Notable decline, cervical SEDAVF? & medical ping pong in NZ

[NotAKiwi]

Hi,
Sorry if this is a bit long-winded. I am not a Kiwi but I am living in NZ. Before we came back here, in 2019 I had a brain scan as I kept feeling like I was falling slightly to my right. Prior to that I had had odd unilateral parasthesias. Anyway, my brain scan was normal.

We got stuck in NZ due to the lockdowns, which was very upsetting to me - and I’m homesick always. But that’s another issue.

In 2022, after some intense episodes of feeling sudden extreme hunger, weakness, dizziness etc, where I would have to get to bed ASAP and take ages to recover (and felt my right leg slightly giving way en route), I presented to ED for the first time with my right leg ‘tremoring’ (shaking a LOT). These episodes also ended up involving body/arms but predominantly right sided.

After 3rd hospital visit, where I stayed a couple of nights and had another brain MRI (normal), I was sent home (unable to walk much without triggering these violent seizure-looking episodes) with nothing but a very strong medicine and a referral to psychiatry. My friend had to find a wheelchair to get me from room to car - no kind of aids were offered. I later had to call my GP and ask what to do if I couldn’t really walk. I ended up with my first cane.

A few months later, I was declined seeing a neurologist (we don’t have one locally so only the highest priority get accepted) and saw a General Medicine Dr at the hospital who diagnosed me with ‘likely FND’. I knew this was likely and, at the time, accepted it - but was disheartened with the lack of support offered.

Over the years I’ve had periods of big improvement followed by crashes/flares that last months and, I’ve realised, seem to also get a bit worse each time.

I was diagnosed with hypermobile EDS (finally) last year, and POTS and likely MCAS which adds complexity!

I have a permanent right-leg gait issue now. I get intense body weakness (right side predominant), tremors, head pressure, vertigo, nausea, leg giving out, and episodes amongst other things. Nearly 2 years ago I woke up with nerve pain all down my arms and legs and back. After it got better, I still get lots of nerve pain and parasthesia especially in my arms, and especially at night. Thankfully P.E.A has REALLY helped manage that.

This year, after being referred to neurology again last year, I got to see Gen Med again, but first had and MRI of brain AND neck.

The report stated “Prominent paraspinous vasculatures, carotids and vertebral. There is the appearance of arterialized flow extending from the right vertebral artery through the C6-7 foramina on the right side into the ventral epidural space, subsequently exiting through the C5-6 foramina on the right”

They said it was potentially a Spinal Dural AV Fistula, though my hours of research says the description matches a Spinal Epidural AV Fistula. Clearly this, in the neck especially, is pretty rare.

But they then did a Time of Flight MRA of head and neck (COW and neck) in which they couldn’t see any sign of it (they were looking it seems). Unfortunately, this ‘normal’ result seems to have triggered a lowering of priority (and probably leaning on the ‘FND’ diagnosis). Initially I got a letter saying neurology had accepted me and the wait list was about 12 months! Since then I’ve flared and declined again so I am hardly leaving the house.

I have fought and it seems I am getting to see neurology clinic in August, now. I did also try getting my Nurse Practitioner to get my imaging looked at by neurosurgery. I got a charming letter in my patient portal - to the medical practice - saying they wouldn’t be doing anything and that I had a lot of recent imaging for a host of SPURIOUS symptoms. How maddening and upsetting to read this when I’m fighting tooth and nail to claw some life back. I have 3 kids, the youngest is 3, and I hardly get to do life with them!

Anyway, I’ve been on Open Evidence researching like crazy and it seems my MRI findings really do warrant urgent attention - and are very specific, not vague, findings.

I’m here for solidarity, support, suggestions and to be a part of what seems to be a great little corner of the web.

Thanks for reading.

[JD12]

Firstly, welcome! Hopefully our group is able to offer some support and our shared experiences are incredibly diverse. We do see a lot of people struggle with the medical diagnosis and treatment and it is really more difficult the further away from large centres it would often seem. You do have to be your own advocate for sure, particularly in the public health care system.

