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Kimberly Akimbo, a new Broadway musical, won five Tony Awards on Sunday night, including “Best Musical”. The show, by David Lindsay-Abaire, focuses on Kim, a girl with a fictional disease that causes her to age at over four times the normal rate. At sixteen years old, she appears to be a 72-year-old woman. Is this a medical reality? 

Kim’s disease is unnamed, but we get a description of it in the song “Our Disease”: It’s “an incredibly rare genetic disorder / In which several signs of aging are manifested at a very early age” with symptoms including “wrinkled skin, stiff joints, hip dislocation, atherosclerosis, molecular degeneration, hypertension, presbycusis, [and] cardiac issues”. The song also states that “there is no cure” and that “few people with [the] disease / live longer than sixteen years.”

This disease has many similarities to a real disease called progeria. Like Kim’s disease, progeria (also called Hutchinson-Gilford syndrome) is a rare genetic disorder that causes rapid aging of the body. It is estimated to affect 1 in 20 million people. Children with progeria will typically start showing signs of the disease in the first few months of life; early symptoms include failure to thrive, stunted growth, and alopecia. As the disease progresses, individuals may display many of the symptoms mentioned in the song: wrinkled skin, atherosclerosis, cardiac issues, and musculoskeletal degeneration causing stiff joints and hip dislocation. Other symptoms can include kidney failure, loss of eyesight, and scleroderma.

One major difference between the real and fictional diseases is in the phenotypic features. Kim is a teenager who looks like a middle-aged woman, which is why she’s portrayed by Victoria Clark, an actress in her 60s. Individuals with progeria, on the other hand, have a distinctive appearance that includes a small body, narrow face, prominent eyes, small lower jaw, and prominent veins on their scalp (visible due to alopecia).

There is no known cure for either progeria or for Kim’s disease. The average life expectancy for those with progeria is around fifteen years. Even though Kim knows that her life might end soon, she retains a positive attitude. As the musical reminds us in the final song, “Great Adventure”: “Just enjoy the ride / Because no one gets a second time around.”

References:
Lamis A, Siddiqui SW, Ashok T, Patni N, Fatima M, Aneef AN. Hutchinson-Gilford Progeria Syndrome: A Literature Review. Cureus. 2022 Aug 31;14(8):e28629. doi: 10.7759/cureus.28629. PMID: 36196312; PMCID: PMC9524302.

Water being poured into a glass
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Warning: This post tells the tragic true story of a woman’s death.

On January 12, 2007, a Sacramento radio station called KDND aired its morning show, The Morning Rave. That day, the show held an on-air contest called “Hold Your Wee for a Wii”, in which around 20 contestants competed to win a Wii console by drinking as much water as they could without urinating.

As the contest was occurring, the hosts – Adam “Lukas” Cox, Steve Maney, and Patricia “Trish” Sweet – joked about the contest. 

Sweet: “Can’t you get water poisoning and, like, die?”

Cox: “Your body is 98% water. Why can’t you take in as much water as you want?”

A listener then called into the station to inform the hosts that the contest could have dangerous, even fatal consequences. 

Caller: “I want to say that… those people that are drinking all that water can get sick and possibly die from water intoxication.”

Cox: “Yeah, we’re aware of that.”

Maney: “They signed releases so we’re not responsible so it’s okay.”

One contestant was 28-year-old Jennifer Strange. She drank approximately 2 gallons, or 7.6 liters, of water in 3 hours (Hochelaga, 2020). During the contest, Strange complained of a headache, and the hosts made fun of her distended stomach. After the contest, Strange called a friend and said she had an intense headache. Only a few hours later, Strange was dead. She had died from acute water intoxication.

Hew-Butler et al. (2019) explain what happens when a person becomes overhydrated:

When modest amounts of water (or other hypotonic fluids) are ingested above osmotically-driven thirst stimulation (overhydration), osmoreceptors located within the highly vascularized circumventricular organs (CVO’s) within the brain detect a (dilutional) decrease in plasma [Na+] once water is absorbed into the circulation from the gastrointestinal (GI) tract. These CVO’s, located outside of the blood brain barrier, suppress both the release of the body’s main anti-diuretic hormone, arginine vasopressin (AVP), from the posterior pituitary gland and suppress the sensation of osmotically-driven thirst to prevent further dilution of plasma [Na+]. Oropharyngeal receptors, activated by physical contact with ingested fluids, as well as gastrointestinal sensors responding to stretch receptors sensing fullness serve to terminate drinking behavior, perhaps as an anticipatory measure to prevent the pathophysiological consequences of overdrinking (i.e., cellular swelling).

Overhydration, particularly when not accompanied by polyuria (excess urination) can result in the dilation of the bladder, ureters, kidney, and stomach. The most extreme consequence is water intoxication. 

Water intoxication results from an electrolyte imbalance defined as plasma sodium concentration < 135 mmol/L. This excess of total body water relative to extracellular sodium is called hyponatremia. Symptoms of hyponatremia include nausea, blurred vision, tremors, dizziness, lethargy, and seizures (Kotagiri et al., 2022). A recent systematic review analyzed case reports of hyponatremia and found that in 53% of the cases under consideration, the symptoms were severe, including seizures and coma (Rangan et al., 2021). In patients with severe symptoms of hyponatremia, practice guidelines recommend an IV infusion of 100 mL of 3% NaCl over 10 minutes, repeated twice if necessary (Verbalis et al., 2013). Hyponatremia causes swelling of the brain, and if cerebral edema exceeds the physical limits of the skull, it can lead to brain herniation, cerebral hypoxia, and, finally – as in Strange’s case – death (Hew-Butler et al., 2019). 

