Hooch
Do you know what poison is the most potent known to man, yet is ubiquitous in nature? How you can be poisoned by a prison potato?
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Our episode today is called Hooch. Do you know what poison is the most potent known to man, yet is ubiquitous in nature? How you can be poisoned by a prison potato? Listen to find out!
Today's episode starts in the emergency department. You see a patient being wheeled into room five. He's wearing an orange jumpsuit, is handcuffed to the stretcher, and accompanied by two prison guards.
The intern goes to see him, then presents the case. It’s a 34-year-old man whose chief complaint is weakness. The patient reports feeling generally weak all over. He also complains of blurry vision and difficulty swallowing. The illness started 3 days ago with nausea and crampy abdominal pain. He was seen for the same symptoms in the ED yesterday, his workup included an unremarkable CT scan of his brain, normal labs and a normal urinalysis, after which he was discharged back to prison.
Today, the neurological symptoms are not only ongoing, but worsening. He feels like his arms are weak weaker than yesterday. He reports the abdominal pain and nausea resolved, though he’s constipated and hasn't had a bowel movement in three days. He denies any past medical history, reports no medicines or supplement use and has no allergies.
On exam, his vital signs are a temperature of 98.6 Fahrenheit or 37 Celsius, blood pressure 120/80, heart rate of 80 bpm, respiratory rate of 18 and oxygen saturation of 100% on room air.
The intern’s unsure about his neuro exam so you go to the bedside to examine the patient together. You test his cranial nerves, those originating from the brain and brainstem, rather than the spinal cord and control things like smell, vision, and facial muscles. His eyelids are droopy, though it’s hard to tell if this is just how he normally looks or if they are actually weak. His pupils are large and minimally reactive to light. He’s a big, muscular guy so it’s hard to tell for sure, but he does appear to have subtle muscle weakness in his arms. You can’t elicit the reflexes either.
You and the intern return to your desk. You review the results from yesterday, all normal, not even a hint of any abnormality. You ask about tobacco, alcohol, and drugs. The intern says the patient denied smoking, but he didn't ask about alcohol and drugs given the patients in prison. In fact, drugs are widely available in prison and medical care of prisoners can be challenging for a number of reasons. Often preventative care is lacking. Complaints may be dismissed, or attributed to malingering, complicating the picture.
What about our patient? He was already seen and evaluated yesterday for vague complaints. Is he malingering? The hospital is often preferable to prison thanks to violence, mental health issues, drug use, gangs, etc.
No. Our patient has neurological findings on exam. Subtle, but present. Ptosis, the medical term for droopy eyelids, suggests weakness of the 3rd cranial nerve. His pupils are large, this can be due to drugs of abuse, sympathomimetics like methamphetamine or cocaine. But his vital signs are normal, so more likely from something else. His arms seem weak and we can’t get his reflexes.
Is this a stroke? No, strokes cause unilateral, one-sided, not bilateral symptoms. Encephalitis and meningitis can certainly cause neurological problems, but typically he'd have fever, headache, and neck stiffness. Electrolyte disturbances like low potassium and high magnesium cause weakness, but his labs yesterday were normal.
I’d definitely be concerned about Guillain-Barré syndrome, a disease causing descending paralysis. It can occur after exposure to infections like bacteria or viruses, rarely after vaccines, or sometimes with no known cause. Myasthenia gravis and multiple sclerosis are both concerns. We need neurology to help for sure. Anytime you consult neurology, they love to recommend two test, MRIs and spinal taps, so we might as well do those now. The intern orders a brain MRI and sets up for the spinal tap. If the patient has Guillain-Barre, the CSF protein will be elevated. The spinal tap will also rule out infection, like encephalitis. You call the hospitalist to get the patient admitted.
The next day, the intern updates you on the patient. His MRI and spinal tap were negative, ruling out MS, Guillain-Barré or meningitis and encephalitis. Overnight the patient has become progressively weaker, he can barely lift his arms off the bed and his legs are now affected. He’s also having difficulty breathing and has been moved to the ICU.
This isn't a neurology podcast, it's a toxicology podcast. I love Carbon monoxide for vague complaints with subtle neurological findings. However, the entire prison would be sick so no. Coral snakes can cause paralysis, not likely in prison. Same with tick paralysis. Paralytic shellfish poisoning we've discussed previously, again, not likely if no one else is sick. Heavy metals can certainly cause peripheral neuropathy and abdominal pain so I’d keep it in the back of my mind, but his symptoms don’t really fit with lead, arsenic or thallium, for example.
Based on the patient’s symptoms of a descending flaccid paralysis, what toxin might this be? Question # 1 and time to Pick Your Poison.
Tetanus
Strychnine
Botulism.
Poison dart frog venom
Answer: C. This is consistent with botulism toxicity. Tetanus and strychnine cause rigidity, not flaccid paralysis. Poison dart frogs cause flaccid paralysis via batrachotoxin, but it’s rapid with immediate onset in minutes, not progressive worsening over days.