Is it possible to get your scans and seek an opinion from some of the experts elsewhere? There are some incredible facilities in Europe and the US that offer the second, for a cost however. Just an option to be better prepared for the conversations with your current providers. I was a brain AVM’er and mine showed up when it ruptured, so quite different but we all share a lot of similarities and challenges as well. I imagine some of our folks here will pass along there experiences and suggestions. Take Care, John.

[NotAKiwi]

Hi

Thanks for replying to me guys. It seems, from my own research, that spinal epidural avf’s are rarer than sdavf’s, and cervical ones even more so. I’m impatient now and just want to know.

JD12 you’ve reminded me that I should try to get hold of the images in case of needing to send them elsewhere. I have got my medical practice to reach out to Dr Helge Koeck in Wellington who is a private neurosurgeon. We don’t exactly have money to spare but it feels like my options are tricky, particularly because it seems likely I also have craniocervical instability (CCI) and that, according to some, can also cause some of the issues I’m having. So, which is which? We don’t know.

The seizure like episodes (conscious) are pretty violent and are not exactly helping all the rest of my joint issues and so on. I really could do with a wheelchair, but again, it’s all cost.

I’m so hoping that there are some treatments we can try in order to improve symptoms (&that it’s not too late). It’s hard to fight when you’re exhausted and unwell.

I hope that’s not a totally rambling reply!

[DickD]

Hi @NotAKiwi

Welcome to AVM survivors!

I don’t know if many of our spinal patients have a spinal epidural AVF rather than a SDAVF (which I recognise as more usual for our patients – I’ve seen SDAVF mentioned many times) but the people who will know are the @Spinal team themselves.

Anyone able to offer a hand of friendship to our Brit in NZ?

Thanks,

Richard

[NotAKiwi]

Hey Richard,

I have been doing more digging, I’ve discovered that SEDAVF’s in the Cervical spine are actually very similar to/cross over with Verterbro-Veterbral AVF’s AND, apparently these are much more common in MY age group and in women - unlike the SDAVF’s and SEDAVF’s! Plus, they consider having Ehlers-Danlos Syndrome as a factor that can be behind developing them!

Weirdly excited. Even though I still need a professional to listen to me etc.

I saw an email from neurosurgery to my GP over the weekened which alluded to my host of ‘spurious complaints’. Boy was I mad. And upset. I’ve spoken to a nurse at my practice today and just finished an email to HDC Advocacy requesting we update, or add a new request/complaint.

Good thing my ‘full time (unpaid) job’ is a stay at home mum. I feel I should have an honorary medical degree by now, and could probably do a better job helping people get a good diagnosis than many doctors I’ve seen, given enough time and a computer!!

Thanks for reading my ramble.

[DickD]

I agree: without reminding myself about VVAVF, it makes sense. I asked Google AI the difference between SDAVF and an epidural AVF and they seem quite different.

And I agree about the possibility of the hypermobile EDS being a contributory factor: that seems plausible, too. We do have more than a handful of EDS people in here, though I haven’t created a group for them. We do, separately, have a support forum for EDS patients, though it is not as well attended as this AVM forum.

Time for bed here.

Nos da!

[NotAKiwi]

Extra research suggested it’s still more likely an SEDAVF. I think they’re more common in women when in cervical spine. Anyway I’m tired of guessing and just want to know!

I don’t know if it’s this causing various things but, my goodness, the constant feeling of having an inflated balloon in my head when I’m upright, amongst other things, is really getting to me.

And it’s very sad that a medical oriented AI (Open Evidence) has taken me more seriously and provided a sensible, in depth plan of action than any real person.

It also repeatedly underscored the urgency of getting a DSA. It thought I was the patient’s doctor and was telling me how to try and expedite intervention ASAP. Yet here I am… waiting. Vaguely hoping. Scraping through each day.

[DickD]

I had very odd pressure and dizziness pre op but mine was injecting high pressure blood into my right transverse sinus, and that was flowing blood the ‘wrong way’ in all directions in my venous sinuses. It got worse while I was waiting for surgery, to the extent that lying flat at night became very much like the quote attributed to Dean Martin, “You’re not drunk if you can lie on the floor without holding on.” Very difficult. I got my initial diagnosis in Aug 2016, MRI in Sep 2016 but then referral to neurosurgery in Nov 2016 and actually at the front of the surgery queue at the beginning of April 2017 and an angiogram.

