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Transcript
Good morning and thank you for having me. I’m really honored to be here. I’d like to say I’m very pleased that I got asked to talk about the classic presentation instead of the common presentation because I think all too often, we do hear stories that are much like Dr. Blasyzk, where the presentations aren’t really all that classic. So, there are patients with these symptoms but often a lot more as well.
Not really too much to disclose here. I’m on the Medical Advisory Board for the Foundation. We’re building the registry, and I’m super involved in that. Thank you to everybody else who is as well, and I am not the artist on any of these.
So, I’m sure you’re all aware there are some criteria for diagnosis of SIH, or spontaneous intracranial hypotension. Dr. Schievink has been very kind to us and given us a very simple way to approach it. Criterion A is that there’s a leak, and I like that. Then, Criteria B is if you don’t see a leak, then you find one of these other features. And Criterion C is that you have some of those features and an orthostatic headache. He’s given us the most common symptom, and I’m sure we’re all aware of that.
Then, even before Dr. Schievink’s criteria, we had the ICHD-3, and again, this is a headache diagnosis. So, you can have a leak without a headache, but obviously, most commonly, it’s a headache – or at least there is a headache. This was the first criteria.
I think very, very frequently, we do see an abrupt onset – “One day I was ticking along, I was fine, and then I wasn’t.” There’s something going on. And as we said, most commonly, there’s an orthostatic feature – that means it’s related to being upright. Even Miriam was able to tell us – Miriam Webster.
So, 70% to 100% of people, depending on which studies you look at, will have orthostatic or just plain not orthostatic headache. It’s often diffuse, but people often describe this back-of-the-head type pressure. 90% of those headaches are currently reported as orthostatic, and we definitely see that, but we also know in the literature – you find what you look for, so that may evolve over time, and we may see less of that. We don’t know yet. Often relieved by lying down. And we’ve got reference in our last talk to that 48-hour flat test really improving the symptoms.
Sometimes, we don’t see that being upright makes it worse so much as a second half of the day headache. The headache always comes on and worsens, and the worst time is before I lay back down at night.
We often have a description of a coat hanger pain – there’s a lot of neck stiffness reported – and sometimes that intrascapular pain. But keeping in mind that is often also a description of pain in autonomic symptoms. So, this can be hard to differentiate, but we do often see neck stiffness in CSF leak.
Auditory symptoms – that sensation of fullness or ringing. This can be orthostatic or not, and there’s sensitivity to sound or hyperacusis, or like sounding like you’re in a tunnel, or change in the sounds. Nausea is a very, very frequent symptom, reported in 20% to 60%. I would suggest I see it probably slightly more than that in my patients. Dizziness is frequently reported – not just vertigo. That’s probably less frequent than this lightheaded disequilibrium, that trampoline-type feeling – walking on a bouncy castle or feeling off balance. Sensitivity to light is a frequently reported symptom, or other visual symptoms – less commonly diplopia, but we do see blurry vision and diplopia. Photophobia is a very, very frequently reported symptom.
This one is not reported so much in the literature – that brain fog and neuro fatigue. You don’t see it in the classic literature, but you do see it when you – recently, there was a paper where they had the patients report what their symptoms were rather than a checklist of “here’s what I think you should have and tick the boxes” – and it was a much, much higher reported symptom, was that brain fog and neuro fatigue. It’s not usually even called neuro fatigue. When we ask these questions, people call it fatigue, but it is that – if I read or I do something like that, it makes me feel exhausted. And that is something I see in my patients all the time and I think is a very common symptom that we just haven’t been asking about classically.
So, these are the symptoms that we think about as the most common symptoms, and sort of the symptoms that are frequently seen in spinal CSF leak. And there’s lots of papers that are talking about that, but I think we are going to get better and better at this as time goes on.