Botulism toxicity causes descending paralysis, meaning symptoms start at the top and move down. Patients initially complain of difficulty swallowing or blurry/double vision. As with our patient, they often present for medical care, but given the vague symptoms, are commonly sent home only to return with progressive symptoms.
So you’re concerned about botulism. How do you make the diagnosis. Good question. You can test for botulism in a number of different bodily fluids, commonly we use stool and blood and generally specimens are sent to the CDC for specialized testing. They typically do an anaerobic culture and a mouse bioassay. Meaning they inject the specimen into a poor mouse to see if the mouse dies. After discussion with neuro and the hospitalist, you contact the CDC. They agree with your suspicion and agree to run the tests.
What’s the treatment? That’s question # 2.
Supportive care
Prussian blue
Narcan or naloxone
Botulinum antitoxin
Answer: D. We have an antidote, botulinum antitoxin. In the US, the CDC has stockpiles near major airports and airlifts it to hospitals when needed. It’s a heptavalent antitoxin, meaning it treats all 7 botulism toxin subtypes, A-G. Certain subtypes are more prevalent in certain geographical areas or with certain types of infection. Only 3 subtypes typically affect humans and we used to use a trivalent antitoxin. Given the concern for botulism use in terrorism, the heptavalent antitoxin was developed and is the one we now use. The CDC agrees to release the antidote, but it will take time to arrive. In the meantime, the patient worsens, developing respiratory failure. Why? Remember the diaphragm is a muscle and if it’s paralyzed, you can’t breathe.
While we wait, let’s talk about how people are exposed to botulism. Was there a canning mishap in the prison? There are a number of different types of botulism poisoning, so let’s start there, then come back to our patient.
First, is the one everyone knows about, foodborne botulism. This is when you eat food containing botulinum toxin. Classically, yes from canning gone wrong. The toxin is produced by bacteria, Clostridium botulinum. Clostridium is found literally everywhere, ubiquitous in soil, sea water, even the air. The spores are extremely hardy and can survive in very difficult conditions, including boiling for hours at 212 Fahrenheit or 100 Celsius. They are, however, destroyed within 30 minutes at temperatures of 250 Fahrenheit (or 120 Celsius) thus the strict precautions for canning food.
You know, I love pickles and wanted to make some homemade pickles one day. The first 10 pages of the cookbook detailed very specific, very strict techniques for boiling and sterilizing. I got so worried picturing the consequences of a mistake, to this day I’ve never made any.
The second type of botulism toxicity is infant botulism. This is actually more common in the US than food borne. In the US foodborne accounts for about 15% of cases, infant bot 75%. The incidence of this disease is highest in California, New Jersey, Pennsylvania, and Maryland, though why isn’t clear. The babies less than six month. They ingest Clostridium botulinum spores and become sick. After this, the GI tract develops enough to keep the bacteria from growing inside the body. We describe the presentation as a floppy baby, due to muscle weakness. They have difficulty feeding because they're sucking reflexes are weak and weak cries. This has a separate treatment, called baby BIG for Botulism immune globulin.
Question #3. What food is associated with botulism in babies?
Honey
Peanuts
Cow’s milk
Eggs
Answer: A. Honey. This disease is the reason why parents are advised not to give honey to babies until they are one year old. I certainly wouldn’t go against this recommendation, I will say however the evidence is extremely weak and we don’t really know why some kids develop this problem and others don’t.
A third type, wound botulism occurs in the setting of IV drug use where the wound is dirty and becomes contaminated. In the US, it accounts for about 12% of cases. It’s classically associated with black tar heroin.
The other types of botulism are extremely rare. There's a type occasionally seen in the gastrointestinal tracts of patients who've had GI surgery. Iatrogenic botulism is due to therapeutic misadventures with botulism toxin. Inhalational botulism is on the list of potential biological weapons and, interestingly, caused toxicity in 3 veterinary workers after inhaling it from animal fur.
What does the toxin do in the body? Botulinum toxin is considered the most potent toxin known to man. One microgram is enough to be lethal in humans. Once the toxin gets into the bloodstream, it travels to nerve terminals and is taken up into neurons. It blocks the release of acetylcholine into the space between the neurons, the synapse. Meaning nerve impulses can’t be conducted and muscles can’t contract, resulting in flaccid paralysis.
How does the antidote work? By binding to botulism toxin. Its another antidote which is an antibody and binds up the toxin, inactivating it. Now, it can only bind circulating toxin, it doesn’t get into neurons, etc. Meaning it stops progression of the disease, but can’t reverse paralysis that’s already occurred.
Back to our patient. What's his prognosis then? Fortunately, he got timely medical care and intubation. I expect him to recover, including his muscle strength, though the recovery will take a long time. Weeks, more likely months.
