S4:Ep8 – SUNCT/SUNA: Patient and Physician Perspectives



Voice-over: Welcome to Spotlight on Migraine, hosted by the Association of Migraine Disorders. Join us for fresh perspectives by medical experts and advocates as we explore the spectrum of migraine and dig deeper into this complex disease.

This episode is brought to you by our generous sponsor, Praxis.

In this episode, we hear the perspectives of both a patient and a physician about SUNCT/SUNA. First, Dr. Shivang Joshi describes what SUNCT/SUNA is and how it presents clinically. Then we hear from patient Anna Williams about her journey with the condition.

Dr. Shivang Joshi: OK. My name is Dr. Shivang Joshi. I am a headache neurologist at the DENT Neurologic Institute. I am also an associate professor of clinical pharmacy at the School of Pharmacy. And it is my pleasure to talk to you about some very interesting neurological disorders and primary headache disorders. In particular, we’re going talk about SUNCT and SUNA, OK?

So SUNCT is a short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. OK? So that’s SUNCT. And SUNA is short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms, OK? Try saying that very quickly. And let’s start off with a case.

So we have a 44-year-old woman with one week of sudden-onset severe, sharp, lancinating, and stabbing pain in the left retro-orbital region, which radiates to the left temporal region, 10 out of 10 in intensity. There is ipsilateral conjunctival injection and lacrimation and photophobia. Her frequency of attack has increased over the past five days — progressively increased to about a hundred per day after five days. Attacks lasted approximately 5 to 20 seconds.

They’re not associated with any vomiting, nausea, weakness, or any sensory disturbance. There are no other typical migrainous features, and symptoms are not alleviated by over-the-counter medications. And there are no aggravating factors that she mentions. 

Unremarkable past medical history. Unremarkable social history. No meds have been taken that have been effective. The neurological exam, including the cranial nerve exam on day one, was unremarkable. OK? So a complete detailed neurological exam was unremarkable.

Now, based on this, how would you think about the differentials, right? So we know that they’re short. They’re unilateral. There’s conjunctival injection and tearing. So SUNCT could be a differential. Other differentials in this area that are very similar that can be confusing include trigeminal neuralgia, primary stabbing headaches, paroxysmal hemicrania, as well as cluster headaches.

So the course of this patient: she wound up going to the emergency room and was given Reglan, morphine, Dilaudid, with no improvement. There was indomethacin that was given, said there was no improvement. They did an MRI of the brain, MRA of the brain — orbits unremarkable. A lumbar puncture was unremarkable. Several labs — including ESR, CRP, and ANCA — were all negative. These were our inflammatory labs. They were all negative.

On the second day of the hospital course, she started to develop a left-sided non-blanching papular rash located in the temporal forehead and dorsum of the nose and eyelid region. It was severe burning pain with light touch. There were no vesicles seen at this time. She was immediately started on acyclovir and given pain medications and gabapentin.

So, in this particular case, SUNCT was presented as a prodromal symptom of acute herpes ophthalmicus, right? So why is this important? Because it helps in the pathophysiological mechanism, understanding the diagnosis, and it’s important to know that there could be secondary factors. 

In fact, SUNCT was first described in 1978 by Sjaastad, OK? The epidemiology, the prevalence and incidence — it’s predominantly more males versus females. This might have changed over time, depending on one study versus another. You might find different things.

Now, you could have idiopathic SUNCT, which is the majority of the cases, and you could also have symptomatic SUNCT, which is something that we found in this sort of scenario. In 1991, Bussone described several different cases of symptomatic SUNCT. One of them includes arteriovenous malformation. Other things include pituitary gland prolactinomas, vertebral artery malformations, multiple sclerosis, meningioma, viral meningitis. So it’s very important to do a full neurological exam, get a thorough history, and then look and think about some of these differentials.

So SUNCT and SUNA are both characterized by sudden brief attacks of severe unilateral head pain in the orbital, periorbital region, and the temporal region, with ipsilateral autonomic features. SUNCT specifically must consist of both conjunctival injection and lacrimation, or tearing. SUNA may include either conjunctival injection or tearing, but not both, right? Other things that are common to both include other cranial autonomic symptoms, and they include nasal congestion, rhinorrhea, miosis, ptosis, eyelid edema, as an example, and several others.

Now, for some patients, the triggers can be very minimal, right? So just simply touching the face, bathing, showering, or even neck movements can trigger SUNCT or SUNA. Now, unlike trigeminal neuralgia, in SUNA, they generally don’t have a refractory period. We know that in trigeminal neuralgia, generally, at bedtime when you sleep, symptoms improve.

