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Eight Stories of Locked-In Syndrome

Imagine losing control over everything. You can't move on your own. You can't scratch an itch. And worse still, you can't tell anyone around you that you have an itch. You can feel pain, hunger, loneliness, and fear, but you can't react to those sensations. You are totally aware of your surroundings, but you can't communicate your feelings or desires, or even your basic needs. The term for this horror is locked-in syndrome. Someone with locked-in syndrome suffers from paralysis of all voluntary muscles except for those that move the eyes. This can be caused by neurological disease such as ALS, strokes, injuries to the brain stem, or drug overdose. The term locked-in syndrome has only been in use since the 1960s. Before that, people who suffered such traumatic injuries generally died from them, or else they were considered to be brain damaged. Here are the stories of eight people who have lived this nightmare.

Julia Tavalaro

One of the dangers of locked in syndrome is the possibility of misdiagnosis. No one knows how many people lived for years and died unable to tell anyone around them that they were conscious and aware. Julia Tavalaro was a 27-year-old housewife and mother in 1967 when she suffered multiple strokes that left her completely paralyzed. Doctors thought she was brain dead. Tavalaro was sent to a custodial institution where she lived for six years without anyone knowing she was aware of her surroundings. She was fed through a tube and cared for physically, but had no real interaction with her caretakers. In 1973, a speech therapist noticed Tavalaro's eyes moving in reaction to her words. Afterward, Tavalaro was given physical therapy and an opportunity to communicate, first by a letter board, then later by operating a computer by tapping a switch with her cheek. She also learned to control her wheelchair by head movements. Tavalaro wrote a book about her experiences, Look Up For Yes, and became a renowned poet. She died in 2003, at the age of 68.

Nick Chisholm

nick.jpgNew Zealander Nick Chisholm was 23 when he had a rugby accident in 2000. A resulting series of strokes left him paralyzed and unable to communicate. For the first three months, he couldn't even open his eyes. During this time, he was fully conscious and heard medical personnel discussing his imminent death and asking his mother if she wanted to remove life support. After several months, his mother and girlfriend convinced doctors that he was aware and thinking. Chisholm could move his eyes, and communicated by staring at letters on a letter board. His story is told in his own words, with medical explanations added.

Words can't describe the situation I have been left in—but this is as close as I can get it: an extremely horrific experience that I wouldn't wish on my worst enemy.

When you're like this (despite having 24 hour care) it's an incredibly lonely existence at times. It's amazing how much time I have to think about things now since the accident. There's heaps of thoughts that I don't bother even expressing.

Chisholm writes about contemplating suicide, although he didn't have the ability to carry it out. He has since regained some movement, and can pronounce some words. Chisholm is working toward a full recovery.

Bob Veilette

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Bob Veilette was a Connecticut journalist and an accomplished jazz pianist in 2006 when a stroke left him with locked-in syndrome. After six months in a hospital, his family elected to care for him at home instead of putting him in a nursing home. That decision meant that Connecticut's Medicaid program would not cover the costs of his care, even though a nursing home would have been much more expensive. Veilette joined a movement to reform the system, although the changes they are working for will not benefit him personally for years. Private fundraisers support the family, but Veilette has had no speech or occupational therapy, and only limited physical therapy due to expenses. Veilette communicates with a letter board held by an assistant. He had no luck with a computer that tracks eye movements, because fluctuations in the size of his pupils upset the tracking.

Catherine O'Leary

200catherine-oleary.jpg31-year-old Catherine O'Leary suffered from hiccups for three years before the cause was discovered to be a brain tumor. During surgery to remove the tumor, a series of strokes left her completely paralyzed. She communicates by blinking; one blink for yes, two blinks for no. Eight months after the surgery, she can move her facial muscles somewhat, but still cannot speak due to a tracheotomy that allows her to breathe. Her brother is working to raise enough money to send her to America for medical treatment.

Jean-Dominique Bauby

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French journalist Jean-Dominique Bauby was 43 when he suffered a massive stroke in 1995. He was left with only the ability to blink his left eye. Bauby died less than two years later, but managed to write his memoir by waiting for an assistant to recite the French alphabet. He would blink when the right letter was pronounced. Using this method, he had to construct and edit each sentence and chapter in his head. The resulting book was The Diving Bell and The Butterfly (Le scaphandre et le papillon). Bauby died of pneumonia only ten days after the book was published. It was made into a film released in 2007.

Gary Griffin

Air Force veteran Gary Griffin is immobile due to ALS (Lou Gehrig's disease). He uses a device called the NeuroSwitch to control a computer and communicate with his family. Sensors are attached to the skin over a patient's muscles and signals are sent to an interface that translates the slightest muscle contractions into usable code. The company's website says even muscles that are considered non-functional may be used.