The earliest reported case of botulism toxicity was in 1735 and attributed to improperly cooked blood sausage in Germany. Botulus in fact is the Latin word for sausage. Foodborne botulism is not surprisingly, most often associated with home-cooked food rather than commercial food. Which brings us back to our patient and how the heck he got botulism in prison?
Health department and prison officials are conducting an investigation. Before he was intubated, they interviewed the patient. He denied eating anything other than prison food. And the records confirmed that he hadn't recently had any visitors who might have brought him homemade food.
Question #4. What US state has the most food-born botulism cases?
California
Pennsylvania
Alaska
Hawaii
The answer is C. Alaska.
Why Alaska? Fermented food is another common source of botulism. This includes fermented fish, fish eggs, seal and whale meat. Interestingly enough about half of US food borne botulism cases occur in Alaska, despite having only 0.2% of the population. It’s because of the popularity of these fermented foods, reflecting the historical challenges of surviving in an extreme environment. An example from Greenland is called Kiviak - birds are caught and sewn inside a seal carcass to ferment. Botulism might also ferment. Anything that can go wrong will. In one interesting case, several people developed botulism after drinking peyote tea. The catus buttons were covered in water and stored in a jar for several months, brewing also botulism.
So was our patient poisoned by the prison kitchen? Always possible, but if so, why is he the only person affected? The words prison and botulism together bring to mind one word. Pruno. Prison moonshine or hooch. Prisoners brewing alcohol inside the prison from regular food items like fruit. There have been several outbreaks of botulism due to pruno. In Utah, 8 men ended up on ventilators. In Mississippi, 31 patients developed botulism toxicity after drinking a contaminated batch.
The epidemiologist from the health department confirms your suspicion a few hours later. She sends a picture of a Ziplock bag filled with cloudy yellow-green liquid found hidden inside the patient’s toilet. I’m trying not to gag just thinking about this. She says two other inmates drank the pruno, but both spit it out due to its foul taste, saving them from exposure.
Different recipes exist, but basically pruno is food fermented into alcohol. It’s often made from fruit, sometimes corn syrup, sauerkraut, hard candy or bread and ketchup. The case in the Utah prison was traced to a potato used to make one batch, which had been saved for several weeks. In addition, the mixture was strained through one of the inmate’s socks. If this isn’t enough, in addition to botulism, you can get methanol toxicity as well.
Nauseating details aside, there’s really moving poem by Jarvis Master called Recipe For Prison Pruno which he wrote while on death row. It won the PEN award for poetry in 1992, if you’re interested.
This is a fictional case, as are all our cases to protect the innocent. But it is based on real cases.
This is the great thing about toxicology, we can move right from prison to cosmetics. Can we talk about botulism without talking about Botox? Definitely not!
Question #5. Cosmetic botox injections carry a high risk of systemic botulism toxicity.
True
False
Answer: B false. The risk of systemic botulism toxicity is extremely low after medical or cosmetic procedures if injected by a licensed practitioner. In medicine, we use botox to treat chronic migraines, and muscle spasticity for example. There is a risk of muscle weakness in unwanted areas, such as the eyelids or neck, but it’s uncommon and effects wear off as the toxin does.
That said, iatrogenic botulism toxicity occurs because things can go wrong. Rarely with licensed practitioners, as noted. Unlicensed ones are another matter.
A crazy case published in the New England journal in 2006 highlights the risks. A physician ordered Botox for his clinic. The vial was labeled for laboratory research and not intended for human use. Nevertheless, the clinic diluted the Botox to use. Not incidentally, the physician’s medical license was already suspended for selling narcotic painkillers for profit. He administered this Botox injecting into his own face, his girlfriend and two patients, a husband and wife. He unintentionally gave 2,800x times the lethal dose of botulinum toxin. They all, including him, developed toxicity with weakness and respiratory failure. The physician pled guilty and was sentenced to three years of prison. Why did he use that vial? It was cheaper than the medical grade preparations.
In another case, 68 people were poisoned at a hospital in Turkey after stomach botox. Yes, you heard that right. I had no idea this existed, but apparently you can have botox injected into your stomach via endoscopy for weight loss. Supposedly it makes you less hungry, though the evidence, not surprisingly, doesn’t support its effectiveness. It seems awfully invasive to have a procedure to inject something that only lasts a few months anyway. This is a whole nother topic a will make a great future episode. The price and toxicity of beauty.
Bringing us to the last question in today’s podcast. The Japanese cult Aum Shinrikyo grew Clostridium botulinum and tried to cause toxicity by spraying it near U.S. Naval bases, Narita airport, and the Japanese Imperial Palace. Fortunately, no one was affected (including cult members who accidently exposed themselves). The cult was responsible for killing 13 people, and injuring nearly 1,000 in an attack in the Tokyo subway system. What toxin did they use in this attack?
VX
Cyanide
Arsenic
Sarin
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While I’m a real doctor this podcast is fictional, meant for entertainment and educational purposes, not medical advice. If you have a medical problem, please see your primary care practitioner. Thank you. Until next time, take care and stay safe.