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Squid Game: The Official Podcast

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Keys and Knives - S3 Ep 1 26:28

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Squid Game is back—and this time, the knives are out. In the thrilling Season 3 premiere, Player 456 is spiraling and a brutal round of hide-and-seek forces players to kill or be killed. Hosts Phil Yu and Kiera Please break down Gi-hun’s descent into vengeance, Guard 011’s daring betrayal of the Game, and the shocking moment players are forced to choose between murdering their friends… or dying. Then, Carlos Juico and Gavin Ruta from the Jumpers Jump podcast join us to unpack their wild theories for the season. Plus, Phil and Kiera face off in a high-stakes round of “Hot Sweet Potato.” SPOILER ALERT! Make sure you watch Squid Game Season 3 Episode 1 before listening on. Play one last time. IG - @SquidGameNetflix X (f.k.a. Twitter) - @SquidGame Check out more from Phil Yu @angryasianman , Kiera Please @kieraplease and the Jumpers Jump podcast Listen to more from Netflix Podcasts . Squid Game: The Official Podcast is produced by Netflix and The Mash-Up Americans.…

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E1: A terrorist without a belt

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Part 3: A violin is heard in Denver I hear a violin, I think, Danielle’s arm floats away, we go searching for 5/10,000 of a litre of spinal fluid. We consult a sage in the mountains. Terrorist caught. Chapter 13: A violin amongst the orchestra Dr. Donald Gilden (1937-2016) Chapter 14: The prednisone lull Chapter 15: Danielle's arm floats away Chapter 16: The story Dr. Gilden wrote Varicella Zoster Virus Chapter 17: There's a pump in my fanny pack Chapter 18: A terrorist without a belt Ceremonial belt worn by Green warriors. Transcript Chapter 13. Uh, violin. Uh, no. A Viola. Or. Mm. I mean, I dunno. A, a violin. Yeah i think it was a violin. Chapter 13. A violin is heard in Denver. I literally read hundreds of articles. All while trying to keep our little family going with a newborn and two little kids and work and trying to help Danielle. And then one night, I was reading an article and something caught my eye. A name. I saw it in one of the references. Doctor Don Gilden. I knew that name from somewhere. I went and I read the reference. It's suggested that CNS vasculitis is often caused by varicella zoster, the virus that causes chicken pox and shingles. But Danielle hadn't had shingles. She had had chickenpox when she was a kid, but she wasn't elderly and she wasn't immunocompromised, and in any case, her spinal fluid had been tested for a number of viruses, including the varicella zoster virus, by PCR, nonetheless. But as I was poo-pooing the idea, I heard a violin 13 years earlier, in the year 2000, while I was interviewing for neurology residencies, I had gone to Denver. Now the main thing I remember about Denver was the Tattered Cover bookstore on the 16th street mall. But that night, while I was sitting there, desperately reading review articles on CNS vasculitis. I remembered something else about Denver. I remembered Dr. Gilden's violin. You see, most interviews for residency, say with the chairperson of the neurology department, they were very business-like and formal affairs. But when I went to Denver to interview at the University of Colorado for neurology residency, in 2000, Dr. Donald Gilden was not formal at all and he was not businesslike at all. He had a violin in his office and a music stand and he told me about the origin of the violin which I was too nervous to remember. He told me about great skiing around Denver. As I researched, reminiscing about Dr. Gilden and his violin, Danielle called down to me half asleep. I guess she could hear me typing. She told me something like, you're going to burn yourself out. You need to take care of yourself. Do you need to go to bed? You have to take care of the kids in the morning. I told her I'd be up to bed soon. Later, lying there next to her. I couldn't sleep. Dr. Gilden and his violin kept swimming around in my head. Even though I knew I was grasping at straws. Even though the idea of the varicella zoster virus causing this seemed totally out there, something in what he had written got me out of bed and I went downstairs and I wrote this email. Dear Dr. Gilden. You won't remember me. But you interviewed me for neurology residency in 2000. I remember thinking what a nice man you were. And I think I recall you had a stringed instrument in the office. A violin, was it? Sadly, my wife has fallen ill and I'm hoping you might be able to give me some advice. She appears to have CNS vasculitis, similar to some patients described in your papers. Her spinal fluid PCR testing for HSV 1, HSV 2 and VZV were all negative about five to seven weeks after the onset of her symptoms. As described in your papers. I'm thinking we might need to do antibody testing for VZV on the spinal fluid sample. As you can imagine, this is a very scary and difficult time, and I'm doing my best to try and help her. Sincerely. Roy Baskind. I have sometimes had the problem of not being able to diagnose the difference between a violin and a Viola. And I didn't want to insult him by calling his instrument by its incorrect name, but I wanted