Pathology
Amyotrophic lateral sclerosis or ALS is a motor neuron neurodegenerative disease that, like
Alzheimer’s, is also associated with cognitive impairments as there are
overlaps between ALS and frontotemporal degeneration. The causes for this
disease are not exactly known. Although there are known genetic and
environmental factors that lead to this disease, the interaction of genetic and
environmental exposure can vary individually. You might ask how the interaction
between the two leads to ALS. Well, there are certain genes (we carry them all
along our lifetime) which are acted on by time and environmental exposure.
These continue to have their effects until a certain point in time when the
process of neurodegeneration begins and symptoms appear.
Clinical presentations are divided into bulbar or spinal-onset and
depend on the degree of upper motor neuron or lower motor neuron involvement.
In the bulbar onset the disease affects speech and swallowing (dysarthria and
dysphagia) in patients and progresses to limb symptoms. The bulbar onset
represents approximately a third of ALS cases. The other two thirds of cases
have a spinal form of ALS that begins with skeletal muscle wasting and
weakness.
Upper motor neurons transmit motor info from the brain, cerebral
cortex, down to the spinal cord. Degeneration of these neurons leads to
spasticity, weakness, and minimal muscle atrophy. Lower motor neurons extend
from the spinal cord and carry motor information to skeletal muscles for their
movement. Damage to these neurons would lead to ‘disabling weakness’ and
absence of tendon reflexes.
There are different mechanisms that can lead to ALS. For instance,
like in Alzheimer’s disease there are specific proteins that get aggregated
inside neurons that cause impairments and neural degeneration. And as soon as
neurons stop working, muscle atrophy (or loss of muscular tissue) begins.
Mechanisms that lead to ALS:
1. Mitochondrial dysfunction
2. Glutamate excitotoxicity
3. Oxidative stress
4. Protein aggregates
5. Accumulation of neurofilaments
6. Neuroinflammation
Research
ALS has become a more well-known condition to the general public
over the last few years due to initiatives like “The Ice Bucket Challenge”.
However, research into the effects that physical activity has on this disease
has been occurring for a long period of time. Systematic reviews into this
topic have found that when physical exercise is conducted in the early stages
of ALS, many people gained both physiological and psychological boosts and that
there were significant benefits in terms of quality of life (Almeida et al.
2012). Furthermore, when other systematic reviews combined the results of the
studies they were investigating, they found that for those with ALS who
underwent exercise protocols had significantly improved scored in the
amyotrophic lateral sclerosis functional rating scale or ALSFRS (Bella-Haas et
al. 2013). The ALSFRS is simply a test that analyzes the functional status of
patients with ALS (Coco et al. 2007). This means that the higher an individual
scores on this test, the greater their functional status is. A case study
following a 56-year-old woman with ALS who performed resistance training 6 days
a week for 7 and a half weeks, saw that resistance exercises to the upper
extremities improved strength in 14 muscle groups (Bohannon, 1983). In
addition, a specific study involving 25 patients with a mean age of 60,
underwent an exercise program in which a therapist moved all four limbs for a
total of 15 minutes twice a day (Dory et al. 2001). After three months of this
exercise program, patients that underwent it showed less deterioration, but
that these benefits started to disappear the longer the program was conducted
(Dory et al. 2001). This potentially shows that although physical activity can
help individuals with ALS over the short term, it cannot reverse the condition
or stop it from progressing.
Therefore, physical activity programs performed
both by the individual or a therapist, seems to hold potential benefits for
those with ALS.
Exercise
Get proper care.
Although every other condition we have spoken
about so far on this blog is extremely impactful and that everyone with such
conditions should get the help of medical professionals, when it comes to ALS
it is critical that healthcare providers are contacted to help get someone
physical active. As we have seen in one of the research articles used in today’s
post, even a therapist moving the limbs of a person with ALS can hold potential
benefits for that individual. Therefore, if I person is not able to walk or
function on their own, they may still be able to improve from exercise.
However, in order for this to be possible, a healthcare professional needs to
be employed and this person needs to not only be one that you trust, but also
one that the person with ALS enjoys being around.
**Don’t forget to check out this week's “Interesting Reading of the
Week '' located in the sidebar.
**Please note that the exercise
related programs from the studies are not the only effective training modality
that produce benefits. If you believe that exercise will help you or someone
you know, please contact a registered healthcare professional for more
information.
**For more detail on this topic or to recommend future content,
please email us.
Articles referenced
Bohannon, R. W. (1983). Results of resistance exercise on a
patient with amyotrophic lateral sclerosis: a case report. Physical Therapy,
63(6), 965-968.
Drory, V. E., Goltsman, E., Reznik, J. G., Mosek, A., &
Korczyn, A. D. (2001). The value of muscle exercise in patients with
amyotrophic lateral sclerosis. Journal of the neurological sciences, 191(1-2),
133-137.
Coco, D. L., Marchese, S., La Bella, V., Piccoli, T., & Coco,
A. L. (2007). The amyotrophic lateral sclerosis functional rating scale
predicts survival time in amyotrophic lateral sclerosis patients on invasive
mechanical ventilation. Chest, 132(1), 64-69.
Dal Bello-Haas, V., & Florence, J. M. (2013). Therapeutic
exercise for people with amyotrophic lateral sclerosis or motor neuron disease.
The Cochrane database of systematic reviews, 2013(5), CD005229.
https://doi.org/10.1002/14651858.CD005229.pub3
de Almeida, J. L., Silvestre, R., Pinto, A. C., & De Carvalho,
M. (2012). Exercise and amyotrophic lateral sclerosis. Neurological Sciences,
33(1), 9-15
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