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Muscle Weakness, Fatigue and Spasticity (There is no cure for ALS and…

- - - - Environmental factors may affect sporadic ALS
        
        smokers are at higher risk
        
        environmental toxins (more research required here)
        
        miliary service. Patient served in Gulf War
        While not completely understood, military service
        affects likelihood of contracting ALS. Suspected
        due to chemicals, injuries or intense exertion.
    - - Approximately 2 out of every 100,000
        people in the US have ALS.
        Cowboys stadiums holds 100,000
        people as a point of reference.
      - Amyotrophic Lateral Sclerosis got it name as follows:
        A - (not), Myo (muscle) trophic (nourishment)
        Lateral - for the parts of the spinal cord where the
        white matter tracts are found
        Sclerosis - scarring or hardening of the nerves affected
      - In the US, ALS is also known as
        Lou Gehrig's Disease after a famous
        hall of fame baseball great who played
        17 seasons with the New York Yankees.
        Sadly, Mr. Gehrig contracted and
        died from this disease.
      - Stephen Hawking a world famous
        physicist lived with ALS for 55
        years and is considered to most
        certainly be an outlier in life
        expectancy for this disease
      - To raise awareness for this fatal
        and cruel disease, Pete Frates, who had ALS
        began an ice bucket challenge in 2014.
        Through this campaign, $220 million
        was raised for ALS research. In July
        2019 he and his family attended
        a dedication at Boston College to the
        Pete Frates Center, Indoor Training
        Facility.
- - - - Some ascending tracts
        have only 2 orders as they do not
        go beyond the thalamus such as the
        Spinocerebellar tract
      - Spinothalamic tract
        is a good example of an
        ascending neuron with 3 orders
      - 3 neurons
        
        2nd order Neurons
        
        live in spinal cord. These
        are the interneurons in the
        dorsal horn in the gray matter and
        send information to the thalamus
        
        3rd order Neurons
        
        neuron in the thalamus
        that connects with the cerebral
        cortex (to the relevant sensory
        homonculus in the post central
        gyrus of the parietal lobe)
        
        1st order Neurons
        
        has receptors and collects
        information and sends to spinal cord
    - - 2 neurons
        
        Upper Motor Neuron
        
        in the brain or brainstem
        
        Lower Motor Neuron
        
        the neuron that synpses
        with the muscle
      - Through studies, it
        has been determined that
        ALS causes progressive
        degeneration in both the
        upper and lower motor neurons
      - Example #2 is the Vestibulospinal
        Tract. Known as an INDIRECT motor tract
        as the decision to move comes from brain
        stem and is involved in more basic
        involuntary functions to keep you breathing
        and balanced (alive)
      - Example of a descending
        tract is the Corticospinal tract.
        Known as a DIRECT motor
        tract as the movement directions
        come from the brain and are therefore
        voluntary.
  - - - Two basal nuclei disorders
        include:
        
        Parkinson's disease leading to
        tremors
        
        Tourette's syndrome due to
        shorter-lived inactivation of the
        basal nuclei leading to short muscle
        spasms often seen has quick unwanted
        contractions of facial muscles. Can be vocal
    - - Contains the motor
        homunculus which is a
        motor map of the body.
        The size of the body part
        in the motor homunculus
        dictates how many motor
        neurons leave the brain
        to go to that part of the
        body. This dictates
        the variety of movements
        of that body part.
        
        This picture gives a
        good visual of the various
        sizes of body parts found in
        the motor homunculus
- - - - Gyri and Sulci increase the
        surface area of the brain which
        is very important as it allows
        more neurons to be packed
        inside the brain and process
        more information
        
        An important sulcus that separates frontal
        and parietal lobes of the brain and is called
        the central sulcus
        
        Anterior to central sulcus is
        precentral gyrus and posterior is
        the postcentral gyrus
    - - Photo above shows locations
        of lobes with the exception of
        insula which is deep to the
        other lobes and not shown.
        Lobes will be described in further
        detail in physiology section
  - - - Dorsal Gray Horns
        Found on the posterior
        side of the spinal cord
        Contains interneurons
      - Ventral Gray Horns
        Found on the anterior
        side of the spinal cord
        Contains motor neurons
        (somatic)
      - Lateral Gray Horns
        These are lateral to the
        dorsal and ventral gray
        horns
        Contains autonomic
        motor neurons
- - - - Radicava is a second
        drug prescribed and known
        to slow the progression of the
        disease
  - - - Anti-depressants are
        prescribed and these will
        often help with sleep disorders
        as well.
- - - - Photo above shows a good overview
        of neural messages between
        the brain, spinal cord and muscles.
        Please focus on the red line. Upper
        motor neuron begins in the brain and
        travels to spinal cord. Lower motor
        neuron travels from spinal cord to
        skeletal muscle.
        
        Both Upper and lower motor
        neurons appear to be affected by
        ALS.
        
        Eventually muscles die away
        because they are not activated