RUNNING HEAD: AMYOTROPHIC LATERAL SCLEROSIS

Amyotrophic Lateral Sclerosis

Anne McFadden
The Pennsylvania State University

AMYOTROPHIC LATERAL SCLEROSIS

Abstract
Amyotrophic Lateral Sclerosis is a neuromuscular condition in which the upper and
lower motor neurons of the body degenerate gradually. As the motor neurons begin to deteriorate
the individual begins to lose voluntary control of those muscles. The condition evolves to the
point where individuals become increasingly weak and immobile with progressive paralysis. In
addition to paralysis, a number of internal systems are also affected. Swallowing and speaking
becomes difficult and mental and cognitive characteristics can be impaired as well. The cause of
ALS is unknown and unfortunately there is no cure. The survival rate for patients is
approximately three to five years depending on how the disease progresses. Due to dramatic
changes in the individuals physical, mental and emotional health there are a number of
accommodations one can utilize to improve quality of life.

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Overview
Amyotrophic Lateral Sclerosis (ALS) also termed Lou Gehrigs disease is a
neuromuscular condition that is characterized by the gradual degeneration of motor neurons. The
term Amyotrophy refers to the atrophy of muscle fibers leading to weakness of affected
muscles and visible muscle fibers. Lateral sclerosis, indicates the hardening of the anterior and
lateral corticospinal tracts as motor neurons in these areas degenerate (Wijesekera & Leigh,
2009). These motor neurons are nerve cells that transmit impulses to the muscles, so when these
neurons are damaged it makes it difficult to control voluntary muscle function (Falvo, 2014). The
initial manifestations of this disease may be subtle, however, as it progresses and begins to affect
the chest and the diaphragm, individuals may notice trouble breathing.

Diagnosis
According to the article Amyotrophic lateral sclerosis, found in the Orphanet Journal
of Rare Diseases, the diagnosis of ALS is based on the presence of very characteristic clinical
findings in conjunction with investigations to exclude ALS-mimic syndromes (Wijesekera &
Leigh, 2009). In other words, physicians are not searching for anything specific but rather
looking for signs suggestive of upper and lower motor neuron degeneration that cannot be
explained by any other disease process along with progression compatible with a
neurodegenerative. An electromyogram is conducted to confirm the presence of lower motor
neuron disease and to help exclude possible mimicking disorders (Gordon, 2012). An
electromyogram measures the electrical activity of muscles when in use and at rest as well as
how fast the nerves can send electrical signals. The patients health history also must be taken

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into account in addition to ruling out other conditions to which these manifestations can be
accredited.

Cause and Incidence Rate

Currently there is no specific cause of ALS, although the existing theory suggests that
genetic, viral, autoimmune and neurotoxic factors contribute to its manifestation. The CNS
Drugs Academic Journal states, the causes of ALS have proved difficult to identify, in part
because the disease is rare, with an incidence of approximately two per hundred thousand a year
in Western countries (Gordon, 2012). Most cases occur between fifty-five and sixty five years
of age and this disease slightly affects more males than females. Although there is no specific
cause, five percent of cases reported have a positive family history for amyotrophic lateral
sclerosis. Between ten to twenty percent of those cases have mutations in a certain gene on
chromosome twenty-one. Other connections have also been reported in people with familial
ALS; however, the genetic lesions in these cases are still unknown (Mitchell & Borasio, 2007).

Manifestation
As stated above manifestations of amyotrophic lateral sclerosis are gradual. The disease
begins with limb weakness in about sixty five percent of patients. Some early symptoms include
foot drop, difficulty walking, and loss of hand dexterity or shoulder weakness (Gordon, 2012).
As the muscles begin to degenerate, muscle atrophy occurs and there may be spasticity with
exaggerated reflexes. The condition progresses to the point where individuals become
increasingly weak and immobile with progressive paralysis. In addition, one may have difficulty
swallowing and increased difficulty forming words and sentences (Falvo, 2014). This inhibits

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their ability to aspirate food or liquids as well as communicate with others. Cognition and mental
ability is also impaired in twenty five to fifty percent of patients. These abnormalities include
changes in personality, language, judgment, decision-making and affect. Dementia is another
common comorbidity in fifteen percent of patients. The development of this disease on top of
ALS is known to lower survival rates due to compliancy with interventions and therapy (Gordon,
2012). The survival rate for this disease tends to be around three to five years with about four
percent of patients surviving past ten years ((Wijesekera & Leigh, 2009). This disease
singlehandedly takes over the body to the point where one no longer can function.