Dr. Gilden to know that he had met me. That I had been in his office. That we had some tenuous connection that I was trying to rely on now. I sent that email. At 12:16 AM on January 8th. 2014. And when I wrote it, I was kind of thinking about it as a real shot in the dark, a real hail Mary. We had already tested Danielle's spinal fluid with the most sensitive test we have, which is PCR, and it was all negative for any infectious cause of vasculitis. So honestly, I didn't really think much about the email. Just a sort of desperate husband grasping at straws kind of thing. I also had no idea if Dr. Gilden would respond to me. I had met him 13 years earlier for maybe, uh, 20 minutes. But when I woke up the next morning, Dr. Gilden had already responded. Dear Roy. I am so sorry to hear about your wife's illness. Since she has a CSF pleocytosis and infarction, vasculopathy is indeed a consideration. I would be pleased to check her CSF and serum for antibody to VZV. Please send me at least 500 microliters of CSF and one to two milliliters of serum overnight mail at room temperature to me at the address. Don Gilden MD Louis Baum endowed chair, and professor department of neurology, mail stop B182 University of Colorado School of Medicine, Aurora, Colorado. What you hear in that email is not politeness. Don was truly sorry about my wife's illness. And if you knew Don, you know he was genuine about that. The problem was this. We didn't have any spinal fluid to send to him. Not even 500 microliters. It was all gone. Used up. You'll remember that Danielle's balloon had been popped and it was leaking when she had had the lumbar puncture. She had been on two blood thinners. And so the tiny little hole that the needle made. And the canvas-like dura around her nervous system. That hole wasn't healing. And so the spinal fluid was slowly leaking out of the nervous systems compartment. So she had low spinal fluid pressure and because of that she couldn't stand up. Every time she would, she would get a headache and neck pain and become nauseated. She was literally pinned to the bed. I had to basically lie down. Otherwise, I would get a headache. There was not going to be a second lumbar puncture. Not for a shot in the dark diagnosis, thought of by her husband grasping at straws because he remembered a violin. Especially not while she was on two blood thinners that we were too afraid to stop. There was no way any of us were going to repuncture that leaky balloon. There had been no DNA of that virus on a super sensitive PCR test. And as everybody knows, especially now after the COVID pandemic. PCR is king. Antibody tests. Meh. But Dr. Gilden, just his few kind words. And that memory of his violin. And his grandfatherly way, they were something to grab onto in the tsunami that was dragging us out to sea, and I grabbed it. I called the lab to see if there was any spinal fluid left and the lab tech put me on a long hold while she checked in the fridge. When she came back on the line. She told me, sorry, we've used it all up. I sighed, defeated. As the phone. I was going down on the receiver. She said something and I put it back to my ear. All I heard was SickKids. In Toronto, the hospital for sick children has grown into it's oddly straightforward nickname, SickKids. For a long time. It was where all the PCR testing for HSV and VZV in the city would be sent. The chicken pox virus in the spinal fluid is usually considered a pediatric problem. The technician was telling me that a tube of fluid had been sent to sick kids eight days earlier and I should check to see if there was any fluid left there. Odds of that, um, about zero, I thought. I called the extension she gave me. It was a freezing cold Wednesday in the midst of the ice storm. The lab technician was annoyed. Some doctor from another hospital asking if there's any spinal fluid left from a test from eight days ago. She said, hang on, hold on. Let me check. And she never came back. I emailed our hospital lab manager, Manwell. Geraldo. Hi, Manuel. I hope you're doing well. I have a strange request. My wife is ill. I need some of her CSF, but I don't think we have any left in the hospital. Is there any way you can see if you can track any of it down from any send-out labs that might have been done. I would so appreciate it. He responded. The team's looking for any CSF leftover. The chances are low since the sample was sent more than five days ago, but we will keep checking. I hope your wife will get better. Let me know if I can be of further assistance. And then 24 minutes later on Wednesday, January 8th. At 10:24 AM. He responded with this. Good news. SickKids has 0.5 MLS. What test would you like to run? Call me. He told me that the lab normally keeps spare spinal fluid for five days. Seven days had passed since her lumbar puncture. But since those seven days straddled the new years holiday. They didn't count. We were in luck. Toronto had been stricken with the worst ice storm in decades and the only problem now was getting the fluid across the frozen city and onto a plane to Denver. I sometimes think how hilarious it is that there were probably thousands of liters of gas used to get that half a milliliter of spinal fluid from the hospital for sick children to North York General Hospital to Pearson international airport and up in a plane over the Rocky