ALS: The Ice Bucket Disease

Amyotrophic lateral sclerosis

I was recently challenged to participate in the latest viral social behavior: the ice bucket challenge. The activity raises awareness for Amyotrophic lateral sclerosis, now more commonly known as ALS. I have decided to respond to the challenge with this blog post rather than pouring water over my head, since I’ve come to realize that watching over twenty ice challenge videos has taught me next to nothing about the disease!

ALS is both the most common and most aggressive type of adult motor neuron degeneration. The death of upper motor neurons in the motor cortex and lower motor neurons in the brainstem and ventral horn of the spinal cord result in a progressive loss of muscle control. This progression is characterized by stiffness, overactive reflexes, muscle twitching, muscle atrophy, and finally full paralysis. Approximately 15% of patients also suffer from cognitive and behavioral problems known as frontotemporal dementia due to the death of neurons in the prefrontal and temporal cortex in the brain[1,5].

Finally, it is a heterogeneous disease: the age, site of onset, rate of progression, and the presence and degree of cognitive dysfunction can vary widely between patients. The disease is fatal within 3-5 years of onset[5].

ALS bucket time

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Tetanospasmin and Tetanus: The Second Deadliest Toxin

Remember my past post on botulinum toxin, the deadliest substance known to mankind? Well, it’s time to visit the second runner up. Tetanospasmin is a neurotoxin created by another member of the Clostridium genus: Clostridium tetani. That is one genus you don’t want to mess with! This particular member of Clostridium is the cause behind all those lovely tetanus shots and boosters you have been subjected to through the years. When you consider the fact that you’re being vaccinated against the second deadliest toxin in the world, maybe you won’t mind the needle jab quite as much. In a rather bizarre twist, the neurotoxin has no apparent use or benefit to C. tetani in its natural environment. The intoxication of other organisms, such as us, appears to be mainly incidental.


Photographs adapted from

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Botulinum Neurotoxin: From Weapon to Cosmetic

Botulinum neurotoxin is the deadliest substance on Earth; a single gram would kill over a million people if inhaled and 8.3 million if injected. So, naturally, humans have developed a specific method to inject it into our faces. Believe it or not, this neurotoxin is the primary component of Botox!


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