Psychosocial and Functional Capacity

Individuals diagnosed with ALS are forced to depend on others to function. Due to loss of
function physically, cognitively and intellectually it is impossible for a patient to live
independently with this disease. That being said, ALS can take a toll on an individuals emotional
health. Depression and anxiety are common in this disease and many feel a sense of hopelessness
especially after being diagnosed. The CNS Drugs Academic Journal reports that anxiety can
accompany symptoms of respiratory insufficiency and depression may lead to reduced appetite,
poor sleep and impaired ability to make decisions (Gordon, 2012). These emotional burdens
along with physical barriers make it difficult for one to attend school, work, socialize, or even
engage in their daily routine.

Vocational Implications
As ALS progresses and muscle function deteriorates it makes it difficult for an individual
to continue to obtain employment. As stated the survival rate tends to be around three to five

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years so after diagnosis the disease begins to develop more rapidly. It gets to be extremely
difficult to be employed once an individuals condition progresses to a certain degree. When one
can no longer communicate or navigate around the workplace it makes it impossible to perform
required tasks. However, for an individual with ALS who is still employed it is important to have
a wheelchair accessible in the work environment even if they are still mobile. Also, if there are
any physical demands they should be light and sedentary due to the gradual degeneration of the
individuals muscle function. Luckily as new interventions emerge that may slow the progress of
this disease and prolong survival, the ability to maintain competitive employment also increases
(Falvo, 2014).

Accommodations
Unfortunately there is currently no cure to amyotrophic lateral sclerosis. There are,
however, methods to slow the progression and accommodations to help individuals live with this
disease. As stated immobility and progressive paralysis are probable outcomes of ALS and
require the individual to heavily depend on others. The article The Role of Physical Therapy
and Occupational Therapy in the Treatment of ALS states that when assistance becomes
necessary, there are devices to help the caregiver transfer the patient safely and efficiently. A gait
belt is one example of these devices and allows the caregiver to provide lifting assistance without
putting stress on weak shoulder muscles. Another transfer device is a pivot disk, which can be
useful if the patient loses their ability to turn around. The patient sits with their feet on top of
two discs and when they stand the caregiver is able to turn the patient with the top disc while the
bottom remains still. Mechanical lifts are also another common device that is helpful when
transferring an individual with ALS. A U-shaped sling or butterfly sling has been proven to

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decrease stress and helpful when one needs to use the restroom. It is important the caregiver be
trained in the use of any of these devices so that the execution is safe and effective (Lewis &
Rushanan, 2007).
Physical therapists (PT) and occupational therapists (OT) can play a dramatic role in
improving the quality of life for an individual with ALS. These two interventions are often
prescribed to individuals struggling with daily routines or muscle function. Also stated in the
article The Role of Physical Therapy and Occupational Therapy in the Treatment of ALS, the
main goal of PT and OT is to maintain independence with functional mobility and activities of
daily living. These therapists work with the patients towards goals as well as assess needs for
equipment, prescribe appropriate exercises, prevent complication and eliminate or prevent pain.
In a patient suffering with the deterioration of the functions of their body, achieving these goals
through adaptive techniques and equipment can greatly improve quality of life. Occupational and
physical therapists have a number of different forms of equipment that can help slow the
progression of this disease and enhance independence. The use of their services can help with
travel, home, work, and environmental accommodations (Lewis & Rushanan, 2007).
Medication has been proven effective at slowing the progression of some symptoms in
ALS patients. However, medications come with a cost, and a very pricey one at that. According
to the book, Amyotrophic Lateral Sclerosis: A Guide for Patients and Families, in an attempt to
control costs many states have passed legislation that allows pharmacies to fill prescriptions with
the generic correspondent. Also insurance companies along with health maintenance
organizations (HMOs) and federal programs have developed formularies. A formulary is a
restrictive list of available medications from which the physicians can chose. An individual with
ALS may still receive drugs that are not on the approved formulary; however, they will most

