Transcription

• Speaker #0

  This is Case Encounters, a journey to the true medical mysteries solved by pathologists. Like Robert Allen, who thought his age was simply catching up with him. Little did he know.

• Speaker #1

  Consider the maze of our identities. Each thought, each memory, each interaction. All steps through a labyrinth creating our sense of self. But what happens when the maze unfolds on itself, or every path leads to an unexpected dead end? If we can never really know what's around any corner, can we ever confidently know who we are?

• Speaker #2

  Morning, Mr. and Mrs. Allen. I'm Dr. Rao. Hope you don't mind I've brought a resident in with me today. This is Dr. Hayes. What brings you two in today?

• Speaker #3

  Ah, nice to meet you, Dr. Hayes. Well, Dr. Rao, I'm not sure if I'm just getting old or if something's happened. My memory's been slipping for some time now, but somehow in the last three months, it's gotten much worse. It started with little slips, forgetting where I put my keys, losing my place when I'm talking to someone. We all do that sometimes, but, well... I found my shoes in the freezer the other day, and yesterday I got lost on the way home from the library.

• Speaker #4

  We're all a bit worried. He developed a slur when he talks sometimes, his hands are shaky, and he keeps losing his balance. He also started having these spasms, suddenly jerking when he's just sitting watching a show. Could he have had a stroke?

• Speaker #2

  Hmm. Is there a history of stroke, heart disease, or dementia in your family, Robert?

• Speaker #3

  My Uncle Gord had dementia pretty young, I think, and an aunt who had a heart attack.

• Speaker #2

  Okay. Let's see how your coordination is. Can you hold your arm straight out in front of you for me? Great. Now, can you touch your nose with your right hand? Hmm.

• Speaker #5

  Your coordination's a bit off, Mr. Allen. Your reflexes seem overreactive, and you're a bit unsteady.

• Speaker #2

  This could be caused by a number of things, Robert. I'd like to admit you, so we can monitor you and run a few tests. We'll need a CT scan and MRI so we can see what's going on. I'll also order an EEG to check your brain activity.

• Speaker #3

  Okay, Dr. Rao. I just feel like I'm losing myself, like my body's got a mind of its own.

• Speaker #2

  I hear you, Robert. We'll do our best to get to the bottom of this, okay? A nurse will come in to do some paperwork and get you admitted.

• Speaker #1

  The brain is the seat of the self, the great archive of our memories, the stage where identity performs, and the guide to all our actions. But when its circuits falter, who do we become? Robert Allen, as he knows himself and as his family knows him. is slipping through the cracks of his own mind. Even familiar paths become unknown, and routine actions betray him.

• Speaker #2

  Hi, Robert. Hi, Amelia. How are you doing today?

• Speaker #3

  I slept okay, but would really like to get myself home.

• Speaker #2

  I'm sure you would. We'll get you on your way as soon as we can. But first, let's explain what we're seeing in the tests we ran. First of all, the CT scan came back normal. No signs of stroke, no bleeding, no tumor.

• Speaker #4

  And the others?

• Speaker #2

  Well, that's where we have some concerns. Your EEG shows some abnormal brain activity. Normally, we see a pattern that looks like a rolling wave, smooth like water. Robert's looks like sharp peaks, sudden bursts, repeating about once a second. And the MRI is a bit concerning as well. Dr. Hayes, do you want to explain this to Robert and Amelia?

• Speaker #5

  Sure, Dr. Rao. Normally, the outer layer of the brain, the cortex, looks uniform. Your MRI shows the outline of the cortex lighting up. which signals that the neurons there are being injured or disrupted. We're also seeing that the deeper part of your brain that controls movement and coordination is also damaged.

• Speaker #3

  My brain's injured? But how? I haven't fallen or anything. Is it cancer?

• Speaker #2

  We're not exactly sure why or how, but we'd like to rule some things out. The best way for us to do that is with a brain biopsy.

• Speaker #4

  A biopsy? What are you ruling out?

• Speaker #5

  It's a small procedure. A neurosurgeon will do the biopsy using Robert's scans, like a map, to guide a tiny needle into the area where we're seeing the problem and remove just a sliver of tissue, thinner than a strand of baby hair. That tissue will tell us if this is an infection, inflammation, or something else.

• Speaker #2

  That's right. We need to rule some things out. And the biopsy will tell us what we're dealing with. Is that okay, Robert?

• Speaker #3

  I suppose if it will keep me from putting my shoes in the freezer, we should probably do it.

• Speaker #2

  Okay, we'll get it scheduled as soon as we can.

• Speaker #5

  Dr. Rao, I'd love to follow this through pathology if that's okay with you. Dr. Zhang is in this week, and I'd like to see this case from the inside out.

• Speaker #2

  Sure thing, Dr. Hayes. Stay with it.

• Speaker #1

  Imagine a slice of tissue. thinner than a strand of baby hair, contains the answers to Robert Allen's symptoms, and holds the entire weight of his identity. It raises the question, if our self unravels, what remains of who we think we are?