mountains to Denver. Chapter 14. Chemical weapons The treatment for primary CNS vasculitis. Is the same treatment we use for all inflammatory diseases that are presumed to be auto-immune. Prednisone. And then wait. Wait to see if it works. I was really upset about going on a high dose steroids. The month that I was on high dose steroids was terrible. It felt like I, I, it made me feel really loopy. I would look at myself in the mirror and feel really distorted I don't know if I look the same or if my face got puffy or not, but I just felt like everything was very distorted. And, um, going to be on this medication for a long time and we didn't even know if it was going to help and so there was a lot of anxiety and uncertainty. I was feeling very depressed. I was feeling very, kind of traumatized But as bad as prednisone might be, the alternative was going to be much worse. If it didn't work, meaning if things turned south if she had another Tia or another stroke. Then the next treatment would be cyclophosphamide. One of the big guns, as we say. Which is ironic because actually cyclophosphamide is a nitrogen mustard, derived from a chemical weapon, actually. It's chemotherapy with the idea that we destroy your immune system so it can build back better. Chapter 15. Danielle's arm. Floats away. After six weeks of hell. Things seem to settle down. Things went quiet. Danielle's post lumbar puncture bubble in her head dissolved. She had no more migraine auras or numb arms and legs or headaches. She had been on prednisone for about three weeks and so it seemed like the prednisone was working. We moved back into our house. Micah was almost three months old now. And things seemed okay. One night, I slumped down on the basement couch after putting the kids to bed. Danielle sent down to update her friends and family in an email and I was calmed by the sound of her typing at her furious typing pace. She sort of had a, yeah, things will work themselves out kind of rhythm to her typing. She was on the computer behind me. And I was flipping through the channels. What got my attention was that. That pause. I turned around almost reflexively. Her left hand was still on the keyboard. But she was holding up her right hand and staring at it. Like it was an alien. She was turning it back and forth, looking at both sides. What's wrong? I asked. How fast do you think you type, like how many words per minute? I don't know. Okay. You think I was typing so fast that-? I just remember in my mind, you're like furiously typing ch-ch-ch-ch and suddenly silence. And then I turn around and look at you and you're staring at your hand. I remember I don't know if that's a false memory or not, but I remember standing in front of you next to the glass wall in the basement. You telling me that your hands floating away and as I think we're discovering here, me being absolutely terrified she's having a stroke, you just telling me that you're going to go to bed, me following you up the stairs, thinking should I call 9 1 1 now is and waiting to what was going to happen. You getting into bed and me just standing there, if you're going to have a stroke or not like, and at what point should I call 9 1 1? This time, her neurologist husband knew she was having a stroke. But by the time we got upstairs, she told me it was passing. Just a TIA then. She told me it felt like her arm had floated away. And then come back to her. It was midnight. She was already on aspirin and Plavix and prednisone, so what the hell was an ER doctor going to do for her? Anyway, I thought. I decided we did not need to call 9 1 1 or go to the ER. Nope. I did not seek medical attention. Instead I went back down to the computer and I sought my father's attention. I Skyped him. My dad is a surgeon. But he has an old school breadth of medical knowledge and decades of experience doctoring and a razor sharp mind. While we were complacent thinking things were getting better. She had just had another TIA. Back to square one. Three weeks on prednisone, a waste. My dad told me what I already knew. The game was up. We had no other choice. Chemical warfare. Cyclophosphamide. This is how auto-immune diseases work. Long, slow war. You don't want to kill too many civilians. So you, you keep your big guns back until your little guns fail. Cyclophosphamide is the big gun. It was time to scorch the earth. Chapter 16, the story Dr. Gilden wrote. I texted our neurologist who, although he must have been very annoyed by me texting him at midnight, responded to me kindly. We made a plan for Danielle to come to his office the next day and start cyclophosphamide. I imagined her losing her immune system. While we treated an invisible auto-immune army in her brains, blood vessels, at least with cancer, you can see it on a scan. You can watch it shrink or watch it grow. You can see its innards under a microscope Now, we were going to give her chemo. For what? A presumed, probable, likely, what else could it be kind of diagnosis? As one does, when you Skype your parents generally. You check your email at the same time. At least I do. Come to think of it. I checked my email while I'm video conferencing with just about anyone these days. I said, hang, hang on, dad. And there it was. An email from Dr. Don Gilden. January 23rd. 2014. Hi, Roy Danielle serum and CSF, both contained antibody VZV