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                                7. SOD1-related ALS: what has it told us about motor neuron degeneration? - part 2- Prof. Dame Pamela Shaw
 
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                                9. Oxidative stress in amyotrophic lateral sclerosis (ALS) 1- Prof. Dame Pamela Shaw
 
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                                10. Oxidative stress in amyotrophic lateral sclerosis (ALS) 2- Prof. Dame Pamela Shaw
 
Printable Handouts
Navigable Slide Index
- Introduction
- ALS has a metabolic component: weight loss
- ALS has a metabolic component
- ALS mutations impair energy metabolism
- Outstanding questions
- Weight loss occurs years before onset of motor symptoms
- Weight loss is typically observed in ALS mouse models
- Are weight loss and metabolic abnormalities target for ALS treatment?
- Weight loss predicts survival
- High-calorie diet increases survival of ALS mice
- LIPCAL-ALS
- Summary: ALS progression is related to weight loss
- What are the key cell types driving weight loss in ALS?
- Location of the hypothalamus and its nuclei
- Hypothalamic structural alterations
- Hypothalamic volume is correlated to BMI
- Hypothalamic alterations are mostly found in the LHA
- MCH neurons degenerate in the LHA
- Loss of MCH blunts food intake in mutant SOD1 mice
- Pharmacological strategies?
- Summary and conclusions
- Acknowledgment
Topics Covered
- Amyotrophic Lateral Sclerosis (ALS)
- ALS and weight loss
- Progression of ALS
- High-calorie diet
- Hypothalamus in ALS
- Melanin-concentrating hormone (MCH) neurons
- Relationship between MCH neurons, ALS, and weight loss
Links
Series:
Categories:
Therapeutic Areas:
Talk Citation
Dupuis, L. (2023, December 31). Importance of nutrition and weight loss in motor neuron disease [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved October 31, 2025, from https://doi.org/10.69645/VRJZ3110.Export Citation (RIS)
Publication History
- Published on December 31, 2023
Financial Disclosures
- Dr. Luc Dupuis has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
A selection of talks on Neurology
Transcript
Please wait while the transcript is being prepared...
      
      
        
                  0:00
                
                
                  
                    Welcome to the talk
on the importance
                  
                    of nutrition and weight
                  
                    in amyotrophic lateral
sclerosis motor neuron disease.
                  
                    I have a PhD and
                  
                    working as an Inserm
research director
                  
                    in Strasbourg, France.
                  
                
              
                  0:17
                
                
                  
                    Why do we say that weight
loss is important in ALS?
                  
                    Because ALS has actually
                  
                    a component that is
related to metabolism.
                  
                    Weight loss in ALS is
caused by multiple factors,
                  
                    but two of these are actually
the most important ones.
                  
                    One is decreased food intake,
                  
                    which is particularly
                  
                    related to dysphasia
that occurs during
                  
                    disease progression
and the other one
                  
                    is increased energy expenditure-
                  
                    both of them might be
                  
                    related to altered
hypothalamic signaling.
                  
                    I hope that in this talk
                  
                    I will convince you that the
hypothalamus is actually
                  
                    instrumental in weight loss in
                  
                    ALS patients and that
dietary intervention and
                  
                    in particular high caloric,
high-fat diet might
                  
                    be important in terms of
therapeutic counseling.
                  
                
              
                  1:14
                
                
                  
                    Another component of ALS is
                  
                    related to energy metabolism
and is actually systemic.
                  
                    There are pathological
abnormalities in ALS tissues
                  
                    beyond the classical origins of
the CNS that are involved in ALS.
                  
                    For instance, the
hypothalamus is atrophied,
                  
                    there are TDP-43 aggregates,
                  
                    especially in the lateral
hypothalamic area,
                  
                    and there are impairments of
                  
                    various hypothalamic signaling,
including melanocortin.
                  
                    In the pancreas,
                  
                    there have been reports of
impaired insulin secretion.
                  
                    In the adipose tissue,
                  
                    the tissue has been
atrophied and is abnormally
                  
                    distributed as compared
to healthy controls.
                  
                    Skeletal muscle shows
increased lipid usage,
                  
                    impaired glucose usage, and
mitochondrial dysfunction.
                  
                
               
       
    











 
                    
                     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
     
        
      
    