Slides
Transcript
Thank you so much for the organizers for inviting me. I am thrilled to be here. Last year, I did this virtually, and I think it’s just going to be so much more fun interacting with all of you who are here in person, and also a shout out to those of you who are watching remotely.
These are my disclosures. The only one that’s relevant is a non-paid job – the Spinal CSF Leak Foundation. So, I’m going to do this talk as case presentations rather than just showing you a bunch of data, although I will show some data at the end.
These are some of the patients I saw, and a couple of them are from the literature. The first patient is a 31-year-old woman who came in because she was having double vision and intermittent headaches that had become constant over a period of about two weeks. She went to see an optometrist. He diagnosed a right sixth nerve palsy. She couldn’t move her right eye out, and she had a CT scan of the orbits that was normal.
I saw her a couple months later, and at that point, she was complaining, well not complaining, but she told me that she had right temple pain. She had intermittent burning of the right cheek and the ear, very sharp pain behind the eye, and also pain up on top of the head, as well as dull pain in her neck and dull pain in the back of her head. Very severe sensitivity to light, mild sensitivity to noise – those are the questions we always ask about migraine, but they certainly show up in the presence of a CSF leak. Confusion, nausea, and she wasn’t vomiting, but she was dry heaving.
When she first woke up in the morning, she said that her pain was rated about a 4 out of 10. By the end of the day, it was rated an 8 out of 10. It was worse with coughing, sneezing, bearing down, and, of course, standing. It improved with caffeine and improved with lying down. She had a normal MRI of the brain with contrast.
To your point, Dr. Blasyzk, these are the people as the neurologists that we see. Their imaging is often normal. I’m not going to tell you what happened to these people because I’m only supposed to talk about what they experienced.
The second case was kind of sad. Not more sad than anybody else’s, but sad because it took so long for him to come to see me. 61-year-old guy, he was the Director of Treasury was his official title, for a large company in Dallas. He experienced a three-year history of cognitive decline. He was sent to me by the memory group.
It got to the point where he was not paying bills at home. Now, this is bad when you’re the Director of Treasury and not paying bills at home. This is a real problem. He was falling asleep at his desk. He forgot how to log on to his computer, and couldn’t even recall the day of the week. He didn’t really have much in the way of headaches. The headaches started two years later. They just occurred a few times weekly. They were really relatively mild, and he easily treated them with acetaminophen. There was no postural component, no worsening with Valsalva. He didn’t have any of those other things you just heard about from Dr. Rau – no imbalance, tinnitus, or hearing problems. But he was definitely walking more slowly. He was doing everything more slowly.
They obtained this MRI at the memory clinic, and they actually had imaged him numerous times. They kept getting the report back saying that this is abnormal and looks like a spinal fluid leak, and they didn’t do anything. So that’s the bad part. So, he clearly has a spinal fluid leak. He’s got major brain sag, and even on the right panel, you can see in the middle of the screen there’s this white stuff that is showing up in the area of the hippocampus. That probably explains his problem with his memory.
The next case is a 65-year-old retired professor of nursing. She had persistent headaches after a spinal tap, so this was not spontaneous. They were worse with any positional change, mostly in the front of her head, with aching and vomiting when the pain was severe. About six months later, she developed generalized shaking and also generalized weakness to the point that she was using a walker. She had pretty marked dural enhancement, and they said, “Oh, you have meningitis,” so they treated her with all kinds of stuff, including IVIG, for this meningitis that she didn’t really have.
She noticed that all of her symptoms were better in the morning, including her memory, her balance. She started falling and had to move in with her 92-year-old mother, who was taking care of her. So here she is – she’s in the hospital.
“Other one, too – great. Back and forth. My finger, your nose – good. Other hand. Good.”
“Now, the one thing I think is a tiny bit odd is when I lie down, mostly the tremors go away.”
I don’t know if you can hear, but she’s saying “When I lie down, this stuff goes away.” Very odd. You can see her walking, her hands kind of flapping around.
“Great, come on back.”
She actually had seen a movement disorder specialist and was diagnosed as having functional neurologic disorder, meaning it’s not real.
64-year-old woman who had the rapid onset of orthostatic headaches 4 days after having a skull injury. It was not an open skull injury when a screwdriver fell on her head from four meters in height. She had a rapid onset of orthostatic headache and developed droopy eyelids – both eyelids were droopy. She had blurred vision but no double vision. Her previous eye exams had all been normal. This is a case from the literature. Her MRI showed bilateral subdural fluid collections, midbrain sag, cerebellar tonsillar descent, and loss of the ambient cistern. Her exam when they saw her showed this bilateral droopy eyelids and incomplete eye movements. She had marked limitation of her eye movement.
As you look at the photo, it’s as if you’re looking at her, and all nine panels show different ways she’s looking. So, when you look at the top panel, she’s trying to look up. You look at the right side – she’s trying to look to the right, etc. You can see that her eyes don’t exactly move like they’re supposed to be moving. Over here you can see when she’s looking straight ahead, her eyelids are really droopy. She had a CSF leak as well.
Last case is a 53-year-old man with a seven year history of slowly progressive bilateral arm weakness and atrophy of his arm muscles. It was asymmetrical. This happened quite a while ago. In 2008, he went to see a neurologist. He had an EMG, and it suggested that he had Lou Gehrig’s disease, or ALS, which is a fatal disease, but he didn’t die of it. He’s still alive 11 years later. He had worsening of his limb weakness and wasting, and he also developed hearing loss. And then he had an MRI of his brain that I’ll show you that showed very extensive superficial siderosis that was covering basically the surface of his entire spinal cord. It was also in his brain. He had a large fluid collection with bony spurs in his cervical spine. He later developed parkinsonism that responded to treatment for Parkinson’s disease. They thought it was unrelated to his leak. He continued to go on, I’m not sure what happened to him treatment-wise.
