Cerebral hypoperfusion in Sjögren’s syndrome

[Sarah Tee]

After reading all the research on hypoperfusion in autonomic conditions and CFS that’s been coming out lately, I wondered if anyone had checked for it in Sjögren’s syndrome. It seems likely it would be found given the close connection between SS and autonomic problems.

And sure enough, I found two studies. One goes into it in some detail, but is behind a paywall. The other is free but only touches on it as it is a review article.

“Intracranial hemodynamic changes in primary Sjögren syndrome: a transcranial Doppler case-control study” (behind paywall)

https://link.springer.com/article/10.1007/s10072-015-2204-3

“Cognitive Function in Primary Sjögren’s Syndrome: A Systematic Review” (available)

https://www.mdpi.com/2076-3425/9/4/85

I also saw in Dr Peter Novak’s lecture at the Dysautonomia Intnl conference last year that he and other dostors from Brigham and Women’s are about to release research showing cerebral hypoprefusion in people with Ehlers-Danlos syndrome, and not just in those with POTS. (I am keeping an eye out for the paper and hope it will be out soon.)

[MTRJ75]

Without reading these, are they saying a transcranial Doppler might be helpful (if available)?

[Sarah Tee]

Probably not. I think it was done more for research purposes, to see whether cerebral hypoperfusion could be contributing to brain fog and cognitive problems in Sjogren’s patients.

If you have Sjogren’s syndrome and can get diagnosed with autonomic OI problems via a tilt table test, then you don’t need transcranial Doppler testing as well, because the abnormalities in blood pressure and heart rate are sufficient. The cerebral hypoperfusion can be inferred as going along with POTS or OH.

If you have Sjogren’s syndrome with OI problems but nothing shows up on a standard TTT, then having a TTT with transcranial Doppler ultrasound added on could help by showing whether your cerebral blood flow is dropping independently of your blood pressure and heart rate. Then your doctors would know to pursue treatments that might help, such as volume expansion.

A different scenario might be adding proof of cerebral hypoperfusion to help with a claim for insurance or government support, if you weren’t getting anywhere. This might be easier to get checked via a SPECT scan, depending on the access in your area to either that or transcranial Doppler.

I know of somebody with POTS who had a SPECT scan that, even though taken supine, showed reduced perfusion to some parts of her brain. She had this in order to help her claim government disability support. I imagine it would be hard to argue with “reduced blood flow to the brain”, as opposed to trying to get bureaucrats to understand brain fog in Sjogren’s or POTS.

But this was just a single case, and I don’t know whether people with Sjögren’s, POTS or similar would necessarily show lack of perfusion to the brain on a SPECT scan. I can’t find any research on it. The person I encountered was being looked after by Australia’s top POTS specialist, who has access to resources that maybe your average specialist wouldn’t. He ordered the test in order to try to help with her support claim, knowing how unwell she was, rather than knowing that it would affect her treatment or even necessarily show anything abnormal. But obviously he must have had a hunch that it would, knowing her cognitive problems.

[MTRJ75]

Cardiologist told me that they could put me on a tilt table, but all it would do is make me feel awful without changing treatment plan. 

I'm curious because when I started having vision issues, my doctor at the time, ordered a scan of the carotid arteries, which was "consistent with a 67% blockage". The doctor thought I had a stroke until the MRI gave a different picture. 

That was a couple of years before I knew POTS was a thing. And I've always assumed it was the cause of the initial scan findings ever since. 

[Sarah Tee]

@MTRJ75, that is very interesting about the carotid scan. I seem to recall that one research paper on cerebral hypoperfusion during TTT used four measurement points on each subject, including the carotid. I imagine it was because researchers wanted to see whether the points differed. If I remember correctly, all points showed similar reductions in blood flow velocity during the tilt test.

(Unfortunately, I can’t find the paper right now. I think it was one of the ones by Visser, van Campen, Rowe, et al.)

However, in my reading of papers a few months ago, most researchers seemed to use the middle cerebral artery. Not sure why. Might just be the most convenient. I did read that, in perhaps 10% of patients, the thickness of the skull and other normal variations in anatomy make transcranial Doppler imaging impossible. But maybe they can be scanned at the carotid instead of on the head?

I think you are right to assume that POTS could be the cause of the low blood flow finding on your scan. Do you remember how the test was done, lying or sitting? (Just an idle question on my part.)

I have been nagging my specialist to order me transcranial Doppler testing, medicated and unmedicated, to prove that I have cerebral hypoperfusion that resolves with vasodilators, which basically proves OCHOS. He has not been keen. He kept saying “Oh, but they might say no” and I kept saying “I realise that, but you won’t know until you ask”. He sometimes behaves like a an unconfident teenager who doesn’t want to make a phone call about a part-time job. I recognise myself aged 17 in him. I feel for him, as lacking confidence is horrible, and he is only recently qualified as a specialist and doesn’t have his own rooms yet, but at the same time he is 30 something years old and getting paid a lot of money.

So I am whiling away this hot day in Asutralia reading about cerebral hypoperfusion while I wait for my next appointment and hope he didn’t say anything stupid to the neurology department to put them off testing me …

[MTRJ75]

17 hours ago, Sarah Tee said:

I think you are right to assume that POTS could be the cause of the low blood flow finding on your scan. Do you remember how the test was done, lying or sitting? (Just an idle question on my part.)

Almost certainly lying, though I could have been tilting or leaning for the initial one. The MRI that cleared everything was lying down of course.