital collaterals even then!
After reading the opinion from the neurosurgeon on February 22, and expecting little more from him, I decided to go on the page for idiopathic intracranial hypertension on radiopaedia.org and challenge myself by going through each of the radiological indications and seeing which ones I can find. There was also a 2024 paper comparing IIH Internal Jugular Vein Stenosis indications on CT and MRI that had a checklist of sorts.
February 2024 IJVS Radiology Paper
Last paragraph of section 2.3 Imaging Analysis:
As we aimed to compare IJ appearance using CT and MRI, our analysis was also conducted on a per-vein basis, involving a total of 70 veins. For further assessment of cerebral venous outflow, jugular vein dominance, styloid process enlargement/stylohyoid calcification/ossification, venous collaterals, condylar emissary vein size, and signs of intracranial hypertension (empty sella turcica, optic nerve tortuosity, transverse sinus stenosis) or confluent white matter hyperintensities were assessed. Causes of the stenosis on CT (compression by the digastric muscle, styloid process compression, stylohyoid ligament, lateral C1 vertebra) were also identified. IJ dominance was assessed by visual comparison of vessel calibers. Styloid process enlargement or elongation, and stylohyoid ligament calcification were recorded in a binary fashion. Condylar and mastoid emissary veins were assessed by measuring the largest intraosseous diameter on the axial source images.
I found no obvious indications after examining the optic nerves for tortuosity.
I found that CT1, CT2 and CT5 together appear to show a progressively emptying sella turcica (from <33% to >50% over 14 years, MRI from same time shows more clearly than CT1).
[Progressive concavity of superior aspect of pituitary gland]
The report accompanying CT2 asserts “mega cisterna magnus”, and I found CT1 and CT5 to have the same, notably with asymmetrical ventricles, especially the lateral posterior horns. I read somewhere that this is a little more indicative of intracranial hypertension (or was it hydrocephalus?) than, say, either of these two phenomena alone, which I think is usually dismissed as a congenital formation or some such.
[“Brain holes”]
Also, as mentioned above, I was able to opacify some very subtle non-focal white-matter hyperintensities supero-postero-laterally about the lateral ventricles. They appear to be more pronounced on the left side, which is not the very dominant, more stenosed, and symptomatic side. Can’t say for sure that this isn’t just artifactual… There were at least a couple papers from Beijing [TODO] that talk about “cloudy white-matter hyperintensities”, but the examples given were pretty subtle and my scans weren’t done with the same protocols.
[WMH?]
I have an appointment for a second opinion with another neurosurgeon on April 12, 2024 downtown at St. Michael’s. I’ll be raising these with him. He appears to have co-authored atleast two papers in relation to this topic.
In parallel, I’ve requested referrals from my family doc for an otolaryngologist consult for “Winged Jugular Eagle’s Syndrome”, and another for a nerve conduction study of my accessory nerve and C1/C2 spinal nerves. Feeling robbed of my best years and homelessness nearing every month, I’m wanting to find a path to surgery before summer ends. Reading through the Canada threads, it looks like I’d have to cave for a surgery in the US.
I’ve since compiled a “Patient Opinion” sheet that I take with me to appointments now (see below).
Also, I’d be more than happy to send DICOM sets to anyone interested in taking a look if you send me a message. Likewise, I’d be more than interested in providing annotated and citation-heavy comments (being a total amateur) on your DICOM sets if you have them. Please note that I’ll be very slow to respond as my time on the keyboard is very limited by an extreme rest-to-work ratio, but I will respond.
If anyone in Toronto reading this has or has had this condition (or any of the other variants), let’s have a chat.
Patient Opinion
Diagnosis:
- (Trauma-Exacerbated Quasi-Hydrocephalic (De Bonis, 2019)) Winged (Mitchell, 2023) Jugular Eagle’s Syndrome (Siniscalchi, 2023)
- aka Styloid Jugular Nutcracker (qv. Angelini, 2023)
- aka Styloidogenic Cervical Spondylotic Internal Jugular Venous Compression Syndrome (qv. Scerrati, 2021)
- aka Moderate Cervical Cerebral Venous Congestion (qv. Arun, 2021)
Desired Course:
- Jugular Decompression by styloid process resection or excision, and/or atlas excision, without stenting
Worthwhile Tests:
- DUS jugular stenosis degree
- Absolute-value intracranial pressure US, with adequate precision (< 2 mmHg?)