and the serum to CSF ratio of anti VZV IgG was greatly reduced. Those are the typical findings in patients with VZV vasculopathy. Call me if you want to discuss. I call Don Gilden. He didn't remember meeting me 13 years earlier, but he did confirm it was a violin. I recounted to Dr. Gilden what had happened to Danielle and the last three months. The headaches. The confusion. The strokes. The blood vessels. The inflammation in her spinal fluid. The post lumbar puncture headache. The prednisone. And then the TIA she had had just a few hours before going to bed the night before. I told him that we were on our way to initiate treatment with cyclophosphamide that morning. He took a long considered pause. And then he told me no, do not do that. Cyclophosphamide will kill her. She has a shingles infection in the brain's blood vessels. She needs to come off prednisone aSAP. I was incredulous. But she had never had shingles. He walked me through the antibody test. And then he took me by the hand and he told me the story that he had been writing through his whole career. First. In 1983, Dr. Gilden and colleagues. Published an article in nature. Proving that the visa VI virus. Hides latent in the central nervous system. Waiting for its opportunity to reactivate. Second, shingles can and does occur without a rash. I had never heard of this. I've come to realize that most doctors have never heard of this. So doctors, are you listening? There's a long, but not well-known story in the medical literature that spells it out. Zoster sine herpete or shingles without a rash has been suspected for decades, but in 1992, and again, in 1994, Dr. Gilden and his colleagues published papers, marshalling all the evidence proving that zoster sine herpete can and does occur, shingles can occur without a rash. And then he told me something that blew my mind. The virus that causes chicken pox. And then goes into hiding and the nervous system. And can reactivate to cause shingles. Sometimes without it's rash. It has a, another nasty trick up its sleeve. Instead of creeping out to the skin, along the nerves it can go down another nerve branch. And settle in the walls of the brain's blood vessels and set them on fire causing, are you ready for this? CNS. Vasculitis. But I was incredulous. The DNA of the VZV virus had been checked with a PCR test. And it wasn't in Danielle's spinal fluid. Looking in the wrong place, he explained to me. Point number four. Unlike other infections in the nervous system that you can see on the battlefield in the spinal fluid. This was an infection hiding inside the blood vessel wall, and so the virus itself, its DNA and its proteins, they weren't in the spinal fluid. The DNA PCR test was insensitive. The wrong test. And since you can't go and swab the inside of a blood vessel wall, you have to look for antibodies being made and the nervous system against the virus. You have to look for your immune system's response to the hidden terrorist. I was stunned. We cancelled our appointment to go and start cyclophosphamide. It all made sense to me, but I was skeptical. Dr. Gilden was used to facing skepticism. As I'm sure he had done a thousand times before, he patiently explained to me what happens when you misdiagnosed VZV vasculopathy for primary auto-immune CNS vasculitis. In 1995. Dr. Gilden did something so quietly bold. It gives me the heebie-jeebies. He and his colleagues, as he politely put it, quote, "restudied the pathological material of a patient who had died and been presented in the new England journal of medicine, clinical pathological conference." The patient was a 73 year old man who had died of fatal vasculitis of the central nervous system while on prednisone and cyclophosphamide. Dr. Gilden, like the investigator who arrives long after the plane's gone down deemed an accident, he sorted through the wreckage and found his terrorist hiding. This time in the, are you ready for this? In the blood vessel walls and the brain. That's right. In 1995. Dr. Donald Gilden found VZV DNA and proteins in the inflamed and closed off blood vessels of the brain of a man who had died and been deemed by the new England journal of medicine to have died of an auto immune vasculitis. Meanwhile, his immune system had been immobilized by first prednisone and then cyclophosphamide while the virus ate away at his brain's blood vessels. I love the title of the journal article. Varicella zoster virus, a cause of waxing and waning, vasculitis: The new England journal of medicine. Case five-1995. Revisited. Huh, revisited. Chapter 17. There's a pump in my fanny pack, in my fanny pack, in my fanny pack. There's a pump in my fanny pack, my fanny pack, ah pum. Now we had to turn the ship around. We had to wean the prednisone as quickly as possible and start Danielle on an antiviral treatment. 