Johnnie Ray

johnnyray.gifAdvances in assistive technology gives new hope to those with disabilities, including locked-in syndrome. Johnny Ray suffered a brain stem stroke in 1997 that left him unable to move. In 1998, he volunteered for an experimental procedure developed by by Philip Kennedy, Roy Bakay, and the team at the Neural Interfacing Research Institute. Sensors were implanted in Ray's brain that allowed him to move a cursor on a computer with his thoughts.

"We simply make a hole in the skull right above the ear, near the back end of the motor cortex, secure our electrodes and other hardware to the bone so they don't migrate, and wait for a signal," Bakay says. The implant is an intriguing hybrid of electronics and biology - it physically melds with brain tissue.

After implantation, the research team calibrated the interface by asking ray to think about certain movements, and the software was programmed to respond to such signals. Ray used the cursor to spell words and even generate musical tones on a computer.

Erik Ramsey

erik-ramsey.jpgDr. Kennedy is taking that technology a step further for his latest patient. Erik Ramsey was seriously injured in a car wreck in 1999 when he was 16 years old. A blood clot lodged in his brain stem and caused a stroke. Erik lost all voluntary muscle function, except for the ability to move his eyes up and down. He learned to communicate with his family using a letter board. In 2001, he inexplicably lost the ability to spell when he was hospitalized for pneumonia. Since then, he communicates only by rolling his eyes up for yes, and down for no. In 2004, Dr. Kennedy and his team implanted sensors in Ramsey's brain that can convert thoughts into speech. So far, he can think of the movements that produce vowel sounds and the computer recreates those sounds. The team hopes to program the interface to achieve consonant sounds within a year.

Links to resources on locked-in syndrome.

This article was inspired by a post at Metafilter.

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Health
The First Shot to Stop Chronic Migraines Just Secured FDA Approval
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Migraine sufferers unhappy with current treatments will soon have a new option to consider. Aimovig, a monthly shot, just received approval from the Food and Drug Administration and is now eligible for sale, CBS News reports. The shot is the first FDA-approved drug of its kind designed to stop migraines before they start and prevent them over the long term.

As Mental Floss reported back in February before the drug was cleared, the new therapy is designed to tackle a key component of migraine pain. Past studies have shown that levels of a protein called calcitonin gene–related peptide (CGRP) spike in chronic sufferers when they're experiencing the splitting headaches. In clinical trials, patients injected with the CGRP-blocking medicine in Aimovig saw their monthly migraine episodes cut in half (from eight a month to just four). Some subjects reported no migraines at all in the month after receiving the shot.

Researchers have only recently begun to untangle the mysteries of chronic migraine treatment. Until this point, some of the best options patients had were medications that weren't even developed to treat the condition, like antidepressants, epilepsy drugs, and Botox. In addition to yielding spotty results, many of these treatments also come with severe side effects. The most serious side effects observed in the Aimovig studies were colds and respiratory infections.

Monthly Aimovig shots will cost $6900 a year without insurance. Now that the drug has been approved, a flood of competitors will likely follow: This year alone, three similar shots are expected to receive FDA clearance.

[h/t CBS News]

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The 98.6℉ Myth: Why Everything You Think You Know About Body Temperature Is a Lie
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When you were kid, you probably knew that to score a magical sick day home from school, you needed to have a fever. When the thermometer came out of your mouth, it had to read higher than 98.6℉—the long-accepted "normal" human body temperature. (If you wanted to really seal the deal, you may have hoped to hit 100℉.) Since then, you may have used a temperature above 98.6℉ as a metric to work from home (or call out sick entirely).

But here's the thing: The average body temperature isn't actually 98.6℉—a fact that we've known for more than 25 years. The myth originated in the 19th century with a single doctor, and despite evidence to the contrary, it's persisted ever since.

THE GIANT—AND FAULTY—ARMPIT THERMOMETER

In 1851, Carl Wunderlich, the director of the hospital at Leipzig University, began going from room to room with a comically large thermometer in tow. He wanted to understand how body temperature is affected by different diseases, so in each room, he would hold the foot-long device in patients' armpits for a full 20 minutes, waiting for a temperature to register. Once it did, he'd note the temperature on the patient's chart (Wunderlich is thought to be the first physician to do so). He and his staff did this for years, repeatedly taking the temperatures of some 25,000 patients and logging them on their charts, until he had millions of readings. In 1868, he finally published this data in Das Verhalten der Eigenwarme in Krankheiten (On the Temperature in Diseases: A Manual of Medical Thermometry). He concluded that the average human body temperature was 98.6℉, underscoring the idea that fever is a symptom of illness, not a cause.