Directly following Strange’s death, KDND discontinued the Morning Rave program and fired ten station employees, including the three DJs who had hosted the program. Strange’s family issued a wrongful death lawsuit on January 18 against Entercom, the company which owned KDND. The wrongful death case began in September 2009. On October 29, 2009, the jury awarded Strange’s family $16,577,118 in monetary damages and found Entercom Sacramento LLC to be 100% at fault for Strange’s death.

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References

Hew-Butler T, Smith-Hale V, Pollard-McGrandy A, VanSumeren M. Of Mice and Men-The Physiology, Psychology, and Pathology of Overhydration. Nutrients. 2019 Jul 7;11(7):1539. doi: 10.3390/nu11071539. PMID: 31284689; PMCID: PMC6682940. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6682940/

Hochelaga. Death on the Radio [Video]. YouTube. June 10, 2020. https://www.youtube.com/watch?v=a_qoQZYdjSE 

Kotagiri R, Kutti Sridharan G. Primary Polydipsia. 2022 Apr 30. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 32965922. https://www.ncbi.nlm.nih.gov/books/NBK562251/

Rangan GK, Dorani N, Zhang MM, Abu-Zarour L, Lau HC, Munt A, Chandra AN, Saravanabavan S, Rangan A, Zhang JQJ, Howell M, Wong AT. Clinical characteristics and outcomes of hyponatraemia associated with oral water intake in adults: a systematic review. BMJ Open. 2021 Dec 9;11(12):e046539. doi: 10.1136/bmjopen-2020-046539. PMID: 34887267; PMCID: PMC8663108. https://bmjopen.bmj.com/content/11/12/e046539.long

Verbalis JG, Goldsmith SR, Greenberg A, Korzelius C, Schrier RW, Sterns RH, Thompson CJ. Diagnosis, evaluation, and treatment of hyponatremia: expert panel recommendations. Am J Med. 2013 Oct;126(10 Suppl 1):S1-42. doi: 10.1016/j.amjmed.2013.07.006. PMID: 24074529. https://www.sciencedirect.com/science/article/pii/S0002934313006050?via%3Dihub

 

Page of a dictionary
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In today’s blog post, we investigate the histories behind some of the health-related phrases we use every day.

Snake Oil 

Snake oil refers to “a substance or product sold as a remedy for a disease or medical condition but which has no real medicinal value” (OED). But in the 19th century, snake oil was a legitimate medical product which Chinese immigrants introduced to Americans to help reduce inflammation. One explanation for why this works is that snake oil is high in omega-3 fatty acids, particularly eicosapentaenoic acid (EPA) (Kunin 208). Traditional snake oil was made from Chinese water snakes, but because that species isn’t native to the U.S., some American businessmen claimed they were selling the Chinese product when they were actually using oil from rattlesnakes, which was less effective, possibly due to its lower concentration of EPA.

One of those businessmen was named Clark Stanley, and he marketed Stanley’s Snake Oil. But it wasn’t just the use of rattlesnake oil that made Stanley’s product fraudulent; a 1917 investigation found that Stanley’s Snake Oil actually did not contain any snake oil at all, rattlesnake or not! Now, the phrase “snake oil” is used to refer to any fraudulent medical products, forever besmirching the traditional Chinese medicine instead of the American charlatans who profited off of it.

An Apple a Day Keeps the Doctor Away

Sure, it’s not too surprising that eating apples helps you stay healthy, but how long have people actually been saying so? The February 1866 edition of the journal Notes and Queries mentioned a proverb spoken in Pembrokeshire, a county in Wales: “Eat an apple on going to bed, And you'll keep the doctor from earning his bread.” That’s the earliest attestation of the phrase in any form, and it appears that by the late 19th or early 20th century, the phrase had been solidified into the form we know today.

A 2015 study researched the truth of this adage. The article concluded that “evidence does not support that an apple a day keeps the doctor away; however, the small fraction of US adults who eat an apple a day do appear to use fewer prescription medications.”

Laughter is the Best Medicine

The exact origin of the phrase is unknown, but the idea has been around for quite a while. Proverbs 17:22 of the King James Bible says: “A cheerful heart is good medicine / but a crushed spirit dries up the bones.” And this isn’t a purely metaphorical statement. Simply search for the phrase on PubMed, and you will find that many studies have shown the positive healing effects of laughter. (It seems almost inevitable that if a medical article concerns the effects of laughter, the authors will use this phrase in the title.)

Under the Weather

This idiom almost certainly originated in the nautical world, although theories abound as to how exactly the phrase came to be. The most popular theory posits that when a sailor felt ill or seasick, he would retire under the weather bow of the ship (i.e. the windward-facing side) so as to prevent potential exposure to bad weather. Thus the sick sailor would be “under the weather”. 

A Spoonful of Sugar Helps the Medicine Go Down

You might have thought it had older origins, but this pithy phrase was invented for the 1964 movie Mary Poppins. Julie Andrews had not yet committed to the main role and had mentioned to Walt Disney that she did not like one of the songs. Disney really wanted Andrews for the part and so he instructed the songwriters, brothers Robert and Richard Sherman, to come up with a more catchy song to replace the original. 

The story goes that Robert Sherman arrived home from work a few days later, and his wife told him that their children had gotten their polio vaccine that day. He asked his son if the shot hurt. The boy responded that it was not a shot; rather, the medicine was placed on a sugar cube which he swallowed. Inspired by that story, Robert came up with the song “A Spoonful of Sugar Helps the Medicine Go Down”. Apparently, the song was good enough for Julie Andrews, and the rest is movie musical history!