21 days of intravenous acyclovir. Acyclovir works slowly by feeding the virus blank bullets as it tries to build new DNA. Starting acyclovir, I had this IV drip in my arm. I had to carry around- was that for a whole month? I had to carry around a little pack with an IV bag in it. I remember it cycled and the pump started going on every few hours. It was the sound of the pump worrying, like we're click work, click work. I shudder to think of what would have happened if she had gone on to cyclophosphamide. It would have further weakened her immune system. And she may have ended up like that patient in the new England journal of medicine. Danielle was cured. But the cure was not sudden, and it was not miraculous. There was no victory celebration. There were just no more headaches. No more auras. And no more strokes. I guess we still didn't know what the, what the long-term consequences of this whole experience were and, I remember it put a lot of stuff into perspective about day to day and the kids, and just thinking like nothing else matters, except that we're and healthy together. What do you think the long-term consequences have been? Well, this is multifactorial, but I feel like I never got back to my level of, not competency, but like ability to, multitask and be organized and get stuff done. And, um, but it could be because I'm not back at work, it could be because I've got three kids, it could be, cause of all these other things what do you think about your mood? Has it changed your mood? I think my mood has changed, but again because I'm just less patient. I get more overwhelmed. I lash out more. I probably used to do it before as well, but it wasn't as pronounced if I didn't have any kids. And then if I had one kid or, and then I had two like the amount of lashing out increases. Proportionate to the children you have? How do you think that whole experience, whether it was the disease or the experience with just being sick or whatever dynamics, whatever the whole thing, if we just, just call the whole experience, how do you think it changed you? I feel like this whole experience has marked us. Um, it's not just a happy-go-lucky life where not everything goes right anymore. It's like, this is, this is life. There's bad experience too. And they are part of your story. Chapter 18. A terrorist without a belt. Yeah. Indeed. That is our story. But the thing is, it's much bigger than just our story. I think the big story here is the story that Dr. Gilden wrote over his long career. He saw something that others didn't and he showed with careful science something that even to this day, most physicians don't know that this virus can cause stroke. I know I'm not the first doctor or the last who has missed a diagnosis of shingles without its rash. And I know I'm not the first neurologist who has missed a diagnosis of varicella vasculopathy. Well, almost missed it. I bet you even the world's greatest neurologists have missed it. The problem is that when there's a visible rash, the diagnosis is a cinch. But if there's no visible rash, the diagnosis is not a cinch. The word shingles comes from the Latin word for belts. Cingulus as in a cinch because the rash that comes with shingles reminded early physicians of a ceremonial warriors belts. A belt they borrowed from the Greeks who called the rash zoster. A ceremonial belt that a Greek warrior would wear around his waist with little studs on it. And the Norwegians have the best name for it. Not only telling you where the belt comes from but how much it hurts. Helvetesild: the belt from hell. So the names of the disease, regardless of the language, they harken back to the rash that it can cause. But it's not just a rash It's a neurological terrorist that hides in the nervous system. And like a terror cell it waits for the right opportunity to reactivate, and when it does, it can cause all kinds of terror in the nervous system. And here's the point. That terrorist, it can come from you with or without its belt. I found that very hard to believe that something as common as shingles can cause so many different issues in people. Um, that the ones that aren't visible, the ones that don't cause lesions on the skin are not known about. And I just found this unbelievable. So thinking about all the people in the world over time, you have had unexplained strokes they're not sure what's going on and like, wow, this is really something that neurologists need to know about. Sadly. Dr. Gilden died in 2016. In 2009, he was interviewed by the Lancet neurology and they asked him: What had been the greatest achievement of his career? He answered. Our lab was the first to prove that VCV is latent in human ganglionic neurons. We also prove that VCV can cause zoster sine herpete, pain without a rash, as well as vasculopathy. And when he was asked “What would be your advice to newly qualified doctors?” he said: Never forget that it is a privilege to care for sick people and that your responsibility is to continue studying medicine as long as you practice. Deeper Dive Burgoon, M., Hammack, B., Owens, G., Maybach, A., Judith Eikelenboom, M., Gilden, D. (2003). Oligoclonal immunoglobulins in cerebrospinal fluid during varicella zoster virus (VZV) vasculopathy are directed against VZV. Annals of Neurology, 54(4), 459-463. Cohrs, R., Barbour, M., Gilden, D., Meier, J., Holman, R., Croen, K., Smialek, J., Straus, S., Cohrs, J., Srock, K., Barbour, M., Owens, G., Mahalingam, R., Devlin, M., Wellish, M. (1996). Varicella-zoster virus (VZV) transcription during latency in human ganglia: detection of transcripts mapping to genes 21, 29, 62, and 63 in a cDNA library enriched for VZV RNA. Journal of Virology, 70(5), 2789-2796. Gilden, D., Cohrs, R., Mahalingam, R., Nagel, M. (2009). Varicella zoster virus vasculopathies: diverse clinical manifestations, laboratory features, pathogenesis, and treatment. The Lancet Neurology, 8, 731-740. Kronenberg, A., Schupbach, R., Schuknecht, B., Bossart, W., Weber, R., Gilden, D., Speck R. (2002). Multifocal Vasculopathy Due to Varicella-Zoster Virus (VZV): Serial Analysis of VZV DNA and Intrathecal Synthesis of VZV Antibody in Cerebrospinal Fluid. Clinical Infectious Diseases, 35, 330-333. Gilden, D., Kleinschmidt-DeMasters, B., Wellish, M., Hedley-Whyte, E., Rentier, B., Mahalingam R. (1996). Varicella zoster virus, a cause of waxing and waning vasculitis. Neurology, 47(6), 1441-1446. Nagel, M., Forghani, B., Mahalingam, R., Wellish, M., Cohrs, R., Russman, A., Katzan, I., Lin, R., Gardner, C., Gilden, D. (2007). The value of detecting anti-VZV IgG antibody in CSF to diagnose VZV vasculopathy. Neurology, 68(13).…