No one questioned Wunderlich's methods, or his average, for about 140 years. Then, in the early 1990s, internist Philip Mackowiak—a professor of medicine at the University of Maryland, a medical historian, and, apparently, a clinical thermometer junkie—saw one of the physician's instruments at the Mutter Museum in Philadelphia. He told the Freakonomics podcast that he'd always had doubts about the 98.6℉ standard. "I am by nature a skeptic," he said. "And it occurred to me very early in my career that this idea that 98.6 was normal, and then if you didn't have a temperature of 98.6, you were somehow abnormal, just didn't sit right."

Getting his hands on Wunderlich's thermometer—which the museum let him borrow—only deepened his doubts. The huge thermometer was unwieldy and non-registering, meaning, Mackowiak explained, "that it has to be read while it's in place." Not only that, but Wunderlich had used the device to measure temperatures in the armpit, which is less reliable than temperatures taken in the mouth or rectum. The instrument itself also wasn't terribly precise: It measured up to 2 degrees Centigrade higher than both ancient and modern instruments.

In 1992, Mackowiak decided to test Wunderlich's average. Using normal-sized oral thermometers and a group of volunteers, he determined that the average human body temperature actually hovers around 98.2℉. Mackowiak found that body temperature tends to vary over the course of the day, with its lowest point around 6 a.m. and its highest in the early evening. Body temperature can also fluctuate monthly (with the menstrual cycle) and over a lifetime (declining decade by decade with age), and may even be differentially linked to sex and race assignments. He concluded that normal body temperature is so unique to each person that it's almost like a fingerprint and, given that wide variation, not actually a very reliable indicator of illness.

As a result of his study, Mackowiak proposed raising the threshold for fever to 98.9℉ for temperatures taken in the morning (and 99.9℉ at other times). While it's a relatively minor change in terms of actual degrees, this fever threshold is actually lower than the CDC's, which is a temperature of 100.4℉ or higher.

There are potential real-life consequences in this gap, for everyone from students (who'd have to attend school with what would be considered a low-grade fever by Wunderlich's 98.6℉ standard) to employers and daycares (who use temperature to set attendance policies). What's more, anyone who is actually sick but ignores a low-grade fever—one that meets Mackowiak's threshold but still falls under the CDC's—could pose a risk to people with compromised immune systems trying to avoid unnecessary exposure to illness in public places.

THE BALANCING POINT

There's a reason the average trends near 98℉ instead of 92℉ or 106℉. As endotherms, mammals expend a great deal of energy maintaining body temperature when compared with cold-blooded creatures. To find and conserve a just-right body temperature, central nervous system sensors gather data (too warm? too cold? just right, Goldilocks?) and send that information to the pebble-sized hypothalamus near the base of the brain. There, the data is converted into action: releasing sweat and widening the blood vessels if too warm; raising metabolism, constricting the blood vessels, and inducing shivering if too cold.

According to a study by Aviv Bergman and Arturo Casadevall in the journal mBio, the precise balancing point for ideal body temperature is the sweet spot where the metabolic cost for all this thermoregulation balances with the evolutionary advantage of warding off fungal disease. (While warm-blooded animals are prone to bacterial or viral infections, they rarely experience fungal infections because most fungi can't withstand temperatures above 86℉. Cold-blooded animals, on the other hand, are prone to all three.) For Bergman and Casadevall, this benefit even explains what tipped Darwin's scales in favor of mammals, allowing them to edge out other vertebrates for dominance after the Cretaceous-Tertiary mass extinction wiped out the dinosaurs.

Of course, rules call for exceptions, and the one place where human body temperature demonstrates sustained elevation is outer space. Astronauts on prolonged missions clock significantly higher average body temperatures than they do when terrestrial—even up to 104℉. This so-called "space fever" is probably a product of some combination of radiation exposure, psychological stress, and immune response to weightlessness. Researchers believe this phenomenon could yield crucial information about thermoregulation—and may even offer insight into how humans might adapt to climate change.

WHY THE MYTH PERSISTS

It's been 26 years since Mackowiak's study, yet the newer data has not taken hold among medical professionals or the public. What gives?

Mackowiak tells Mental Floss that he finds it a bit mystifying that the myth persists, especially since many people, when pressed, know that the so-called "average" temperature varies. Part of the problem may be psychological: We cling to beliefs despite evidence to the contrary—a phenomenon called belief perseverance [PDF]. It's a significant force upholding a surprising number of medical myths. The idea humans should drink eight glasses of water a day? Not science. Sugar causes hyperactive behavior? Nope. Reading in dim light harms eyesight? Not really.

Unlearning persistent myths—especially ones loaded with the weight of medical authority—is difficult. "Deep down, under it all," Mackowiak says, "people want simple answers for things."

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