References

Oxford English Dictionary (OED). https://www.oed.com/ 

Ely, Margaret. (2013, September 24.) History behind ‘An apple a day.’ The Washington Post. https://www.washingtonpost.com/lifestyle/wellness/history-behind-an-apple-a-day/2013/09/24/aac3e79c-1f0e-11e3-94a2-6c66b668ea55_story.html 

Gandhi, L. (2013, August 26.) A History of ‘Snake Oil Salesmen.’ NPR. https://www.npr.org/sections/codeswitch/2013/08/26/215761377/a-history-of-snake-oil-salesmen 

Gutosky, Ellen. (2022, September 30.) The Cloudy Origins of the Phrase ‘Under the Weather’. Mental Floss. https://www.mentalfloss.com/posts/under-the-weather-meaning 

Kunin, R. A. (1989). Snake oil. The Western journal of medicine, 151(2), 208. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1026931/

MRI images of the brain

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Warning: This post contains discussion of murder.

A handful of serial killers have gained extreme notoriety in popular culture. You might recognize the names Richard Ramirez, Jeffrey Dahmer, John Wayne Gacy, and Ed Gein. These four infamous men have something else in common too, and it might surprise you. All four reportedly suffered head trauma as children.

Traumatic brain injuries (TBIs) are a leading cause of death and disability in the juvenile population (Williams et al., 2018). In a previous post in this series, we examined the effects of chronic traumatic encephalopathy, which results from repeated head trauma. However, even just one moderate-to-severe head injury can have serious repercussions.

A traumatic brain injury is defined as “an insult to the brain by an external mechanical force” (Williams et al., 2018). Moderate-to-severe TBI cases are characterized by at least one of the following: >30 minutes loss of consciousness, >24 hours altered mental status, >24 hours post-traumatic amnesia, or a score of <13 on the Glasgow Coma Scale (DynaMed). In such cases, internal bleeding and hypoxia can occur. 

When someone suffers a TBI, there is a risk of developing long-term neurocognitive issues including loss of executive function, impulsivity, and aggression (Williams et al., 2018). In turn, this could lead to social issues; for example, people with serious brain injury might have fewer educational and employment opportunities, and might be more likely to abuse drugs (Williams et al., 2018). 

Thus, TBIs could potentially lead to a number of behavioral and social risk factors for violent crime. Studies have borne out this connection; for example, a 2011 meta-analysis found a significantly higher prevalence of TBIs among incarcerated people as compared to the general population (Farrer & Hedges, 2011).

So, do TBIs turn someone into a killer? No, it’s not that simple. First, of course, the overwhelming majority of people who suffer head trauma in their youth do not become serial killers. Moreover, it can be hard to distinguish correlation from causation in these cases because certain factors, such as low socioeconomic status and aggressive and risk-seeking behavior, can predispose one to both TBI and criminal activity (Williams et al., 2018). As well, there are a great number of other biopsychosocial factors associated with serial killers, including experiencing child abuse or having mental illnesses like antisocial personality disorder or psychopathy.

The four serial killers mentioned here were convicted of the murders of 65 people in total. Did traumatic brain injury play a role in this massive loss of life? 

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References

DynaMed. Moderate to Severe Traumatic Brain Injury. EBSCO Information Services. Accessed January 19, 2023. https://www.dynamed.com/condition/moderate-to-severe-traumatic-brain-injury

Farrer TJ, Hedges DW. Prevalence of traumatic brain injury in incarcerated groups compared to the general population: a meta-analysis. Prog Neuropsychopharmacol Biol Psychiatry. 2011 Mar 30;35(2):390-4. doi: 10.1016/j.pnpbp.2011.01.007. Epub 2011 Jan 14. PMID: 21238529. https://pubmed.ncbi.nlm.nih.gov/21238529/ 

Williams WH, Chitsabesan P, Fazel S, McMillan T, Hughes N, Parsonage M, Tonks J. Traumatic brain injury: a potential cause of violent crime? Lancet Psychiatry. 2018 Oct;5(10):836-844. doi: 10.1016/S2215-0366(18)30062-2. Epub 2018 Feb 26. Erratum in: Lancet Psychiatry. 2018 Jul 17;: PMID: 29496587; PMCID: PMC6171742. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171742/

A baby sleeping
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Warning: This post contains mention of child abuse.

In October 2003, Julie Baumer took her infant nephew Philipp to the hospital. The boy presented with lethargy, irritability, vomiting, and unwillingness to eat. Doctors performed a CT scan and saw that the baby had a skull fracture and subdural and retinal hemorrhaging. The bleeding was so great that his fontanelle (the soft spot where a baby’s skull is forming) was bulging (Bazelon, 2011). Philipp was also dehydrated and septic. Due to the extensive amount of time taken before an emergency operation, Philipp suffered severe brain damage; he survived the ordeal, but lives with cerebral palsy (Bazelon, 2011). For Julie Baumer, the worst was yet to come.

Baumer was charged with first-degree child abuse. Doctors testified that the baby had experienced blunt force trauma and Shaken Baby Syndrome (SBS) at Baumer’s hands. In 2005, she was convicted and sentenced to 10-15 years in prison. 

In 2010, thanks to the Michigan Innocence Clinic, Baumer was granted a new trial. This time, six experts testified that the baby had been suffering from cerebral venous sinus thrombosis (CVST), the effects of which can appear similar to SBS. Vaslow (2021) states that “for infants presenting with subdural hemorrhage, retinal hemorrhage, and neurological decline the ‘consensus’ opinion is that this constellation represents child abuse and that cerebral venous sinus thrombosis … is a false mimic…. [but] this conclusion is false for a subset of infants with no evidence of spinal, external head, or body injury.” 

CVST is known by many names, including cerebral sinovenous thrombosis and cerebral venous thrombosis (CVT). Whatever you call it, this is a stroke characterized by a blood clot in a cerebral blood vessel (DynaMed). Within the pediatric population, newborns account for 30-50% of cases (Ichord, 2017). Although the clinical presentation in children can vary, pediatric patients will typically present with headache, vomiting, and depressed mental status (DynaMed). Other symptoms may include seizures and otalgia (ear pain). 