• Speaker #6

  Good morning, Dr. Hayes. Nice to see you weren't scared off by our last case together.

• Speaker #5

  Not at all. Dr. Rao was kind enough to let me split my service so I could see this one through.

• Speaker #6

  Super. Okay. We've got Mr. Allen's scan slides from the National Pre-On Disease Pathology Surveillance Center. But before we look at them, tell me what you'd expect to see in a normal brain biopsy.

• Speaker #5

  Right. I'd see neurons tightly packed in the gray matter, supporting glial cells around them. The tissue should look smooth with no gaps or distortion.

• Speaker #6

  Exactly. Organized. Solid. Without voids. Now, tell me what you see in Mr. Allen's scans.

• Speaker #5

  There are holes. Empty spaces. Everywhere.

• Speaker #6

  That's right. Spongiform change. Tell me what it means.

• Speaker #5

  His brain tissue is breaking down into a sponge. Significantly degenerating. And there's no inflammation. We can rule out infection. Or autoimmune disease. This looks like a classic prion disease.

• Speaker #6

  Tell me what you know about it.

• Speaker #5

  Oh, wow. It's a degenerative brain disorder that's super rare and unstoppable, sadly. It can be hereditary, like this one appears to be, or mad cow disease. Basically, a normal protein changes into an abnormal shape, and that one change spreads, and other normal proteins in the brain change, too, spreading the damage. Over time, they build up and destroy brain cells, leading to memory loss, confusion, movement problems, and eventually, death.

• Speaker #6

  That's right. Creutzfeldt-Jakob disease. Sadly, Hayes, we don't have a cure for this one. At least not yet. His chances of getting this are about as rare as being struck by lightning.

• Speaker #5

  Wow. He mentioned that he felt like he didn't know himself anymore. I guess that's actually kind of true. He also mentioned an uncle with early dementia. Okay, Dr. Zeng. Dr. Rao and I had better go talk to the Allens.

• Speaker #6

  Really sorry you have to deliver this news, Ace. Let me know how it goes.

• Speaker #1

  Not every maze can be solved. Some lead only to walls that close in tighter the further you walk on any path. For Robert Allen, the path through a once familiar labyrinth of memory and identity. will become less a place of comfort and more one of uncertainty and disappearance, where what he once knew will become strange, and his self will slip away long before his body.

• Speaker #3

  Hey there, Dr. Rao, Dr. Hayes. Do you finally have some news for us?

• Speaker #2

  We do, Robert. I know it can feel like forever to get these results back. And unfortunately, we have difficult news for you. Your biopsy results show a rare degenerative brain disorder called Kreutz-Farber. Phil Yockup disease. It explains all of the symptoms you've been experiencing.

• Speaker #4

  How do we treat it?

• Speaker #2

  This is the difficult part. I wish I could say there was a treatment, Mrs. Allen. I'm really sorry to tell you right now there isn't a way to slow or reverse it.

• Speaker #3

  What are you saying, Dr. Rao?

• Speaker #2

  I'm saying we'll do everything we can to manage the symptoms you've been experiencing. The jerking movements, agitation, trouble sleeping, and keep you as comfortable as we can. But I have to prepare you both for a decline in Robert's health over the coming year.

• Speaker #4

  The coming year? What happens now?

• Speaker #2

  I'd like to focus on getting Robert comfortable and stable. We'll start him on a treatment plan to get his symptoms under control and help him get some better sleep. We'll see how you respond and monitor any changes in your condition. This isn't easy news to hear, Robert. You and Amelia take some time. I'll get the orders in for those medications and we'll be right back in.

• Speaker #6

  How did it go, Hayes?

• Speaker #5

  Hard to sit in that room, knowing what we know and having nothing more to offer.

• Speaker #6

  Sometimes that's the work, Hayes. We don't always have cures. We see the loss from a distance, which doesn't make it any less tragic.

• Speaker #5

  Do you ever get used to it? Discovering results like this, knowing we can't do anything about it?

• Speaker #6

  Nope. And I hope I never do. Getting used to it would mean I've stopped seeing that there are living, breathing humans on the other side of my lab.

• Speaker #1

  The self is the most fragile possession we have. A lattice of memory, motion, meaning. Robert Allen's self unraveled in silence, undone by a single, unavoidable, natural betrayal. One that, while identified, could not be undone. His story reminds us that, as vigilant as we remain, there are always shadows lurking along the pathways of our lives, and of ourselves.

• Speaker #0

  You've been listening. Case Encounters. This story is inspired by a true medical mystery solved through collaboration, curiosity, and a pathologist. Names and locations are fictitious. Till next time, stay curious. The voices you just heard are those real pathologists. People who work long days and longer weeks to solve medical mysteries big and small. To learn more about the work pathologists do for patients, visit yourpathologist.org. For a full list of the pathologists featured in this episode, those who advised the creation of this story, please visit the show notes. Case Encounters is a production of the College of American Pathologists. Creative support, Studio North. Produced and directed by Natalie Gregory. Sound design, editing, and original music by Jake Sorgen. Written by Paige Freeborn for Studio North.