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likely have to pay from them. That being said these formularies are much more cost effective for
a patient needing multiple medications to manage this disease (Mitsumoto, 2009).
Communication skills are severely affected in patients with progressive ALS so they need
to develop alternative ways to interact with others. Augmentative communication is the use of
effective strategies that help generate verbal or nonverbal messages in addition to or as an
alternative to speech. Some of these strategies may include spelling messages, pointing to items
on a communication board with a list or picture symbols, or using a computer that speaks. There
are also a number of technologies referred to as Augmentative Alternative communication
(AAC) devices that help to aid the patients communication skills. These devices can be low
technology such as handmade picture boards and notecards or high technology such as
alternative keyboards and speech generating devices. Whether low or high tech AAC devices can
allow the individual to remain interactive and engage with others (Mitsumoto, 2009).
Being diagnosed with amyotrophic lateral sclerosis is a life-changing event, not only for
the individual but also for their family and loved ones as well. It impacts every aspect of the
individuals life and their family is soon to be the one caring for them as they slowly lose ability
to function. You may ask yourself, how is it possible to stay strong and hopeful after a diagnosis
like this? To answer this question, one way that individuals and their families cope with this is
through ALS support groups. According to the book, Amyotrophic Lateral Sclerosis: A Guide for
Patients and Families, these groups have been active for more than twenty-five years. They have
provided an opportunity for people with ALS, family members, friends and the community to
become more aware and educated about this illness (Mitsumoto, 2009). These types of groups
help promote communication among participants and offer the opportunity to meet others

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struggling with this illness. These support groups have been known to be a safe and supportive
environment, which can be very therapeutic for individuals and their families.

Conclusion
Amyotrophic Lateral Sclerosis is a rare neuromuscular disease that gradually takes
control over the functions of the body. Due to the rate of decline, an individual with this
diagnosis is only expected to live a couple years. This can be a devastating diagnosis not only for
the patient but their family and loved ones as well. Luckily there are accommodations to help
prolong the rate of deterioration and promote quality of life. Transfer devices, therapy services,
formularies, augmentative alternative communication devices, and support groups are just to
name a few. These measures can help manage the physical, mental and emotional aspects of this
disease and lessen some of the financial burden as well. It is important that through managing
this disease the patient and their family remain positive and optimistic.

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10

References
Falvo, D. (2014). Neurodegenerative and Neuromuscular Conditions. Medical and Psychosocial
Aspects of Chronic Illness and Disability, (pp.121-134). Massachusetts: Jones & Bartlett
Learning.

Gordon, P. (2012). Amyotrophic Lateral Sclerosis: Pathophysiology, Diagnosis and Management.

CNS Drugs, vol. 25(1), pp. 1-15. 10.2165/11586000-000000000-00000

Lewis, M., & Rushanan, S. (2007). The role of Physical Therapy and Occupational Therapy in
the Treatment of Amyotrophic Lateral Sclerosis. NeuroRehabilitation, vol. 22(6), pp. 451-461.
Mitchell, J., & Borasio, G. (2007). Amyotrophic Lateral Sclerosis. The Lancet, vol. 369(9578),
pp. 2031-2041.

Mitsumoto, H. (3rd Ed.). (2009). Amyotrophic Lateral Sclerosis: A Guide for Patients and
Families (vols. 1-5). New York, NY: Demos Medical Publishing

Wijesekera, L., & Leigh, P. (2009). Amyotrophic Lateral Sclerosis. Orphanet Journal of Rare
Diseases, vol. 4(3). DOI: 10.1186/1750-1172-4-3