In newborns, acute systemic illness is the primary risk factor for CVST (Ichord, 2017). In children, the predominant cause of CVST is acute head or neck infections, such as mastoiditis; chronic diseases such as nephrotic syndrome or inflammatory bowel disease are also possible causes. In order to diagnose CVST, neuroimaging must be used, typically an MRI (Vaslow, 2021). 

What about Philipp’s skull fracture? This was a “red herring” in the case which at first seemed like evidence of child abuse, but with further analysis, doctors were able to determine that this was an older injury attributable to Philipp’s difficult birth (Bazelon, 2011). 

Julie Baumer was acquitted of all charges, but some – including the family who ended up adopting Philipp – still believe she is guilty. Although it is not the legal responsibility of a doctor to determine a caretaker’s innocence, medical professionals are required to document injuries objectively and report suspected abuse to authorities (U.S. Department of Justice, 2014). Therefore, research into and awareness of the differences between SBS and CVST is necessary to keep young children safe and healthy and to keep innocent caregivers out of prison.

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References and Further Reading

Bazelon, Emily. Shaken-Baby Syndrome Faces New Questions in Court. The New York Times Magazine. February 2, 2011. https://www.nytimes.com/2011/02/06/magazine/06baby-t.html?pagewanted=all&_r=0

Brown SD. Ethical challenges in child abuse: what is the harm of a misdiagnosis? Pediatr Radiol. 2021 May;51(6):1070-1075. doi: 10.1007/s00247-020-04845-4. https://link-springer-com.proxygw.wrlc.org/article/10.1007/s00247-020-04845-4 

Denzel, Stephanie. Julie Baumer. The National Registry of Exonerations. Updated February 27, 2020. https://www.law.umich.edu/special/exoneration/Pages/casedetail.aspx?caseid=3017

DynaMed. Cerebral Venous Thrombosis (CVT) in Children. https://proxygw.wrlc.org/login?url=https://www.dynamed.com/condition/cerebral-venous-thrombosis-cvt-in-children Accessed May 26, 2022.

Ichord R. Cerebral sinovenous thrombosis. Frontiers in pediatrics. 2017;5:163-163. doi:10.3389/fped.2017.00163 https://www.frontiersin.org/articles/10.3389/fped.2017.00163/full 

U.S. Department of Justice. (2014). Recognizing When a Child’s Injury or Illness Is Caused by Abuse. https://permanent.fdlp.gov/gpo84798/243908.pdf

Vaslow DF. Chronic subdural hemorrhage predisposes to development of cerebral venous thrombosis and associated retinal hemorrhages and subdural rebleeds in infants. Neuroradiol J. 2022 Feb;35(1):53-66. doi: 10.1177/19714009211026904. https://proxygw.wrlc.org/login?url=https://journals.sagepub.com/doi/pdf/10.1177/19714009211026904 

Vinchon M, Noulé N, Karnoub MA. The legal challenges to the diagnosis of shaken baby syndrome or how to counter 12 common fake news. Childs Nerv Syst. 2022 Jan;38(1):133-145. doi: 10.1007/s00381-021-05357-8. https://link.springer.com/content/pdf/10.1007/s00381-021-05357-8.pdf

A person measuring their weight on a scale

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Warning: This post contains discussion of murder and eating disorders.

At the turn of the 20th century, there was a doctor who literally starved her patients to death. Dr. Linda Hazzard did not have a medical degree, but she was nevertheless licensed by the state of Washington as a practitioner of alternative medicine. Hazzard called herself a fasting specialist, and her methods were rooted in her belief that all health issues were caused by consuming too much food. She treated her patients by making them fast for long periods of time in order to let the digestive system “rest”. They would consume only small portions of vegetable broth, take enemas, and be subjected to violent so-called “massages”. 

That doesn’t sound like a very pleasant course of treatment, so you might be surprised to learn that Hazzard was very popular in her time. She attracted many patients to her Institute of Natural Therapeutics in Olalla, WA, which opened in 1907. Her most famous patients were the British sisters Claire and Dora Williamson, who came to the institute in February 1911. The sisters had a number of minor ailments and were hopeful that Hazzard’s methods held the cure.

For two months, the sisters consumed no more than two cups of broth a day and endured hours-long enemas. When their friend Margaret Conway received a note from one of the sisters that concerned her, she came to visit them. But she was too late – as soon as Margaret arrived, she was told that Claire had just died. Dr. Hazzard, who had performed Claire’s autopsy herself, explained Claire’s death as the result of drugs administered in her youth which had eventually caused cirrhosis of the liver. Margaret, who had been the sisters’ childhood nurse, thought that explanation seemed unlikely. And then she saw Dora – or what was left of her. At this point the nearly skeletal Dora weighed only about 50 pounds (Lovejoy, 2014). Margaret was now convinced that Claire had starved to death and Dora was next in line.

What leads to starvation? Sadly, of course, there are many people in the world who lack basic access to food, and they might succumb to starvation. There are also a number of reasons that people might make the choice to abstain from eating for long periods of time, such as religious practice or as a political statement, but reports of people dying from voluntary starvation in this way have been limited to extreme cases. In developed countries, death by starvation is most often associated with eating disorders such as anorexia nervosa, a psychiatric illness characterized by extreme calorie restriction and disturbed body image. Although we do not know whether the Williamson sisters suffered from anorexia, an examination of the physical effects of anorexia, starvation, and extreme weight loss in general can give us a sense of what happened to the women. 

