Understanding Amyotrophic Lateral Sclerosis (ALS)
By: Brian Hoeflinger, MD
April 13, 2025 | #42
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Disclaimer: Opinions are my own. Not medical advice.
Medical Trivia of the Week
Which part of the nervous system controls voluntary muscle movements? (the correct answer is at the end of this email)
- A) Autonomic Nervous System
- B) Central Nervous System
- C) Peripheral Nervous System
- D) Somatic Nervous System
What is ALS?
Amyotrophic Lateral Sclerosis (ALS), historically known as Lou Gehrig’s disease, is a progressive neurological disorder that affects the nerve cells of the brain and spinal cord, ultimately leading to muscle weakness and paralysis.
The term ALS comes from the Greek language and translates to “no muscle nourishment.” When a muscle lacks proper nourishment, it begins to waste away. In someone with ALS, this often begins with wasting of the hand muscles. Other early warning signs may include slurred speech, muscle twitching, or changes in balance.
As the nerve cells responsible for voluntary muscle movement begin to die, people with ALS gradually lose their ability to speak, eat, move, and eventually breathe.
The average life expectancy following an ALS diagnosis is typically between 2 to 5 years. However, there are rare exceptions. Stephen Hawking, for example, lived 55 years after his diagnosis. He was diagnosed at age 21 and lived to 76, ultimately relying on a wheelchair and a voice synthesizer to communicate.
ALS was first discovered in 1869 by French neurologist Jean-Martin Charcot. Despite over 150 years of research, there is still no cure for the disease. While several drugs have been approved for the treatment of ALS, the condition remains fatal.
Approximately 90% of ALS cases occur spontaneously, meaning there is no family history or identifiable genetic mutation. Only about 10% of cases are familial (inherited), and for those with a known genetic mutation, there is a 50% chance that each child will inherit the gene and develop the disease.
Most people develop ALS between the ages of 40 and 70, with the average age of diagnosis being 55. However, there are well-known exceptions. Lou Gehrig was diagnosed on his 36th birthday. Stephen Hawking was diagnosed at 21. Most recently, actor Eric Dane announced his diagnosis at age 52.
In the United States, approximately 5,000 new cases of ALS are diagnosed each year, and there are currently around 30,000 people living with the disease. On average, someone is diagnosed with ALS and someone passes away from ALS every 90 minutes.
As a neurosurgeon, I see several patients each year referred to my office for what is thought to be a spine problem, only to ultimately diagnose them with ALS.
Early signs of the disease often include muscle weakness, stiffness, and twitching (called fasciculations) in the arms or legs. Difficulty with balance, frequent tripping, or challenges with fine motor skills such as buttoning a shirt may occur. Other early symptoms can include difficulty swallowing or slurred speech.
As ALS progresses, muscle weakness worsens. Patients often require a cane or walker for mobility. Eating and swallowing become increasingly difficult. Speech becomes harder to understand, leading to communication challenges.
In the later stages of ALS, paralysis of the arms and legs occurs, leaving patients completely dependent on others for care. Eating and swallowing cease altogether, requiring a feeding tube for nutrition. Eventually, breathing is affected, requiring a breathing tube for survival. Without it, death is inevitable.
Ultimately, ALS is a devastating disease that reminds us how fragile and precious life truly is. While there is still no cure, ongoing research continues to offer hope for better treatments and understanding of this complex condition. For those living with ALS, and their families, every day becomes a testament to courage, resilience, and the will to keep moving forward despite unimaginable challenges. As a neurosurgeon, I am constantly reminded of the importance of early recognition, compassionate care, and supporting the search for a cure. Awareness is the first step, because the more we understand, the more we can help.
Impactful Quote of the Week
"However difficult life may seem, there is always something you can do and succeed at”
- Stephen Hawking
Best wishes,
Brian Hoeflinger
P.S. - if you enjoyed this newsletter, you may enjoy my book that details my life as neurosurgeon and the loss of my oldest son, Brian (see below a synopsis).
My Book
Life and Death . . . Two words with such opposite meaning and which inflict such contradictory emotions and yet are so closely intertwined in our lives. As parents, we bring meaning and life into this world through our children. Our lives become defined as a result. We learn the joy, hardship, and responsibility of shaping an innocent life. But a day will come when that life will be taken. For some, death will come too soon. This is the story of my son, Brian Nicholas Hoeflinger, who died unexpectedly at age 18.
https://doctorhoeflinger.com/products/the-night-he-died-the-harsh-reality-of-teenage-drinking
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Medical Trivia Answer:
The correct answer is D) Somatic Nervous System
