The document discusses amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. ALS is a progressive neuromuscular condition that affects motor neurons in the brain and spinal cord. Key symptoms include muscle weakness, atrophy, spasms, and increased reflexes. While sensory and cognitive functions remain intact, over time patients have increasing problems with movement, swallowing, speaking and breathing. There is currently no cure for ALS, though treatments aim to reduce symptoms and multidisciplinary care can help prolong life expectancy and quality of life. Occupational therapy focuses on maintaining independence through adaptive equipment and strategies.

1. Conditions in Occupational Therapy
Jonathan Alonso & Michael Muñoz

2.  ALS also known as Lou Gehrig’s Disease or Motor Neuron
Disease (MND).
 ALS was first described in 1869 by Jean-Martin Charcot, a
French neurologist.
 And was brought to national attention once baseball
legend, Lou Gehrig was diagnosed with the disease in
1939.

3.  It is a life threatening disease that negatively affects
millions of people each year.
 It is a progressive neuromuscular condition
characterized by muscle weakness
(hypotonicity), muscle wasting (atrophy), muscle
twitching (fasciculation), and increase reflexes
(hyperreflexia) (Aebischer & Kato, 2007).
 The progression of the disease is different from one
person to the next.
 Important! This a motor neuron disease;
sensory, executive functions, and intellect remain
intact.

4.  The cause of ALS remains unknown. Theories to the cause
of such disease are thought to be
genetic, viral, autoimmune disorder.
 Enzyme deficiency, environmental factors, and neurotoxic
hypothesis (Benot-Abreu, Damme, Van Den Bosch &
Robberecht).
 Research postulates concern over neurotoxicity relating to
abnormalities of essential neurotransmission
anions, calcium and glutamate, entering the
neuron, damaging the cell metabolism, and resulting in
pathological changes (Wijesekera & Leigh, 2010).

5.  A report on incidence shows that ALS on average affects 1.89
per 100,000 a year and prevalence average of 5.2 per 100,000
(King, Duke, & O’Conner, 2009).
 The mean age of onset for ALS is about 60 yrs, with a slight
male prevalence male/female ratio 1.5:1
 Approximately 2/3 of patients with typical ALS have a spinal
form of the disease where the symptoms may start either
distally or proximally in the UE and LE (King, Duke, &
O’Conner, 2009).
 Most common motor neuron disorder in adults (Atchison &
Dirette, 2007).

6. Depend on the location of the disease ALS divides
into three areas: lower motor neuron, corticospinal
tract, and corticobulbar tract dysfunction
Regardless the part of the body first affected by the
disease, muscle hypotonicity and atrophy spread to
other somatic effectors as the disease progresses
(Atchison & Dirette, 2007).
Patients have increasing problems with
moving, swallowing (dysphagia), and speaking or
forming words (dysarthria) (Wijesekera &
Leigh, 2010).

9.  Patients must have signs and symptoms of both
upper and lower motor neuron damage, that’s not
attributed to other causes.
 The process consists of a history and physical
exam, repeated at regular intervals, to document
hyperreflexia, fasciculation, and upper and lower
movement (Bento-Abreu, et. al, 2010).
 MRI tests document denervation and distinguish
benign fasciculation from those of ALS (Atchison
& Dirette, 2007).

10.  Currently there is no known medical cure to alter the fatal
progression of ALS.
 Gradual death 1-5 years from diagnosis due to
respiratory problems, though course is progressive and
rapid.
 Riluzole remains to be the only compound licensed for
use since it reduces damage to motor neurons by
decreasing the release of glutamate and modifies the
rate of evolution (Corcia & Meininger, 2008).
 Anti-inflammatory medications
 Antispasmodic
 Non-invasive ventilator support
 Multidisciplinary teams (including OT) may increase
quality and length of life.

11.  Occupational Therapy provides a longer duration and
better quality of life (QOL).
 Providinghope for patients retaining purpose in
occupational activity (Corcia & Meininger, 2008)
 Prescribingappropriate equipment, which helps with
functional independence within areas of occupation
(AOTA, 2008) .

12.  Assistive technology and equipment commonly
recommended to minimize energy output and improve
performance with self care tasks and other ADLs:
 Reachers
 Dressing sticks
 Long handle shoehorns
 Long handle sponges
 Buttonhooks
 Shower seats
 Three-in-one commodes

13. http://www.youtube.com/watch?v=teMElxrV-iw&feature=youtube_gdata_player

14.  The cause of ALS remains unknown and most common motor
neuron disorder in adults
 Important! This a motor neuron disease; sensory, executive
functions, and intellect remain intact.
 Symptoms include: muscle
spasticity, hypotonicity, atrophy, cramps, fasciculation and
hyperreflexia. Also, dysphagia, dysarthria, and an overactive gag
reflex.
 Treatments include: Anti-inflammatory and Antispasmodic
medications. Also, non-invasive ventilator support
 Multidisciplinary teams (including OT) may increase quality and
length of life.
 Death from respiratory failure, 1-5 years after Diagnosis due to rapid
and progressive nature of the disease.

15.  Aebischer, P., & Kato, A. C. (2007). Playing defense against Lou Gehrig’s disease. Scientific
American, 297(5), 86-93. Retrieved from EBSCOhost.
 American Occupational Therapy Association. (2008). Occupational therapy practice
framework: Domain and Process (2nd ed.). American Journal of Occupational Therapy, 62,
625-683
 Atchison, B. J., & Dirette, D. K. (2007). Conditions in Occupational Therapy (3rd ed., pp.
268-271). Baltimore, MD: Lippincott Williams & Wilkins.
 Bento-Abreu, A., Van Damme, P., Van Den Bosch, L., & Robberecht, W. (2010). The
neurobiology of amyotrophic lateral sclerosis. European Journal of Neuroscience, 31(12),
2247-2265. doi:10.1111/j.1460-9568.2010.07260.x
 Corcia, P., & Meininger, V. (2008). Management of amyotrophic lateral sclerosis. Drugs,
68(8), 1037-1048. Retrieved from EBSCOhost.
 King, S. J., Duke, M. M., & O'Connor, B. A. (2009). Living with amyotrophic lateral
sclerosis/motor neurone disease (ALS/MND): decision-making about ‘ongoing change and
adaptation’. Journal of Clinical Nursing, 18(5), 745-754. doi:10.1111/j.1365-
2702.2008.02671.x
 Wijesekera, L. C., & Leigh, P. N. (2009). Amyotrophic lateral sclerosis. Orphanet Journal of
Rare Disease, 4(3), 1-22. doi:10.1186/1750-1172-4-3