As starvation sets in, nutrient scarcity leads to a decrease in basal metabolism, and the resting metabolic rate (RMR) and heart rate both drop (Olson et al., 2020). Without proper nutrition, the body starts breaking down muscles for protein and fat for energy. People with anorexia often have sarcopenia and loss of skeletal muscle mass (Cost et al., 2020). Starvation can also cause hypoglycemia (Cost et al., 2020; Palmer et al., 2021). This can lead to ketoacidosis and liver failure. Extreme weight loss also affects bone development, sometimes leading to irreversible osteoporosis (Epocrates).

Extreme weight loss can cause an abundance of issues with all of the body’s physiological systems, including the cardiac, reproductive, renal, and gastrointestinal systems (Epocrates). Cardiac issues can range from myocardial atrophy to pericardial effusion and even sudden cardiac death (Cost et al., 2020). On the gastrointestinal level, constipation, diarrhea, and functional bowel disorders are common complaints in people with extreme weight loss (Cost et al., 2020).

Back at the institute, Margaret’s horror only increased once she learned that Hazzard had been appointed executor of Claire’s estate and legal guardian of Dora. Along with John Herbert, the sisters’ uncle, Margaret convinced Hazzard to allow Dora to depart from the institute (only after Herbert paid an extortionate amount of money that Hazzard claimed Dora owed in medical bills). Herbert and another man, Lucian Agassiz, then decided to research Hazzard further. They discovered that Hazzard was connected to the deaths of several other wealthy people – more than a dozen – many of whom had signed over their estates to her. They now believed that Hazzard was less a doctor and more a manipulative serial killer. 

Agassiz initiated a court case against Hazzard. In August 1911, Hazzard was charged with the first-degree murder of Claire, although she continued to claim her innocence. In February 1912, Hazzard was convicted of manslaughter. She served two years in prison and had her medical license revoked. However, in 1920, Hazzard was able to return to Olalla to re-establish her institute, now called the School of Health, where she worked until 1935 when the institute burned down. In the late 1930s, Hazzard fell sick and undertook a fast of her own; she died. 

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References and Further Reading:

Cost J, Krantz MJ, & Mehler PS. (2020). Medical complications of anorexia nervosa. Cleveland Clinic Journal of Medicine, 87(6), 361–366. https://doi.org/10.3949/ccjm.87a.19084 https://proxygw.wrlc.org/login?url=https://www.ccjm.org/content/ccjom/87/6/361.full.pdf 

Epocrates. Anorexia nervosa. Accessed May 19, 2022. https://proxygw.wrlc.org/login?url=https://online.epocrates.com/diseases/440/Anorexia-nervosa  

Lovejoy, B. (2014, October 28). The Doctor Who Starved Her Patients to Death. Smithsonian Magazine. https://www.smithsonianmag.com/history/doctor-who-starved-her-patients-death-180953158/

Olsen, G. (2005). Starvation Heights: A True Story of Murder and Malice in the Woods of the Pacific Northwest. Crown.

Olson B, Marks DL, Grossberg AJ. (2020). Diverging metabolic programmes and behaviours during states of starvation, protein malnutrition, and cachexia. J Cachexia Sarcopenia Muscle, 11(6):1429-1446. doi: 10.1002/jcsm.12630. Epub 2020 Sep 28. PMID: 32985801 https://proxygw.wrlc.org/login?url=https://onlinelibrary.wiley.com/doi/10.1002/jcsm.12630  

Palmer BF, Clegg DJ. (2021). Starvation Ketosis and the Kidney. Am J Nephrol., 52(6):467-478. doi: 10.1159/000517305. Epub 2021 Jul 19. PMID: 34350876. https://proxygw.wrlc.org/login?url=https://www.karger.com/Article/FullText/517305  

Washington State Archives. (n.d.) Linda Burfield Hazzard: Healer or Murderess? https://www.digitalarchives.wa.gov/Collections/TitleInfo/2508 

Salmonella invading a human cell.
"Salmonella Bacteria" by NIAID. This image is licensed under CC BY 2.0.

It was the largest bioterrorist attack in American history. In 1984, followers of the mystic Rajneesh contaminated salad bars at ten restaurants in The Dalles, Oregon with Salmonella. 751 people suffered food poisoning, 45 of whom had to be hospitalized. 

The followers of Rajneesh lived in Rajneeshpuram, a religious commune. They hoped that by incapacitating voters in The Dalles, they could get their own candidates to win the upcoming Wasco County election and thereby gain political influence. Two Rajneeshpuram officials, Ma Anand Sheela and Ma Anand Puja, purchased Salmonella enterica Typhimurium from a medical supply company. The enterica subspecies of Salmonella accounts for around 99% of Salmonella-related diseases in humans (Chen et al., 2013). Several other Rajneeshees then helped spread the bacteria by sprinkling it on ingredients in salad bars.

As a result, 751 people suffered from acute gastroenteritis. The victims ranged in age from infancy to 87 years old. Symptoms included diarrhea, fever, chills, nausea, vomiting, headaches, and abdominal pain. According to the IDSA guidelines, if you suspect a patient to have infectious diarrhea and they are presenting with fever or bloody diarrhea, you should evaluate the patient for enteropathogens including Salmonella and Shigella, typically by testing a stool sample (Shane et al., 2017). Doctors did just that and lab testing determined that Salmonella was the culprit.

Salmonella is “the most common bacterial pathogen causing gastrointestinal infection worldwide” (Chen et al., 2013). Salmonella can be divided into two types: those that cause nontyphoidal gastrointestinal illness and those that cause typhoid illness (FDA). S. Typhimurium, which was used in this case, belongs to the first category. Don’t get this confused with S. Typhi, which is typhoidal. We’re focusing on non-typhoidal Salmonella here when discussing symptoms and treatment. 