So up there where the arrows are, they’re pointing to the bright stuff on the left side on the spinal cord, which is the CSF. But if you look right next to the CSF, there’s a really dark space that goes all the way down the spine. So, he also has these dark areas. If you look at the brain up there on the left, he has these dark areas. There’s an arrow and a little star next to one of them pointing to the dark areas of the cerebellum and throughout the brain that are the superficial siderosis.
Last case is a patient I saw – a 39-year-old music teacher. She had a history of migraine that started when she was 30 and very predictable, occurring with her menstrual period and easily treated with rizatriptan. On the fateful day of May 3rd, 2014, she went on a band trip. She’s a music teacher, and she probably hauled a few too many tubas off of the bus. She developed a “migraine” that lasted six weeks, and she was never healthy after that. She tried rizatriptan – it didn’t work. She tried steroids, but they only helped for one day. She put herself on a migraine diet. And she found that the lights in school were very bothersome to her. So, the sensitivity to light gradually worsened. So, in September 2014, she could still drive she could tolerate sunlight. She could go to work and more or less tolerate the fluorescent lights at school. By spring of 2015, she could only tolerate incandescent light. By summer of 2015, she was eating dinner by candlelight, not for romantic reasons. In August 2017, she basically had to eat in the dark. That’s how bad her photophobia was. At the time of her visit, all light exposure would trigger a bad headache. She also had what we call allodynia of her scalp – just touching the scalp was painful.
So, when I saw her, she had been around. She had seen one of my colleagues who is an expert in photophobia, who said, “You have to get used to being in the light again,” but couldn’t really tell her why she had photophobia. She said this felt like she had a knife stabbing in her head or a skewer in her head or acid burning down her head – pretty graphic description. The pain was on both sides of her head, neck pain at times. Besides the light sensitivity, she was also sensitive to noise and smells. She wasn’t really nauseated unless she rode in the car, had trouble concentrating, off balance, felt like she would fall, and if she started vomiting, she had to go to the emergency room because she couldn’t stop. Sometimes she got diarrhea as well.
On her exam, she came to see me in neuro-ophthalmology because of the photophobia. She had a completely normal neurologic exam and neuro-ophthalmic exam. I got the records – they were paper records, a stack like this big, and I’m thinking, uh-oh, this is going to be like a wild visit. It wasn’t a wild visit – very appropriate affect. And I knew who she had seen before. She wore dark sunglasses in the office. She covered her head with a towel whenever possible. She covered the computer monitor in the room with another towel that she brought with her, and she even brought her own floor lamp. I must say, in my entire long career, I have never seen anybody bring their own floor lamp into the exam room – with a 38-watt incandescent bulb, which you can’t even buy anymore. She had an MRI of the brain that was also normal, which is why nobody really thought about it.
So here you have patients that have a wide variety of manifestations – very severe light sensitivity, this functional tremor, a picture in their eye exam that looks like a disease that we call chronic progressive external ophthalmoplegia, meaning your eyes aren’t moving in all directions very well, dementia, sixth nerve palsy with double vision, burning acid on the head, and then this ALS-like picture.
There was a meta-analysis looking at the clinical presentations of these patients. I won’t go through the details because of time, but this was published in the literature by D’Antona and his group in 2021. You can see the most common things that Dr. Rau also talked about. But when you get down here to the bottom of the list – other visual symptoms, light sensitivity, movement disorders, non-orthostatic headache, double vision, and cognitive symptoms are on the bottom.
Well, we did our own study. Thanks for the shout-out. And this one has not been published yet because it was things that people filled out on their questionnaire, not responses to validated questionnaires. Head pain and neck pain were number two. We asked people to rank their top three symptoms, and we kind of looked at all those. But look at what really showed up. Some of these patients had a confirmed diagnosis, and some just had a suspected diagnosis because they had a great story, but their imaging was negative.
Problems with concentration, mental exhaustion, moderate memory problems, word-finding problems, and fatigue was also mentioned. Right, number four – unexplained physical exhaustion. When you add all of these up, cognitive dysfunction is probably number one. It came in as number three, but there’s different ways that people described it that really put it high, high up on the list. This is clearly something that has not been in the literature, but it will be as soon as we get our paper submitted.
Other unusual manifestations in the literature – you heard about some today that aren’t even really on the list. Chest pain, for example. One that we didn’t ask about much at the time is on the bottom – dysautonomia, palpitations, rapid heart rate. I hear about this a lot. Various movement disorders have been described – spells of altered consciousness, imbalance upon arising, worsening at high altitude. There’s actually a paper in the literature that it gets better at high altitude, and what I usually hear is that it’s better at high altitude. So, we are at a mile-high city, right? So, you guys will tell me if you’re better or you’re worse.
Milk discharge from the breast – I actually couldn’t find the reference to this, but it’s on everybody’s list. I have not personally heard it. Trouble with speech and swallowing and just orthostatic tolerance and dysautonomia – so many, many manifestations. I hear new ones all the time. And really, although most patients have the characteristic history, SIH can produce some very strange things. And I think it’s incumbent on us as practitioners not to dismiss those things. It’s all kind of a package deal, and some of the things that we thought were really uncommon – like cognitive problems, brain fog, and photophobia – may be more common than we realize.
Thank you very much, and I especially want to thank the patients. I started my new practice. I was at UT Southwestern, and I now am in my own solo practice, and y’all are keeping me very busy. So, I didn’t have to advertise. I have you. Thank you so much, and I look forward to meeting you all. Thanks.