- eg. “Two-depth high-resolution transcranial Doppler insonation of ophthalmic artery” (qv. Ragauskas, 2012, “Clinical assessment of noninvasive intracranial pressure absolute value measurement method”)
- D-dimer & WBC tests for IJV thrombosis
- EMG of trapezius, sternocleidomastoid (CN XI), and upper cervical muscles (C1, C2)
Required Reading
Bare Minimum:
Siniscalchi, et al. Is Eagle Jugular Syndrome an Underestimated Potentially Life-Threatening Disease? The Canadian Journal of Neurological Sciences, 2020
(Canada, 1-page)
Angelini et al. Neurosurgical implications of the Jugular Vein Nutcracker. 2023
(Short, CSF theory)
Scerrati et al. Styloidogenic-cervical spondylotic internal jugular venous compression, a vascular disease related to several clinical neurological manifestations: diagnosis and treatment—a comprehensive literature review. 2021
(Specific condition)
Mitchell EC, Yoo J, Ohorodnyk P, et al. “Winged” Eagle’s syndrome: neurophysiological findings in a rare cause of spinal accessory nerve palsy. Illustrative cases. J Neurosurg Case Lessons 6(24): CASE23358, 2023
(Ontario, surgical, specific)
Wider View:
Arun et al. A proposed framework for cerebral venous congestion. The Neuroradiology Journal, 2021
(General framework)
De Bonis P, et al. JEDI (jugular entrapment, dilated ventricles, intracranial hypertension) syndrome: a new clinical entity? A case report. Acta Neurochir (Wien). 2019;161:1367-70
(Hydrocephalus connection)
…etc.
Corroborative Notes
Right vertebral artery:
- Lesions along V2 with venous offshoots directly joining thoracic outlet (qv. 2021-CTE) (post-dissection remodeling?)
- Bulging along V3 (pseudo-aneurysm?)
- Tapering along V4 (hypoplasia; cf. 2010-MRI)
Cranial:
- Partial empty sella turcica (Grade 3/5, unilateral, anterior, discernible hypophyseal cleft) (Grade 2/5 in 2010 MRI)
- 2010-MRI, 2019-CT, 2023-CT/MRI: Unusually premature progressive emptying
- Mega cisterna magnus with asymmetrical ventricles, esp. lateral posterior horns (also in 2010 MRI)
- Subtle white-matter hyperintensities superoposterolaterally around lateral ventricles (also in 2010 MRI)
- Expanded lateral sulcus of temporal lobes (also in 2010 MRI)
- Hyperpneumatization of temporal bones (also in 2010 MRI)
Venous outflow:
- Collateral venous expansion adjacent to jugular stenosis (suboccipital, pterygoid)
- 2021 CTE: flat spiral venous anomaly near proximal anterior jugular vein on right side
- 2010 MRI: jugular narrowing with suboccipital collateral venous expansion
Osteal:
- Elongated styloid process, bilateral (>30 mm) (cf. 2010 CT/MRI, 2021-CTE: 26/28mm, 2023-CTA: 31/32mm)
- Osseous lesion along dorsal raphe of ligamentum nuchae at level of C6 (2021-CTE: ~8mm) (2023-CTA: ~10mm)
- Osseous lesions along throat and trachea… (cf. 2021-CTE)
- Asymmetrical atlas, left-dominant lateral mass
- Osseous cutaneous lesion near right external ear
- Anterior to intersection of helical crus and tragus (~5mm, also in 2010)
Notable Signs, Symptoms, and History:
- Persistent sleep disturbance; distinct cognitive decline; debilitating fatigue; mRS=2
- Frequent chest discomfort; multiple episodes of acute progressive dyspnea, twice on a plane (IJV PE?)
- Frequent pulsatile tinnitus; usually wavers between rhythmic, arrhythmic, and silent
- Right scapular winging: lower trapezius atrophy, upper trapezius and sternocleidomastoid hypertrophy (CN XI?)
- Palpable posterior cervical muscular lumps. Eg semispinalis capitis (C1/C2 nerves?)
- Occasional headaches; usually right-sided, occasionally bilateral; ocular, occipital, temporal, non-specific
- Chronic neck discomfort and pains; IVD herniation, forward head posture, mild scoliosis.
- Distinct weight gain, esp. central; past reflux issues (Endocrinological? CN X?)
- Involuntary facial twitches and shoulder abduction when engaged in euphoric body scan meditation (CN VII, XI?)
- Lingually palpable enlargement of right pterygoid humulus
- Prior throat discomfort, mild dysphagia, occasional glossodynia
- Avid tenor sax player: age 13-21; Intensive video editing: age 15-18; Software engineering: age 19+
- Basketball: age 12-15; Exercise antipathy: age 16-30