So, typically, the neurological exam is normal. In our case, it was abnormal, pointing to a secondary cause. And in the majority of the times, you might not find anything on an exam. But it’s very important to do an MRI of the brain. You want to get dedicated imaging of the trigeminal nerves. Remember, multiple sclerosis was one of the differentials. So if you look at the nerve root zone entry, sometimes you can have multiple sclerosis or demyelinating lesions at the entry zones of the nerve root. And they present usually with bilateral trigeminal-neuralgia-type pains. Nevertheless, it is a differential, so you should get dedicated imaging of the trigeminal nerve as well as the pituitary gland, as well.

As far as the mechanism, I mean, I think that there is some disturbance of the trigeminal nociceptive signaling pathways. We know that the trigeminal vascular system may play a role. There could be possible disinhibition of something called the trigemino-autonomic reflex. And this is what is responsible for the autonomic features: the tearing, the droopiness of the eyelids, et cetera.

Interestingly enough, in cluster headaches, we know that oxygen stops some of these trigeminal autonomic symptoms by inhibiting the parasympathetic nervous system outflow in a specific region of the brain. There may be a possible role for CGRP. We know that it’s being studied in cluster headaches. We know that it’s being studied in trigeminal neuralgia. 

Treatment modality’s primarily focused on prevention. It’s too short for it to be acute treatment. So prevention includes lamotrigine, topiramate, gabapentin, carbamazepine. Other modalities include occipital nerve blocks, supraorbital nerve blocks. Sometimes there’s a surgical approach too with decompression, and also neurostimulation can potentially help too.

There are some devices that are primarily used in migraines. There’s the Cefaly device that targets the supraorbital nerves. There are devices that are going to be coming out: in particular, Neurolief has a device that not only targets the supraorbital nerves, but also the occipital nerves at the same time –Relivion. They are working on that. So there are potential things that — our patients have other options.

I think a lot more research needs to be done. There are great resources with the American Migraine Foundation, the Association of Migraine Disorders. There’s a lot of information out there that patients can reach out to. But I think very important as a clinician is to be aware of these symptoms and to realize that patients are not crazy, that there could be something else going on. Thank you for your attention.

Anna Williams: Hi, my name is Anna, and I’ll be sharing my patient journey with SUNCT today. One thing I have learned is my journey may not look like your journey, and that’s OK. But I hope sharing my story will help those of you unfamiliar with the term understand a bit more about what it is like to have SUNCT, and I hope that if you have SUNCT/SUNA, hearing my story will help you feel not so alone and will encourage you to share your own story.

I’m a single mom of two teenage girls. They have never known me without navigating some sort of chronic pain disorder, and I refuse to let it rob me of being the best mom that I can be. Some days, we’re able to be adventurous and go on road trips, but others are movie days. The hard part is not knowing from one moment to the next, so I always have to be prepared.

In my journey, I have become a patient advocate. I love learning, collecting resources. Every advocacy class I run into, I want to be a part of it because I want to know more about what I can do to make a difference. My dad says it best: “I’m so sorry you have to deal with all of this pain, but then I look at the amazing ways you’re able to speak up and help others.” 

Pain has taken away my ability to do things I once did. However, I still have purpose and goals. They just look differently now. I enjoy helping others tell their story.

My SUNCT attacks are typically stabs of pain that occur for 5 to 30 seconds. My eye is red and watery, my nose becomes stuffy and runny, my eyelid swells, and my eye is droopy. When I’m in pain, my brain gets creative with imagery. During my last episode, my brain was convinced I had a sweat bee stuck in my eye socket that would sting me randomly, sometimes once, and then it would rest; other times, three to four times quickly, and then it would stop. The times between the stings would range from seconds to 5 to 60 minutes.

My first episode was on June 13th of 2020. I’d had an amazing day. My girls and I did our first Miles for Migraine virtual walk. Later, I fixed dinner, but before I could sit down to eat, I was hit with a cluster headache attack. I was still struggling to get oxygen, so I didn’t have a good way to stop the attack. So about two hours later, it went away. 

This time was different though. Once the cluster headache attack subsided, I began to get what felt like needle stabs in my eye. My left eye got red and watery, my nose got stuffy and runny, just like a cluster attack, but the pain was very different. It would last a few seconds and stop. Since it lasted for seconds, I would wipe away the tears and try to open my eyes, only to be met with a fresh stab. So I covered my left eye and tried to open my right. This caused my left eye to move, which triggered another attack, making my eye water even more. I went to bed. I was terrified.