Acute gastroenteritis can appear anywhere from four to 72 hours post-infection; fever is likely to subside naturally within 3 days and diarrhea within 3-7 days (Chen et al., 2013). When treating salmonellosis (i.e. salmonella infection), it is important to ensure the patient is not suffering from dehydration; fluid replacement is often necessary (Shane et al., 2017). Antimicrobial treatment is typically not necessary, but in severe cases ceftriaxone has been shown to be helpful (Chen et al., 2013). Additionally, antinausea and/or antiemetic agents can be considered (Shane et al., 2017). With proper treatment, mortality is less than 1% (FDA). All of the victims in this attack survived.

Oregon health officials initially believed that the poisonings were due to the restaurants’ unhygienic environments. But residents of The Dalles suspected the Rajneeshees of a deliberate attack. A year later, the Rajneeshees were confirmed to have committed the act when a sample of bacteria exactly matching the known contaminant was discovered in a Rajneeshpuram laboratory. Sheela and Puja, who had led the attack, were convicted of attempted murder (as well as other crimes unrelated to this story, including assault and wiretapping) and both served 29 months in prison.

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References

Chen HM, Wang Y, Su LH, Chiu CH. Nontyphoid salmonella infection: microbiology, clinical features, and antimicrobial therapy. Pediatr Neonatol. 2013 Jun;54(3):147-52. doi: 10.1016/j.pedneo.2013.01.010. Epub 2013 Mar 1. PMID: 23597525. https://proxygw.wrlc.org/login?url=https://www.clinicalkey.com/service/content/pdf/watermarked/1-s2.0-S1875957213000119.pdf?locale=en_US&searchIndex=

Food and Drug Administration. Bad Bug Book: Foodborne Pathogenic Microorganisms and Natural Toxins. Second Edition. [Salmonella spp., pp. 9-13]. 2012. https://www.fda.gov/media/83271/download

Shane AL, Mody RK, Crump JA, Tarr PI, Steiner TS, Kotloff K, Langley JM, Wanke C, Warren CA, Cheng AC, Cantey J, Pickering LK. 2017 Infectious Diseases Society of America Clinical Practice Guidelines for the Diagnosis and Management of Infectious Diarrhea. Clin Infect Dis. 2017 Nov 29;65(12):1963-1973. doi: 10.1093/cid/cix959. PMID: 29194529. https://proxygw.wrlc.org/login?url=https://academic.oup.com/cid/article/65/12/e45/4557073?login=true 

Picture of an empty bed

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Warning: This post contains discussion of murder.

Have you ever sleepwalked? It can be disconcerting to realize that you did something even though you were fast asleep. But you probably haven’t been as upset as Kenneth Parks, who woke up to discover that he had committed murder.

On an early morning in May 1987 in Ontario, Canada, Parks drove several miles to his in-laws’ house, opened the door, bludgeoned his mother-in-law to death and tried to suffocate his father-in-law – all while he was asleep. Upon waking up from this sleepwalking episode, Parks immediately turned himself into the police. (The victims’ names were not made public.)

Somnambulism is the fancy word for sleepwalking, derived from the Latin words somnus (“sleep”) and ambulare (“to walk”). It is classified as a type of parasomnia and primarily affects children (DynaMed). There are a number of potential causes including stress, sleep deprivation, alcohol use, and thyrotoxicosis (DynaMed). In one survey, 25% of adult sleepwalkers reported concurrent anxiety or mood issues (Zadra et al., 2013). A number of studies have reported that high levels of sleep deprivation can increase sleepwalking events by a factor of anywhere from 2.5 to 5 (Zadra et al., 2013). Sleepwalking can occur at any point in the sleep cycle, but most often happens during non-REM sleep (DynaMed). While sleepwalking, the person’s eyes are typically open and they appear awake. They can engage in normal-seeming behavior, but their movements may be awkward. More complex behaviors – for example, driving a car, as Parks did – is more common in sleepwalking adults than children (Zadra et al., 2013). The sleepwalker will have no memory of the events that occurred while they were sleepwalking. 

Violence during somnambulism is usually reactive, occurring when the sleepwalker is confronted by another person. The sleepwalker will not recognize the person they are attacking “even if it is a very close family member,” thinking instead that the person is some sort of intruder (Stallman & Bari, 2017). There is little research on the etiology of violence during somnambulism, but Stallman & Bari suggest a biopsychosocial model with multiple potential risk factors. Diagnosis of somnambulism is typically based on the patient’s clinical history but polysomnography can also be employed. In Parks’ case, doctors took a thorough history and also administered two overnight polysomnograms (Broughton et al., 1994).

Broughton et al. (1994) unfold Parks’ story in their case report. In the months leading up to the attack, Parks had been facing high gambling debts and got fired because he was caught embezzling money from his workplace. The stress from this situation had been creating issues with his sleep schedule. Moreover, he and his wife had just welcomed their newborn daughter, which caused more stress and sleep deprivation. On the night of May 23, after an argument with his wife, Parks went to bed. He had made a plan to swallow his embarrassment and explain his financial situation to his parents-in-law, with whom he was close, the following day. But the next thing he knew, he was staring at the face of his dead mother-in-law.

Parks was charged with murder and attempted murder. In trial, he pleaded not guilty via the insane automatism defense, meaning that although he did commit murder, he did not consciously form the intention to do so (Popat & Winslade, 2015). The doctors and neurologists who testified at the case claimed that Parks had a history of sleepwalking and that his propensity toward it was likely exacerbated by sleep deprivation as well as emotional distress and anxiety (Popat & Winslade, 2015). Evidence for this included the fact that on several psychological tests, Parks had scored high for depression and anxiety (Broughton et al., 1994). Parks was acquitted of all charges.