1
E1: A terrorist without a belt

2 years 전

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Part 2: The problem with the branches Wherein Florida. Upon a roof. A Cephalopod on a hot tin roof? Not a cephalopod, a breakdancing frog. And then the cemetery up the street and the real problem: the problem with the branches. Also, a bit of herpetology and a spinal tap. On ice, lots of it. Chapter 8: The flip Chapter 9: Snake's gotta eat Chapter 10: The problem with the branches Ice storm, Toronto, 2013 Chapter 11: Our balloon gets popped Chapter 12: Central nervous system vasculitis Transcript Chapter eight. The flip. As we walked a few hundred meters, I felt the flip. We were on the other side. I'm used to being the healthy one. The doctor in control. Moving fast from patient to patient, putting out fires, making diagnoses. Prescribing treatments, comforting treating curing. Now, we were on the other side. I was the helpless family member watching a disaster. Personal disaster in slow motion. And Danielle was the patient. The sick. The one who waits to see the doctor. The shit had hit the fan. I called the daycare. And told them to expect the grandparents and that they would be late. Fortunately, we didn't have to wait long. Dr. Wong and my other colleagues came to our aid. We had a CT angiogram, a CT venogram. An echocardiogram. EKG. Blood work. And then it was over. It was time to go home. Danielle was given aspirin and Plavix. And those two drugs, and her little strokes, and baby Micah, and me, we went home. Discharge diagnosis. Stroke in a young person. Everything just came crashing down very suddenly. And it was just this whirlwind of, uh, medical professionals and people didn't know what was going on. And that was very disconcerting. When we got home, it was late. Danielle and Micah went to sleep in the guest room. The other two kids were sleeping at my in-laws and I started a frantic search for what the hell was going on. Not in a textbook and not an up-to-date and not on pub med, but in the dark anxious recesses of my mind. Chapter nine. Snakes got to eat. I heard a baby crying. But it didn't sound like Micah in the other room. It was a disturbed, deranged animal baby. It's years earlier, when I was a kid. We were around the dinner table with my family on vacation and Florida. It's one of those evenings thick with cicadas and there is this terrible crying coming from outside. A baby? All of us, get up from the dinner table to go and see. It's a frog. And it's not even a whole frog. It's half a frog. And it's not on the ground. It's literally suspended in mid air, half on and half off the edge of the roof. You know those two back jumping legs that make a frog, a frog. They are inside the giant gape of a snakes unhinged jaw. It looked so impossible that a tiny snake could open its mouth so wide. It looks like the frog has been chased across the roof and just as it was about to jump. The snake caught it and so now the half of frog, just its face and its front legs, they are break dancing. And they're breakdancing in mid air. It was almost funny. But it wasn't funny. The frog was screaming. It knew it was going to die. I stood quietly watching the frog for its long engulfing until it went quiet, its face still free. It was an awful thing to see. My dad just said, the snakes got to eat. And I had forgotten that snake engulfing you from behind feeling until that night after the MRI. Danielle was sleeping with Micah and the baby's room. I was in the big kid's room on the rocking chair, staring out into the snowy dark. The house was all quiet. And the snake struck. I suddenly realized what was going on. Stroke in a young person. New migraines with aura. Vascular cognitive dysfunction. Cadasil. C A D A S I L. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. I remembered Danielle's mother getting an MRI for headaches a couple of years before. And I remembered the non-specific abnormality in the white matter. And suddenly, it all became so clear. That moment every doctor knows when the long-sought diagnosis crystallizes. "Of course!" you think. How did I not see it before? Danielle comes out of the baby's room. She's groggy. “What's wrong?” she asks me. “I figured it out,” I say. “What?” “Remember, your mom had an MRI for her headaches.” She shakes her head. “She did like last year, maybe, it was full of stuff that I didn't think it was a big deal at the time. But not normal. But that, and the headaches and the strokes. Now it makes sense. It's called CADASIL.” She's quiet, standing in the middle of the room. She says, “I need to finish feeding Micah.” She does not see the black ocean around her. She does not see she is drowned. The kids are drowned. Genetic autosomal dominant. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy or CADASIL is a way of your brain slowly losing its blood supply. Inch by inch. Year by year. Function by function. White scar by white scar on your MRI. And when it has engulfed your wife, then it waits. Let you return to dinner. Waits until your children have reproduced. And then it slithers up inside one of them. From its coiled up hiding. And starts its slow, swallow again. And when it's finished with your son and the screeching has stopped and you are sitting at dinner one day, your daughter says she has a headache. Or her hand is tingling. And you watch again. Nothing you can do. The snakes gotta eat. At some point, I fell asleep next to Danielle and Micah. And when I awoke, I had forgotten it for a moment. It was morning, you know? Those few seconds. And then it's back. I ran downstairs and looked up her mom's MRI. I was wrong. It wasn't an MRI of the head she had had. It was of her inner ears. And it wasn't last year. It was a few years ago and it wasn't for headaches. It was for ringing in the ears. I put my head down on the desk, relieved, but feeling how untethered I had become. Yeah. Forget it. Danielle came down with the baby. And I told her to forget about what I had said last night. But she looked at me. And I could see, she didn't know if I was now trying to backtrack and cover up something terrible I'd let slip, or if she could believe me. She just nodded very passively, probably not knowing which she preferred. Chapter 10. The problem with the branches I went outside to clear my head, and the outside, by which I mean everything not inside, was completely covered in ice. I mean, everything. The front porch. The steps, the driveway, the cars, the sidewalk, the street. The light poles. Yes, even the verticals. It was like someone had literally come and poured water over the entire world and then frozen it. I shuffled slowly down the driveway. A few meters up the street from where we live is a large Mulberry tree that looms over the cemetery wall. Well, its one large branch thick as an elephant's leg was coming off at a weird downward angle. And underneath it, pinned and squished like a bug, totally covered in ice, the neighbors Toyota Rav four. Then the tree limb kept going into the street and block the road. The tree trunk reached up over the cemetery wall. And then two thick as street pole branches, angled up like a Y, and that's where the problem started. From there, the ice had made the twigs easy to break. A gust of wind came up and I literally saw the tree shatter in front of my eyes. A pile of twigs and small branches, totally encased in ice came clank ring down like a pile of coins hitting the ground. What I had not taken into account and the terror of the past 12 hours, the MRI, and then the emergency room. And then my late night nightmare was the other part of the MRI. Not the strokes, but the problem with the branches. The arterial tree. Whoever had taken their dirty little fingers and dabbed five strokes onto Danielle's brain had done something worse. They had gone right up to the main arteries going into the brain, the tops of the internal carotids, where they branch into the anterior and middle cerebral arteries, and they had grabbed a hold of those blood vessels and they had squeezed like that Rav four. Pinned and squished down to nothing. Her brain was running on slivers of blood dental floss sized. In 10 years of neurology practice and training, I had never seen anything quite like it I remember that MRI at the Western and looking at the scan after and realizing how inflamed that vessel was or how small that vessel was and how big of a difference the inflammation makes. Chapter 11. Ninety-nine red balloons floating in the summer sky, panic bells, it's red alert. There's something here from somewhere else. The next day, Danielle went to get a lumbar puncture. At this point, I knew our lucky balloon had been popped. Our bubble burst. I knew we were in trouble. Our years of good luck had sprung a leak. But things were about to get worse. Now, the reason it's called a lumbar puncture is because the lollipop shaped sack of fluid that protects your brain and your spinal cord gets popped and the cerebral spinal fluid that bathes your brain and keeps it nice and floaty and buoyant and protected, it starts to drip, drip out like SAP from a maple tree. And that's spinal fluid that bathes your brain, it should be what we call bland. Boring. Like water. Uh, still not sparkling. Clear. Calm. Danielle spinal fluid was not calm. The white blood cells. The lymphocytes were elevated and the protein too, which meant something in or around the brain was not happy. She had an itis. I've always thought the Latinate suffix itis as in meningitis or encephalitis or appendicitis, it doesn't quite have the same searing heat as the more apt, English translation. Inflamed. The elevated white blood cell count, that was the smoke. We were seeing the smoke and the spinal fluid. The headaches, the cognitive changes, the strokes, her squished down blood vessels. They were the house on fire. She was being officially diagnosed by neurologists who were not her husband. Danielle