I had some medications that I’d used to manage my headache disorders, but none of them were helping. I hoped I’d wake up and everything would be okay. I was wrong. I barely slept, as the stabs kept occurring. Every single stab woke me up. I wondered if I should go to the ER, but with the pandemic, I knew someone would have to drop me off at the ER and I would have to enter alone, and I couldn’t even open my eyes to see where I was going. I didn’t know if I could explain the pain to someone or if they would even know what was going on, because I sure didn’t. 

I never even tried to count how many attacks I had, because they were just coming so rapidly. I would be lucky to have 30 seconds pain-free before another attack. Sometimes I could go 20 minutes, and those were exciting minutes when that happened.

Monday morning, my daughter helped me call my neurologist’s office. I left a message and waited. Tuesday at 3 AM was my last attack. This was a grand total of 58 hours. I didn’t do anything different; the attacks just stopped. My eye looked beaten and battered. My vision was blurry. I could open my eyes, and attacks were not triggered. 

Wednesday morning, my neurologist’s office? Yeah, they returned my phone call and said if I had eye pain, I should go to the eye doctor. I knew my message had been lost in translation. At the follow-up appointment a month later, my neurologist said it sounds like it’s related to cluster headache disease, but he had no idea what I could do. 

Fast-forward to January 8th at 4:55 AM. I woke up to a cluster attack. By this time, I had more treatment options. I used high-flow oxygen, and I aborted the attack within minutes, just like I’d been doing. However, within minutes, the stabbing pain was back again.

Of course, it was Friday, and I already knew calling my neurologist wasn’t going to be helpful. I kept telling myself that last time, it went away, so surely it would this time too. However, the hours went by, and the stabbing increased, and I became less and less convinced. The stabbing pains would trigger a cluster attack. I’d stop the cluster attack, only for the stabbing to continue. 

At 7 PM on the 10th — so, 62 hours later — the pain finally stopped. No more stabs. Again, my eye was swollen and sore. I called, got an eye doctor appointment just to rule out that this was not some sort of odd eye disorder causing this pain. 

I had a follow-up with my neurologist a week later. I’d snapped a picture so I could at least show him a picture. Again, he wasn’t sure what was going on or what treatment I could use for it. He said it sure sounds similar to cluster headache; it just has different timing. 

At this point, I started researching more of the attacks, especially through Clusterbusters, and came across SUNCT. I thought surely not another one.

March 9th hit me like a ton of bricks. I’d been struggling to manage my cluster headaches. The all-too-familiar stabbing pain started around 9 AM this morning, and I thought there is no way I’m having another episode so soon. This time, I swore I knew what to do. I’d remain calm because it always goes away, right? So instead of triggering more stabs by trying to open my eyes, I’d just keep them closed and stay calm.

My girls, by this point, knew the routine. My youngest made lunches; my oldest made dinners. They cleared a path since I couldn’t open my eyes. I had a timer going in my head. These attacks should be subsiding. I’d been averaging 60 hours of torture. The 60 hours came and went. 

This episode lasted 71 hours. It hit me so hard because it was during the school week. Thankfully, my kids were doing virtual school, but I soon realized how badly I was impaired, because if they had been going to the school building, I don’t know what I would’ve done.

Even once the attacks stop, it still takes another two to four days to become functional, so we had to cancel a lot of activities. This time when I met with my neurologist, I took in the diagnostics for SUNCT, I brought in photos, and my daughter even helped me catch an attack on video. He agreed that’s what it looked like, but it’s not something he’s familiar with. So I sought out a headache specialist who does telehealth, and after talking with me about my episodes and viewing the video, my diagnosis was confirmed.

Having a name does help; however, the question is what to do for the next episode. This is where I am now. My local neurologist is out of ideas, and most of the medications that are prescribed for SUNCT/SUNA are contraindicated for me. So I’m now working with a headache specialist via telehealth, and we have some ideas on the table. 

My main goal right now is prevention. I have more tools since my episode in March. I’m up to four preventatives for my migraine and cluster disease as well as four abortives. I have pharmaceuticals, devices, vitamins, and supplements. My newest one is gammaCore, and I use it for preventative. I hope in the next couple of months to set up a medical plan of action for my next episode. Since they’re not planned, we have to be able to implement something as soon as it happens. 

I’m a part of many communities. For SUNCT, the first home I have is Clusterbusters. This is where I learned about SUNCT initially, and this community has been a lifesaver for me and is very supportive. From there, a fellow SUNA patient told me about a Facebook group for SUNCT/SUNA patients, and I can be found there, as well as others globally who experience this disease.


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