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.