Your Brain On... ALS

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Dec 11, 2024

Ammar Al-Chalabi and Merit Cudkowicz, leading experts in ALS research, delve into the complexities of amyotrophic lateral sclerosis. They discuss how ALS impacts the body and brain, highlighting genetic predispositions and emerging environmental risks. The duo explores breakthroughs in gene therapy that may reverse the disease's effects, alongside the role of lifestyle and multidisciplinary care in patient management. They also reflect on the Ice Bucket Challenge's transformative impact on ALS awareness and funding.

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INSIGHT

ALS Explained

ALS damages motor nerves from brain to spinal cord and then to muscles, leading to progressive weakening.
Eye movement and continence muscles remain unaffected even in advanced stages.

INSIGHT

ALS Progression

ALS progression is typically rapid, with diagnosis around one year after symptom onset and death within two years after diagnosis.
However, survival time varies; Stephen Hawking lived for over 50 years post-diagnosis.

INSIGHT

Early ALS Symptoms

Muscle twitching (fasciculations) is an early ALS symptom, but it can have other causes like caffeine or lack of sleep.
Other early signs include muscle wasting and weakness, often manifesting as difficulty with fine motor tasks.

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ALS, a nervous system disease, ruled the headlines in the summer of 2014 thanks to the viral Ice Bucket Challenge. How has our knowledge of the disorder evolved a decade later?

Amyotrophic lateral sclerosis, also known as ‘Lou Gehrig's disease’ (named after the iconic baseball player), is a progressive neurological disorder which breaks down a person’s motor neurons. The main symptoms are muscle weakness and impaired physical function — mild, at first, but in the latter stages of the disease’s progression, essential processes like breathing begin to fail.

Most individuals face a life expectancy of two to five years. A small percentage live five to ten years. And an even tinier group survive beyond a decade. Most famously, renowned physicist Stephen Hawking survived more than 50 years past his diagnosis, in part due to the intensive care he was able to afford.

In this episode of the ‘Your Brain On...’ podcast, we discuss:

• What ALS is, and how it affects the brain and the body

• The onset and progression of ALS, from the earliest symptoms to the end-stage impacts

• How ALS patients are diagnosed, treated, and cared for

• Likely causes of ALS, including genetic predispositions and potential environmental risk factors

• How new technologies are rapidly accelerating our understanding of ALS, especially in genetics

Joining us for this installment of the show are two world-class experts on the disease:

• Professor Ammar Al-Chalabi PhD, Professor of Neurology and Complex Disease Genetics at the Maurice Wohl Clinical Neuroscience Institute at King's College.

• Merit Cudkowicz, Director of the Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital.

‘Your Brain On’ is hosted by neurologists, scientists and public health advocates Ayesha and Dean Sherzai.

‘Your Brain On... ALS’ • SEASON 4 • EPISODE 3

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LINKS

PROFESSOR AMMAR AL-CHALABI

at King’s College: https://www.kcl.ac.uk/people/ammar-al-chalabi

at Motor Neurone Disease Disease Association: https://www.mndassociation.org/get-involved/cure-finders/professor-ammar-al-chalabi

Project MinE: https://projectmine.com/

MERIT CUDKOWICZ

at Massachusetts General Hospital: https://www.massgeneral.org/doctors/16904/merit-cudkowicz

at Harvard University: https://researchers.mgh.harvard.edu/profile/1520993/Merit-Cudkowicz

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ANNOUNCING: NEURO WORLD RETREAT 2025

We’re so excited to share something very close to our hearts, which we’ve been working on over the past few months: our first ever brain health retreat!

You’re warmly invited to join us in San Diego, California for the inaugural NEURO World Retreat 2025, taking place September 2–5, 2025 at the breathtaking Paradise Point Resort.

For more information, and to book, visit: https://neuroworldretreat.com/

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Join the NEURO Academy: NEUROacademy.com

Instagram: @thebraindocs

Website: TheBrainDocs.com

More info and episodes: TheBrainDocs.com/Podcast

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References:

Brown, Robert H., and Ammar Al-Chalabi. "Amyotrophic lateral sclerosis." New England Journal of Medicine 377.2 (2017): 162-172.

Benatar, Michael, et al. "A roadmap to ALS prevention: strategies and priorities." Journal of Neurology, Neurosurgery & Psychiatry 94.5 (2023): 399-402.

Voigtlaender, Sebastian, et al. "Artificial intelligence in neurology: opportunities, challenges, and policy implications." Journal of Neurology 271.5 (2024): 2258-2273.

Zinman, Lorne, and Merit Cudkowicz. "Emerging targets and treatments in amyotrophic lateral sclerosis." The Lancet Neurology 10.5 (2011): 481-490.

Raghav, Yogindra, et al. "Identification of gene fusions associated with amyotrophic lateral sclerosis." Muscle & Nerve 69.4 (2024): 477-489.

Su, Feng-Chiao, et al. "Association of environmental toxins with amyotrophic lateral sclerosis." JAMA neurology 73.7 (2016): 803-811.

Talbott, Evelyn O., et al. "Case-control study of environmental toxins and risk of amyotrophic lateral sclerosis involving the national ALS registry." Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (2024): 1-10.