This presentation is a synopsis of Amyotrophic Lateral Sclerosis based on the perspective of the movie; "The theory of Everything". This is part of the online course "Understanding the Brain: The Neurobiology of Everyday Life" with the University of Chicago through Coursera.

1. Amyotrophic
Lateral
Sclerosis (ALS)

2. My presentation is a synopsis of Amyotrophic Lateral Sclerosis (ALS)
based on the biographical romantic drama titled the "Theory of Everything"
(2015) on the life story of renouned theoretical physicist, who lived with
ALS; Stephen Hawking.
I chose this as my example as watching this movie played a major role in
the development of my interests in neurobiology/neuroscience.
The information provided in this presentation is based on research and
academic articles. All information provided are paraphrased and cited.

4. What is ALS?
(Lou Gehrig's Disease)
ALS was previously considered a
motor neuron disease characterised
by gradual
deterioration/degeneration and
death of motor neurons essential for
controlling voluntary muscle
movement (1).
ALS is now considered a
neurodegenerative disorder that
affects multiple parts of the nervous
system* (4).
As was discussed in Week 7 by
Professor Mason, the link between
upper and lower motor neurons and
the types of voluntary muscle
movements they are responsible for
are most affected in ALS (1) (2).
The symptoms of ALS are progressive
and hence, worsens overtime. There is
no cure for ALS, but there are
treatments available that helps to
alleviate and treat some of the
symptoms (1) (2).
*However, based on my example, I plan to use the initial description of ALS in this presentation and will
discuss some aspects of new developments in ALS in slide 9-1 1 (4).

5. The parts of the nervous system most affected in people with ALS are the
upper motor neurons (UMN) and lower motor neurons (LMN).
The upper motor neurons present in the cerebral cortex sends information to
the lower motor neurons in the spinal cord and motor nuclei of the brain (1).
The LMN present in the brainstem are involved in controlling muscle
movements related to swallowing, chewing and speaking. The LMN present
in the spinal cord are responsible for muscle movement of the limbs (walking,
gait and posture) (2) (3).
The nervous system and ALS (The
negative)

6. When the upper and lower motor
neurons degenerate, die and stop
sending messages to the muscles, over
time the muscles starts to twitch
(fasciculations), cramp, become
stiff/tight (spasticity) and waste away
(muscle atrophy) (2) (3). This causes loss
of voluntary movements involved in
walking, talking/word fomulation
(dysarthria), chewing, swallowing
(dysphagia) and breathing (dyspnea) (1).
Figure1. Upper and lower motor neuron functions (3)
What happens to the nervous
system in ALS ?

7. This was portrayed in the movie "The Theory of Everything" where the
progression of the disease in the character of Stephen Hawking (Eddie
Redmayne) initially started with twitches, muscles spams and muscle
stiffness. He had difficulty writing, buttoning his shirt and after some time
even talking. Over the course of the movie (which was illustrating the
characters' life over the years), there was a noticeable reduction in the
muscle mass of the character along with illustrations of slurred speech,
inability to chew and swallow and eventually loss of limb functions.
This indicates that the upper and lower motor neurons present in the
characters' cerebral cortex, brainstem and spinal cord were
progressively deteriorating and dying over the years.
The nervous system and ALS (The negative)

8. Good News!!!
Some other functions of the cerebral cortex involving higher mental
processes are usually intact in individuals with ALS (2) (3) (5)
These are :
Cognitive abilities (intelligence, reasoning, problem solving and
understanding), Sphincter control (medulla oblongata), sensory functions and
skin integrity (2).
The nervous system and ALS (The positive)

9. Based on the depiction of the movie and his own biography, despite having
ALS, Stephen Hawking managed to become one of the worlds most
renowned theoretical physicist and a pioneer in Astrophysics. Furthermore,
some of his accomplishments and feats were done after his diagnosis and
disease progression.
This is the focal point of the story being told in the movie
"The Theory of Everything".
"In my opinion, one should concentrate on activities in which one's physical
disability will not present a serious handicap" - Stephen Hawking
The nervous system and ALS (The positive)

10. The nervous system and ALS (The positive)
This showed that other parts of the cerebral cortex related to cognitive
functioning were still intact in the character of Stephen Hawking (and
clearly it was the same case in real life as well). The movie also showed
that the ability to feel emotions were also intact even if the ability to
embody and express emotions were not due to inability to initiate
movement.
However, the autonomic nervous system (both sympathetic and
parasympathetic ANS) is still active in an individual with ALS, but their
ability to voluntarily act on, for example, arousal is impaired (6).

11. The movie also showed that the individual was also able to recall the names of
his friends, colleagues and wife, hence his implicit memory functions controlled
by his hippo campus were also intact, however, this excludes motor memory
which are found to be impaired in ALS (6) .
As most individuals with ALS retain much of their higher cognitive functions (1)
(6), they are aware of their decline in motor function and a a result are
sometimes subject to experiencing anxiety and depression (1), this was also
displayed in some of the scenes in the movie.
This can suggest the importance of psychological support for patients with
ALS and their caregivers (6).
The nervous system and ALS (The positive)

12. [1] "Amyotrophic Lateral Sclerosis (ALS) Fact Sheet", NINDS, Publication date June 2013. NIH Publication No. 16-916
[2] A. D. Walling, M.D., University of Kansas School of Medicine, Wichita, Kansas Am Fam
Physician. 1999 Mar 15;59(6):1489-1496.
[3] De C M, Swash M. Amyotrophic lateral sclerosis: an update [J]. Current Opinion in Neurology, 2011, 24(5):497-503.
[4] Caga J, Hsieh S, Lillo P, Dudley K and Mioshi E (2019) The Impact of Cognitive and Behavioral Symptoms on ALS
Patients and Their Caregivers. Front. Neurol. 10:192. doi: 10.3389/fneur.2019.00192.
[5] C. Crockford, J. Newton, K. Lonergan, et al. ALS-specific cognitive and behavior changes associated with advancing
disease stage in ALS. Neurology 2018;91;e1370-e1380 Published Online before print September 12, 2018. DOI
10.1212/WNL.0000000000006317
[6] S. M. Gillingham, Y. Yunusova, A. Ganda, E. Rogaeva, S. E. Black, D. T. Stuss & L. Zinman (2017) Assessing cognitive
functioning in ALS: A focus on frontal lobe processes, Amyotrophic Lateral Sclerosis and Frontotemporal
Degeneration, 18:3-4, 182-192, DOI: 10.1080/21678421.2016.1248977
References

13. Thank you for reading and reviewing my presentation