Astounding Comment from Rheumy

[montebello]

Hi all,

I've been a GCA patient since AUG 22. Yesterday, during a visit with my Rheumy, I expressed the slight concern that I might be harboring an aneurism in the brain because of the recent "zinger-type" headaches that I've had over my right temple. My rheumy told me that these headaches were much more likely to be inflammatory in nature, as it was not possible for the arteries INSIDE my skull to inflamed from my GCA condition and therefore I was not to worry about an aneurism from my GCA condition.

But when he told me that it was not possible for the arteries INSIDE my skull to be inflamed from GCA, I said something like "How could that be? I though the mechanism for GCA-related blindness was that the blood vessels to the optic nerve become INFLAMED, swell, and thus cut off vital blood to the nerve and killing it."

He responded my using highly medical terminology. I told him I didn't understand his terminology, so he merely repeated his terminology. I gave up, thinking that I'd come here for help instead relying on my "highly trained Rheumy" for explanations.

Can anyone help me understand this?

Thanks so much

[DorsetLady PMRGCAuk volunteer]

…as it was not possible for the arteries INSIDE my skull to inflamed from my GCA condition ..

I’ve got news for him… they most certainly do.. I’m living walking proof of that, and with sight loss to prove it due to ophthalmic artery being affected … what a complete and utter plonker!

[montebello in reply to DorsetLady]

Yeah, but interestingly if you Google "is it possible for INTRAcranial arteries to be affected with CGA," the answer I get is "no." There's some kind of nuance here that is interesting.

[DorsetLady PMRGCAuk volunteer in reply to montebello]

Not sure that’s strictly true - but happy to be advised if wrong - this from

Anatomy of the Ophthalmic Artery: A Review concerning Its Modern Surgical and Clinical Applications

Anatomy of ophthalmic artery has been thoroughly studied and reviewed in many anatomical and surgical textbooks and papers. Issues of interest are its intracranial and extracranial course, its branches, its importance for vision, and its interaction with various intracranial pathologies. Improvement of our understanding about pathophysiology of certain diseases like aneurysm formation, central retinal artery occlusion, and retinoblastoma and also invention of new therapeutic modalities like superselective catheterization, intra-arterial fibrinolysis, and intra-arterial chemotherapy necessitate a reappraisal of its anatomy from a clinical point of view. The aim of this review is to examine clinical anatomy of ophthalmic artery and correlate it with new diagnostic and therapeutic applications.

[Hisue]

My temporal arteries were visibly bulging & pulsating out of my head on both sides. That rheumatologist needs to go back to school.

[montebello in reply to Hisue]

But those arteries are OUTSIDE of your skull. He's talking about the ones INSIDE your skull

[Hisue in reply to montebello]

Wouldn't arteries inside the skull be inter-cranial?

[PMRpro Ambassador in reply to Hisue]

They are extracranial - outside the skull and dura the lining around the brain. arteries inside the skull are termed intracranial

"Although they look similar, the prefix intra- means "within" (as in happening within a single thing), while the prefix inter- means "between" (as in happening between two things)."

[Hisue in reply to PMRpro]

Thanks for the explanation. What are inter-cranial arteries?

[PMRpro Ambassador in reply to Hisue]

There aren't intercranial, just extra and intra

[Hisue in reply to PMRpro]

Okay , thanks so much for the answer.

[PMRpro Ambassador]

You're right, he's not. Intracranial inflammation in GCA is very rare - but very rare is not never happens.

onlinelibrary.wiley.com/doi...

I imagine his "it can't happen" is based on the fact that GCA only usually occurs in arteries with a thick elastic component to their wall structure and intracranial arteries don't have this component to the wall. Shame you hadn't asked him to write it down!

Right at the end they say that they believe most cases with intracranial vasculitis have a castastrophic disease course - they die. "A more favourable course in some patients with intracranial GCA who are not diagnosed may escape notice,"

Most cranial GCA symptoms are due to vasculitis in the arteries supplying the brain, before they get inside the dura, the covering of the brain. But it's like the age at which it occurs - how can you delineate that exactly? I doubt you can - and so it seems does Carlo Salvarini.

If Dr Salvarini says it - I'll take his opinion over most general rheumies.

[montebello in reply to PMRpro]

So what would you say is the mechanism for the poor folks who lose their site due to GCA? Are the small blood vessels inside the brain blocked, or are the larger vessels outside the brain (which supply blood to the smaller vessels) blocked?