had central nervous system vasculitis and that, my friends, is not a good diagnosis to have. Our balloon had been popped. But who had started the fire? Where did it come from? Who had attacked her nervous system? We didn't know. She was started on prednisone to quell the fire in her blood vessels. Usually, we doctors are not satisfied with patients having natural God-given problems. And so along the course of the patient's illness, we're prone to give them doctor-created problems. Iatrogenic complications like little party favors. Thanks for coming with your autoimmune CNS vasculitis. Here's a little more suffering for you. Compliments of Dr. So-and-so. For Danielle, that Iatrogenic party favor was something in the form of a new balloon. In her words, here this time in her head. And then what happened from the lumbar puncture? Me feeling really sick that night in the shower and throwing up. I couldn't stand it, sick or being in hospital before, um, you know, at least you can sit up and read or watch TV or call someone on the phone or something. But this was like, every time I lifted my head, I would feel, I would get a headache or just feel nauseous, so I had to basically lie down. Otherwise, I would get a headache. It was very discombobulating. I would get not dizzy, but just like, as like, as if I had a really bad sinus cold or my head was full of pressure like a balloon, a balloon. Chapter 12. Central nervous system terrorism. Home grown. We moved in with my in-laws and I set up shop at their living room table with my computer. And paper after paper, review articles on CNS vasculitis, I began to read everything I could get my hands on Primary CNS vasculitis is not good. It's an auto-immune disease of unknown cause it doesn't kill that many people or disable that many people, but that's not because it's not awful, but because it's rare. In five years of residency, and at the time, six years of neurology practice I had maybe seen it a handful of times before. And now, my wife had it? Sometimes very rare infections can mimic CNS vasculitis and Danielle's excellent doctors had checked for these. Gram stain and culture for bacteria. Fungal cultures. Viral pcr viral cultures. But everything came back negative. There was no sign of infection and she had no other systemic illness, no rash, no joint pain. There was no sign of inflammation anywhere else in the body. It's hard to explain how severely narrowed the arteries were. Going to Danielle's brain. The confluence of the circle of Willis, where the arteries meet each other at the top. Was interrupted by areas. Where there was no blood flow at all. And so her dominant hemisphere was being fed by a left MCA artery that kept getting narrower and narrower on each success of scan. One night, out of her earshot, I sat on the edge of Danielle's parents' bed. And I tried to explain to them. That Danielle. Might not make it through this. Or worse might have a catastrophic stroke. Leaving her paralyzed. And unable to speak. It's hard to explain the terror that we were living with. Waiting for a bomb to go off, but you can't diffuse it because you don't know where it's planted. And you don't know why it's there and you don't know who put it there. CNS vasculitis home grown terrorism. I hated this diagnosis. Deeper Dive Calabrese, L., Mallek, J. (1987). Primary Angiitis of the Central Nervous System: Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine, 67(1), 20-39. Chabriat, H., Joutel, A., Dichgans, M., Tournier-Lasserve, E., Bousser, M. (2009). CADASIL. The Lancet Neurology, 8, 643-653. Gonzalez, R., Schaefer, P., Buonanno, F., Schwamm, L., Budzik, R. Rordorf, G., Wang, B., Sorensen, A., Koroshetz, W. (1999). Diffusion-weighted MR imaging: Diagnostic accuracy in patients imaged within 6 hours of stroke symptom onset. Radiology, 210(1), 155-162. Kley, N. (2001). Prey Transport Mechanisms in Blindsnakes and the Evolution of Unilateral Feeding Systems in Snakes. American Zoologist. 41: 1321-1337. Paul, S., Roy, D., Mondal, P., Bhattacharyya, R., Ghosh, C., Das, S., Krishna, H., Patra, C., Kiran, J., Benito-León, J. (2022). Primary angiitis of the central nervous system – A challenging diagnosis. Journal of Neuroimmunology, 366, 577844. Raskin, N. (1990). Lumbar Puncture Headache: A Review. Headache: The Journal of Head and Face Pain, 30(4), 197-200. Warach, S., Dashe, J., Edelman, R. (1996). Clinical outcome in ischemic stroke predicted by early diffusion-weighted and perfusion magnetic resonance imaging: A preliminary analysis. Journal of Cerebral Blood Flow and Metabolism, 16(1), 53-59. Yuh, W., Crain, M., Loes D., Green, G., Ryals, T., Sato, Y. (1991). American Journal of Neuroradiology, 12, 621-629.…

1
E1: A terrorist without a belt

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Part 1: A series of not-so-great diagnoses Wherein my wife and my luck runs out, a terrorist with an invisible belt comes for her, and I make a series of not-so-great diagnoses. Also, a geography lesson, some poetry, and … we don’t take American Express.

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E1: A terrorist without a belt

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E1: A terrorist without a belt

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E1: A terrorist without a belt

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