I guess if you’ve got something flowing into veins high up your neck, you’re not far off the same vessels I was dealing with. I’m sure it will depend on what it is flowing into and how high volume it is.

Getting the DSA done is an important step.

My interventional radiologist was happy that the MRI showed him enough and we didn’t do my angiogram until I was in for surgery but that’s not the usual approach.

[NotAKiwi]

That’s super interesting that they decided the DSA wasn’t needed for you. I’m not yet super familiar with how a brain DAVF would differ, aside from location and symptoms, to a spinal one. From looking on Open Evidence etc it seems there’s quite a specific route of abnormal blood flow already mapped. If nothing else, it should narrow down how big an area would need an angiogram. I think part of the urgency coming up in my research pertains to the length of time I’ve had symptoms, the fact I’m largely home bound, and about 2 months in to another “flare” with instead deterioration.

If I didn’t have a 3 year old, I’d be more inclined to consider rocking up to A&E.

Don’t feel you are obliged to reply to my ramblings. But I’m sure you understand the desire just to have a place to put some of the thoughts that fill my mind a lot.

[DickD]

It’s absolutely fine to ramble. I am completely aware that I was the same

Some might suggest I have never stopped!!

I did have an angiogram, to map out the detail, but the doctor was happy that the route we would follow was the right one. So I went under general anaesthetic for both the angiogram and the catheter embolization procedure. It is normal to go under for the embolization; it’s not normal for an angiogram. Basically, if he’d found something contraindicative during the angiogram, we’d have had to abandon and replan but we were both comfortable that it was a viable way forwards, given one of my other limitations.

Getting a DSA is your next step, I’d say.

[Ahmed]

What i understand and learn include neurologist opinion, spinal avm mostly occurs due to some trauma or injury like condition sometimes even long after it happens while brain avm mostly congental.
Hence mostly spinal avm patient didnt recognise.
However both conditions v painful and need pain management longafter surgery though

Regards
Syed

[talloak]

Dear @NotAKiwi

Sorry for my first deleted post - that was me and I was cross on your behalf!

You are absolutely not rambling and it is exceptionally upsetting how your pain has somehow become ‘spurious’ in the eyes of your clinicians. In my experience, clinicians start ‘blaming the patient’ when they are actually out of their depth with their own knowledge and are too uncomfortable to say so. I have been there too.

I was given a gene defect diagnosis in the course of my treatment for my multiple dural brain fistulae but my clinical team chose not to understand the implications of my gene defect properly. My treatment did not go well (to put it mildly) and eventually I was able to move my care elsewhere. Your EDS should not be used as an excuse/reason for putting you into a FND labelled box without thoroughly and thoughtfully assessing your pain and symptoms.

Can you check what information has been used in your referral to the Neurology clinic? Is it the results of your brain and neck MRI which clearly describes unexpected blood flow patterns, or is it the Time of Flight MRA which seems ‘normal’? It could help to get in writing the actual reason as to why you are being made to wait 12 months. Believe it or not, but before my brain diagnosis, I had a ‘normal’ neck MRI and was told to go away. I think they forgot that there is this thing attached to my neck which is called a head.

The politics of getting appropriate and timely care is a nightmare but I wonder if you can use advocacy and/or your local member of Parliament to support your case for a funded referral to an experienced Neurosurgical spinal specialist if the Neurology Clinic cannot help you within a timescale that is clinically safe?

Very best wishes and

Talloak

[NotAKiwi]

I really appreciate your impassioned response! Sure wish some doctors with authority would feel the same way!

My biggest hope lies with the referral to a private Neurosurgeon in Wellington. He should be able to understand vascular stuff, and he also has an understanding of EDS and, in fact, craniocervical instability apparently, which I seem to have and may be contributing to neuro symptoms.

Unfortunately he’s away until the end of the month, which isn’t that long really, but with the way I’m feeling most days, it feels a long time.