[PMRpro Ambassador in reply to montebello]

Variable - could be either but probably mostly disease in the vertebral and other extracranial arteries. They can't biopsy them so they can only find it at post mortem - the pathologist knows it all and does it all but they are too late ... Anyone who says it CAN'T happen is being a bit silly - no scientist says that!

[montebello in reply to PMRpro]

So when I have a GCA-related headache, its causes are most likely due to inflammation in the arteries outside of my skull rather than inside my skull. This is somewhat reassuring since I was concerned about a possible aneurysm.

[PMRpro Ambassador in reply to montebello]

An aneurysm is a different entity altogether. Have you had a CT scan? If so, they would have found any signs of that.

[montebello in reply to PMRpro]

Hmmm... he's suggesting that GCA related aneurysms are caused by the inflammatory response of the affected artery, which is very, very rare with arteries inside the skull. From everything you've said and all that ive read, I think he's correct.

So the likelihood of a CGA related aneurysm inside the skull is very small.

No I have not had a CT scan.

[PMRpro Ambassador in reply to montebello]

It is also likely to be a greater problem if the inflammation isn't being managed well over a very long time. But yes - very small. Doesn't mean it doesn't happen.

[Dochaz in reply to montebello]

This thread is fascinating! It's a question I've often asked myself. The mechanism of sight loss intrigues me because I don't understand the link between the temporal arteries and the optic nerve.

[PMRpro Ambassador in reply to Dochaz]

The temporal artery bit is a red herring - it was the artery that was visible when swollen and the only reason it is used is because it is superficial and you can manage without it so if it is chopped out in the biopsy, other arteries nearby take over its function. With ultrasound they can look at the temporal artery, and also the subclavian and brachial arteries - which you can't biopsy.

The sight loss comes from poor arterial blood supply to the optic nerve - it MAY be from a dam a long way upstream, in the carotid or branches of it, but it can also be due to poor blood flow much closer to the nerve itself.

[montebello in reply to Dochaz]

I'll risk asking my rheumy and then share his response. I'm fascinated as well .

[DorsetLady PMRGCAuk volunteer in reply to Dochaz]

There isn’t any as such. This picture shows arteries.. and the darker ones are nearer the surface, whereas the lighter ones [the ophthalmic artery in particular] are deeper and is the one that ultimately supplies blood to the optic nerve. Or not as the case may be!

Arteries of head

[Dochaz in reply to DorsetLady]

So if it's unusual for the intracranial arteries, with less elastic walls, to be affected by GCA, how come the ophthalmic artery, which, if I'm interpreting the image correctly is intracranial, gets blocked? Silly question, maybe, but having had no medical training or background, I'm just trying to understand the mechanics and the logic.

I've also seen on the forum that necrosis of the tongue can happen, and again, that seems to be one of the deeper arteries, albeit not intercranial.

[montebello in reply to Dochaz]

I'm with you, dochaz. The more I look into this, the more confusing it gets.

[DorsetLady PMRGCAuk volunteer in reply to Dochaz]

Me no medical training either -but can assure you the ophthalmic artery (and probably part of the lingual artery) was affected in my case -got the T-shirt to prove it…

[Dochaz in reply to DorsetLady]

I know you lost the sight in one eye to this condition and appreciate the fact that you have been through the mill. Maybe in future years (too late for us 😏) there will have been enough research to enlighten the next generation or two. Makes me contemplate leaving my body to science, though I remain hopeful that the condition will burn itself out before the curtain falls!

[LRevell in reply to Dochaz]

I lost all my peripheral sight in left eye due to GCA. It caused scaring on my optic nerve.

[PMRpro Ambassador in reply to Dochaz]

The occipital artery runs along the outside of the head before smaller branching arteries enter the skull towards the back of the neck. The blockage can be at this stage while it is still extracranial and has an elastic component. If you cut the bark on a main branch on a tree, all the twigs distal to that are affected too. Same idea.

"The occipital artery is a branch of the external carotid artery in the neck. It sweeps an oblique posterosuperior course under the skull base to supply regions of the upper neck, occiput and posterior fossa."

The tongue is supplied from a branch of the carotid, well outside the skull, The arteries you see there on the diagram are all relatively superficial, not inside the brain.

[montebello in reply to PMRpro]

Hello Pro, and thanks for this. But if the occipital artery has blockage outside of the skull, then wouldn't everything "downstream" of the blockage be affected? Everything?