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References

Broughton R, Billings R, Cartwright R, et al. Homicidal somnambulism: A case report. Sleep (New York, NY). 1994;17(3):253-264. doi:10.1093/sleep/17.3.253

DynaMed. Sleepwalking. EBSCO Information Services. Accessed August 23, 2022.  https://proxygw.wrlc.org/login?url=https://www.dynamed.com/condition/sleepwalking  

Popat S, Winslade W. While You Were Sleepwalking: Science and Neurobiology of Sleep Disorders & the Enigma of Legal Responsibility of Violence During Parasomnia. Neuroethics. 2015;8(2):203-214. doi:10.1007/s12152-015-9229-4  https://proxygw.wrlc.org/login?url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4506454/  

Stallman HM, Bari A. A biopsychosocial model of violence when sleepwalking: review and reconceptualisation. BJPsych Open. 2017 Apr 26;3(2):96-101. doi: 10.1192/bjpo.bp.116.004390. PMID: 28446961 https://proxygw.wrlc.org/login?url=https://www.cambridge.org/core/journals/bjpsych-open/article/biopsychosocial-model-of-violence-when-sleepwalking-review-and-reconceptualisation/0F22062014B3EA2002ADBE94A3FCF2C2   

Zadra A, Desautels A, Petit D, Montplaisir J. Somnambulism: clinical aspects and pathophysiological hypotheses. Lancet Neurol. 2013 Mar;12(3):285-94. doi: 10.1016/S1474-4422(12)70322-8. PMID: 23415568. http://proxygw.wrlc.org/login?url=https://www.proquest.com/scholarly-journals/somnambulism-clinical-aspects-pathophysiological/docview/1319202693/se-2?accountid=11243 

“Crime Scene Do Not Cross” Signage

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Warning: This post contains discussion of murder and sexual abuse.

Vince Gilmer was a kind, well-liked doctor in his small town of Cane Creek, North Carolina – that is, until July 2004, when he was arrested for the vicious murder of his father. How could this beloved man turn into a violent criminal?

In court, Vince defended his actions by explaining that he had been a victim of his father’s sexual abuse since childhood and that his father had assaulted him on the day of the murder. Despite these claims, Vince was found guilty of the murder.

Five years later, another Dr. Gilmer arrived in Cane Creek. Benjamin Gilmer was not related to Vince, but because of the similarities between the two men (surname, profession, age), the case grabbed Benjamin’s interest. Sarah Koenig, a reporter with the program This American Life, got in contact with Benjamin and they decided to investigate Vince’s story together.

People described Vince’s behavior in the months leading up to the murder as unstable. He had gotten into a serious car accident (possibly intentionally) in which he hit his head; had started drinking heavily following his divorce; and had stopped taking his prescribed antidepressant (Lexapro, an SSRI). When Benjamin and Koenig visited Vince in prison, he told them that he had heard voices on the day he committed the murder. Vince theorized that he might have been suffering from SSRI withdrawal.

Benjamin thought SSRI withdrawal was a plausible diagnosis, and he also wondered if perhaps Vince had gotten a traumatic brain injury from his car accident. To get a psychiatrist’s perspective, Benjamin invited a friend of his to meet Vince. Noticing Vince’s odd gait and wide gesticulations, the psychiatrist had a different suggestion: Vince might have Huntington’s disease. 

Huntington’s is a neurodegenerative disease that can cause motor impairment as well as cognitive/emotional issues like confusion, anxiety, and impulsivity (Novak & Tabrizi, 2010). Koenig describes it as “a cruel trifecta of Alzheimer’s, Parkinson’s, and Lou Gehrig’s disease, rolled into one.” The average age of onset is 40 years, and life expectancy after diagnosis is only 10-25 years. Huntington’s is a genetic disease – offspring of affected parents have a 50% chance of getting it too (Novak & Tabrizi, 2010). It’s possible Vince’s father had it, as he had exhibited some symptoms but was never diagnosed. There is no cure for Huntington’s and no way to halt the progression of the disease, although some drugs such as Tetrabenazine have been shown to reduce symptoms of chorea (the sudden jerking movements characteristic of the disease) (Novak & Tabrizi, 2010). 

Symptoms of Huntington’s include hallucinations, irritability, moodiness, paranoia, hyperkinesia, confusion, memory loss, and anxiety. Vince basically checked every box. To diagnose Huntington’s, a DNA test is required to detect CAG trinucleotide expansion in the HTT gene (DynaMed). A normal HTT gene will have fewer than 36 CAG repeats, while a gene with 40+ repeats is strongly indicative of Huntington’s. Imaging can also be useful for diagnosis, as Huntington’s is characterized by cerebral atrophy (Novak & Tabrizi, 2010). Vince’s DNA test confirmed that he had Huntington’s. 

But does Huntington’s create murderers? People with Huntington’s are actually much more likely to be the victims of violence than to commit violence (Finan, 2022). But perhaps the combination of Huntington’s, antidepressant withdrawal, stress, and years of abuse could cause somebody to “snap”. That’s what Dr. Mary Edmonson suggests happened in Vince’s case (Finan, 2022). What do you think?

After many years, Benjamin succeeded in his mission to get Vince’s conviction overturned. In January 2022, the Virginia governor granted Vince a pardon. Vince is now in late-stage Huntington’s and has become more physically disabled; he has extreme difficulty with talking and swallowing, and though he can still walk, he falls often. As of March of this year, Benjamin was in the process of becoming Vince’s legal guardian and was making plans for Vince to go to a medical care facility where he will receive appropriate treatment. 

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References and Further Reading:

DynaMed. Huntington Disease. EBSCO Information Services. Accessed August 11, 2022.  https://proxygw.wrlc.org/login?url=https://www.dynamed.com/condition/huntington-disease 

Finan E. After a Beloved Small-Town Doctor Murdered His Own Father, the Clinic's New Doc Solved the Mystery of Why. People. March 3, 2022. https://people.com/crime/after-a-beloved-small-town-doctor-murdered-his-own-father-the-clinics-new-doc-solved-the-mystery-of-why/

Gilmer, Benjamin. The Other Dr. Gilmer. Ballantine Books; 2022.