[PMRpro Ambassador in reply to montebello]

Maybe it is. That would also give rise to the occipital headache. Not everything needs as much blood flow as others. The carotid artery can be 70% blocked and still not need surgery, the flow is still enough for the brain to function. But if the flow is partially blocked and then a small clot comes along, it may block the blood flow entirely in one branch, if it is the one supplying the optic nerve and it lasts long enough, the blood supply to the nerve is stopped long enough for part of the nerve to die and that area then tends to spread. Once a tiny area is necrosed, the cells next to it start to die too, my husband used to say there was a critical point - below a certain amount of necrosis it could recover, but there came a tipping point and then it just cascaded over the edge.

There are two sorts of damage to the optic nerve that result in irretrievable damage - longer term reduced flow and that is what causes the optic disc in the retina to swell and become paler as a warning sign, and short term total blockage. It is similar to the difference between angina and a full blown heart attack. In GCA, once that has started there is a finite window during which high dose steroids can reverse the situation where they stop progression and if it is soon enough, the improved blood flow allows no further damage to occur. But once enough damage has been done to cause loss of vision, it is very unlikely it will reverse. Once one eye has lost sight, even with high dose pred there is a 50/50 chance that the other eye will go in the following 2 weeks. DL has said it was the longest 2 weeks of her life.

[montebello in reply to PMRpro]

Absolutely excellent. Thank you.

[montebello in reply to Dochaz]

Here's the message I sent to my rheumy, and then his reply:

My message: "I'm still in awe about the statement you made that the arteries INSIDE the skull are not affected by GCA inflammation. When I asked you how, then, are the arteries supplying blood to the optic nerve blocked, you responded by naming some arteries -- supposedly arteries external to the skull that become blocked and are sort of "upstream" of the optic nerve. Would you please write back and tell me the names of these arteries, or correct me if I misinterpreted you?"

His reply: "These are the ciliary arteries, anterior and posterior."

I'm more confused than ever. According to what I've seen in medical images, these arteries are inside the skull. But when I google "posterior ciliary arteries," it tells me it's located outside the skull. Arggghhh!!

[Grammy80 in reply to Dochaz]

I was undiagnosed for at least nine months while having full-blown symptoms. Sight loss is simply the loss of blood and oxygen flowing to the optic nerve through the ophthalmic artery. Because of the lack of blood and oxygen, the ophthalmic nerve in my left eye died painlessly. It is pale. The optic nerve in my right eye has some 'spots' on it, and I see a neuro-ophthalmologist every 4 months, but my right eye is 20/20, thanks to cataract lens insertion years ago. Do take good care💞

[Grammy80]

That was a wrong answer from the doctor; I experienced the same thing as DL.

If I thought I might have an aneurysm because of this 'different type' of headache, I'd be at a neurologist's office in a heartbeat. I use Google a great deal, but it cannot diagnose you. It sounds to me like you need an MRI. Knowing you are in the States, I would think you could get into the proper specialist. Can your PCP get you a referral to Neurology? I would be disappointed because the rheumy didn't follow up with a referral. Disappointed is too mild a word. Maybe it is time to look for a new one. My best💞

[montebello in reply to Grammy80]

Hi Grammy,

I hope you know that I always appreciate your insights.

But I feel a need to explain how I deal with my physicians. The following comments assume that I trust them, and I do. Although my relationship with my rheumy is the pits (he's very difficult to communicate with), I do think the fellow knows what he's talking about. Having said this, when I go to a physician with a complaint I follow their advice WRT further testing. In this case, I presented all the facts about how I was feeling, and then asked "have I said anything which causes you to think I need further testing?" He said "no." I followed his lead. If he would have said "yes, " I'd have followed his lead. If I'm foolish for handling my physicians like this, so be it but that's how I feel.

All the best to you.

[Grammy80 in reply to montebello]

Hi montebello, No....you are not foolish at all. Each of us has to be true to ourselves to make 'life' work. What works for one of us may cause conflict in another....it is a no judgement zone. My first trip to a hospital in Boston, Massachusetts, was at age 4 and the merry-go-round hasn't stopped....I realize I'm a bit more aggressive. This Grammy didn't mean to come across like a Grizzly~!💞

[Bramble2000]

sounds like my Rheumatologist, very frustrating!

[cycli]

Some medics when confronted fall back on terminology to bolster their supposed superiority but its mostly posterior bovine excrement

[montebello in reply to cycli]

Hilarious! You've made my day