Koenig, S. Dr. Gilmer and Mr. Hyde. This American Life. April 12, 2013. https://www.thisamericanlife.org/492/dr-gilmer-and-mr-hyde  

Novak MJ, Tabrizi SJ. Huntington's disease. BMJ. 2010 Jun 30;340:c3109. doi: 10.1136/bmj.c3109. PMID: 20591965. https://proxygw.wrlc.org/login?url=https://www.bmj.com/content/340/bmj.c3109.long 

Clashing football players

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Warning: This post contains discussion of murder.

June 2013: Odin Lloyd is found dead, and professional football player Aaron Hernandez is charged with first-degree murder. But could brain damage have influenced Hernandez’s actions?

Hernandez, who was a tight end for the New England Patriots, already had a long history of violent and criminal behavior. In 2015, he was found guilty of Lloyd’s murder and received a life sentence.

Two years later, while in the process of filing an appeal for the murder conviction, Hernandez, 27 years old, committed suicide. Hernandez was posthumously diagnosed with chronic traumatic encephalopathy (CTE) at the Boston University CTE Center, which has studied the brains of over 300 deceased football players (Pfeiffer, 2018). This diagnosis led to speculation that Hernandez’s deadly behavior might have been influenced or caused by intense traumatic injury to his brain.

CTE is a neurodegenerative disorder that develops because of repeated trauma to the head. Over time, repetitive head impacts, including both concussive and subconcussive blows, lead to CTE, but the exact amount of trauma required is unknown. CTE is a progressive disease that occurs in a number of stages (either three or four depending on how you divide it up) linked to changes in neuropathology (McKee et al., 2013). The first stage can include confusion, dizziness, and headache. The second stage can include memory loss and impulsivity. The third and fourth stages can include dementia, speech impediments, sensory processing disorders, tremors, depression, and suicidal behavior.

The majority of documented CTE cases have occurred in athletes in contact sports like football, wrestling, and boxing. CTE is common among this population: for example, a recent cohort study found that in a sample of 202 deceased football players, 87% were diagnosed postmortem with CTE – including 110 of the 111 NFL players in the sample (Mez et al, 2017).

CTE is a tauopathy, like Alzheimer’s, described as “a progressive neurodegeneration characterized by the widespread deposition of hyperphosphorylated tau (p-tau) as neurofibrillary tangles” (McKee et al., 2013). As of now, there is no test that can definitively determine the existence of CTE in a living person. The diagnosis can only occur during an autopsy because it requires the removal of the brain in order to analyze the tissue (Pfeiffer, 2018). Although CTE can only be diagnosed posthumously, it should be considered likely in patients with Traumatic Encephalopathy Syndrome (TES) and one or more CTE biomarkers (DynaMed). There is no known cure for CTE, but some symptoms can be managed with medications and behavioral therapies.

At his autopsy, Hernandez was determined to have stage-3 CTE, never before seen in a person as young as him (Pfeiffer, 2018). Did CTE cause Hernandez to kill Lloyd? And if so, is that a viable defense against a murder charge? What do you think?

Do you know of another interesting true crime case with medical connections? Email Rachel Brill at rgbrill@gwu.edu.

References and Further Resources

Bryant C & Clark J. What’s the Deal with Chronic Traumatic Encephalopathy? [Podcast episode]. Stuff You Should Know. November 2016.

Dillard, J. Amy and Tucker, Lisa A. “Is C.T.E. a Defense for Murder?” September 2017. The New York Times.

DynaMed. Concussion and Mild Traumatic Brain Injury. EBSCO Information Services. Accessed July 12, 2022. https://proxygw.wrlc.org/login?url=https://www.dynamed.com/condition/concussion-and-mild-traumatic-brain-injury

McKee AC, Abdolmohammadi B, Stein TD. The neuropathology of chronic traumatic encephalopathy. Handb Clin Neurol. 2018;158:297-307. doi: 10.1016/B978-0-444-63954-7.00028-8. https://pubmed-ncbi-nlm-nih-gov.proxygw.wrlc.org/30482357/

McKee AC, Stern RA, Nowinski CJ, Stein TD, Alvarez VE, Daneshvar DH, Lee HS, Wojtowicz SM, Hall G, Baugh CM, Riley DO, Kubilus CA, Cormier KA, Jacobs MA, Martin BR, Abraham CR, Ikezu T, Reichard RR, Wolozin BL, Budson AE, Goldstein LE, Kowall NW, Cantu RC. The spectrum of disease in chronic traumatic encephalopathy. Brain. 2013 Jan;136(Pt 1):43-64. doi: 10.1093/brain/aws307. Epub 2012 Dec 2. Erratum in: Brain. 2013 Oct;136(Pt 10):e255. https://www-ncbi-nlm-nih-gov.proxygw.wrlc.org/pmc/articles/PMC3624697/

Mez J, Daneshvar DH, Kiernan PT, Abdolmohammadi B, Alvarez VE, Huber BR, Alosco ML, Solomon TM, Nowinski CJ, McHale L, Cormier KA, Kubilus CA, Martin BM, Murphy L, Baugh CM, Montenigro PH, Chaisson CE, Tripodis Y, Kowall NW, Weuve J, McClean MD, Cantu RC, Goldstein LE, Katz DI, Stern RA, Stein TD, McKee AC. Clinicopathological Evaluation of Chronic Traumatic Encephalopathy in Players of American Football. JAMA. 2017 Jul 25;318(4):360-370. doi: 10.1001/jama.2017.8334. https://jamanetwork-com.proxygw.wrlc.org/journals/jama/fullarticle/2645104

Pfeiffer, Sacha. “A Terrible Thing to Waste.” October 2018. The Boston Globe.