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Amyotrophic lateral sclerosis and the trajectory of care

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Abstract

This case presentation illustrates ALS progression and accompanying care. Takeaways: Amyotrophic lateral sclerosis care focuses primarily on palliation. Nurses must know how to identify symptoms, recommend treatment, coordinate care, and educate patients and families. An interprofessional team is essential in providing adequate care and comfort to patients and families. Amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disease whose pathophysiology isn't understood, results in upper and lower motor neuron dysfunction. This neural damage leads to muscular atrophy, spasms, and fasciculations (involuntary muscle contractions). Muscle dysfunction begins in one body region then becomes more generalized as the disease progresses, eventually leading to respiratory muscle compromise. Death from ALS respiratory failure typically occurs between 2 to 5 years after symptom onset. (See ALS stats.) ALS stats Amyotrophic lateral sclerosis (ALS) affects people of all races, ethnicities, and genders. The incidence rate is 2 per 100,000 people. Symptom onset typically begins between ages 55 and 75 years. Approximately 90% of ALS cases are sporadic (no environmental or genetic cause). Only 10% of ALS cases are classified as familial or genetic. Most people with ALS have initial symptoms localized to unilateral limbs and are classified as having limb-onset ALS (70%). Those with initial speech and swallowing symptoms are classified as having bulbar-onset ALS (25%). Truncal-onset ALS is rare and may begin with the respiratory muscles (5%). Symptom pattern variations and unknown causes make ALS difficult to identify and treat, and the low incidence rate means that most nurses don't commonly see patients with the disease. A better grasp of clinical manifestations and diagnostic testing may lead to earlier diagnosis and treatment. Nurses can lead this effort by increasing their knowledge of symptom presentation, pharmacologic therapies, and interprofessional interventions. The following case presentation illustrates disease onset, diagnosis, treatment, and interprofessional care. Case presentation Kiran Singh* is a 73-year-old South Asian cis-man. A retired electrical engineer, he's married with two adult children living out of state. Mr. Singh, who's 5' 9", weighs 138 pounds. His past medical history includes coronary artery disease with three stents, chronic obstructive pulmonary disease, immunoglobulin A nephropathy (in remission), hypertension, hyperlipidemia, and heart failure. Initially, Mr. Singh visits his primary care provider because of frequent bilateral lower leg cramps. He describes the cramps as sudden, causing such severe pain that he can't walk. They resolve after 15 to 20 minutes. He says that calf massage and acetaminophen help slightly with the pain. Lab tests show that Mr. Singh's potassium levels are slightly below normal. The provider prescribes oral potassium supplements and recommends that Mr. Singh stay hydrated. Four years later Mr. Singh has increasing, intermittent lower leg cramping that's worse on the left side. He notices that his left leg is much weaker than his right and uses a cane to walk. In addition, Mr. Singh is losing weight; he now weighs 125 pounds. Because Mrs. Singh is his main caretaker, a housekeeper found through elder care services performs light housework and prepares meals. Mr. Singh still drives to local destinations, but his family encourages him to stop. His primary care provider refers him to a neurologist. The neurologist orders a series of tests, which rule out infection, cancer, and peripheral arterial disease. Pulmonary function tests show that Mr. Singh's forced vital capacity is 75% (normal is 80% to 120%). Blood tests indicate high levels of creatinine kinase (CK), and autoantibody tests are inconclusive. A biopsy of the muscle in Mr. Singh's left calf reveals intramuscular inflammation, with muscle atrophy and degenerative changes. Without a definitive diagnosis, the neurologist refers the patient to a neuromuscular specialist. By the time Mr. Singh meets with the neuromuscular specialist, the grip strength in his left hand has grown weaker, he's walking more slowly with a walker, and his voice is softer and slightly slurred when he's tired. His appetite is good, but he now weighs 115 pounds. He also reports shortness of breath with exertion. The specialist's physical examination shows clear muscular atrophy of the left leg and hand compared to the right. To confirm a diagnosis, the specialist orders nerve conduction studies and an electromyography (EMG). The EMG shows abnormal results, including spontaneous electrical activity of the muscles even at rest. After reviewing Mr. Singh's history, progression, lab work, and abnormal EMG, the specialist confirms a diagnosis of lower motor neuron ALS.
Home !Home Page Featured !Amyotrophic lateral sclerosis and the trajectory of care
Web Exclusives
Amyotrophic lateral sclerosis and the
trajectory of care
February 24, 2022
Author(s): By Kaveri M. Roy DNP, RN
This case presentation illustrates ALS progression and
accompanying care.
Takeaways:
Amyotrophic lateral sclerosis care focuses primarily on palliation.
Nurses must know how to identify symptoms, recommend treatment, coordinate care,
and educate patients and families.
An interprofessional team is essential in providing adequate care and comfort to patients
and families.
Amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disease whose
pathophysiology isn’t understood, results in upper and lower motor neuron dysfunction. This
neural damage leads to muscular atrophy, spasms, and fasciculations (involuntary muscle
contractions). Muscle dysfunction begins in one body region then becomes more generalized
as the disease progresses, eventually leading to respiratory muscle compromise. Death from
ALS respiratory failure typically occurs between 2 to 5 years after symptom onset. (See ALS
stats.)
ALS stats
Amyotrophic lateral sclerosis (ALS) affects people of all races, ethnicities, and genders.
The incidence rate is 2 per 100,000 people.
Symptom onset typically begins between ages 55 and 75 years.
Approximately 90% of ALS cases are sporadic (no environmental or genetic cause).
Only 10% of ALS cases are classified as familial or genetic.
Most people with ALS have initial symptoms localized to unilateral limbs and are
classified as having limb-onset ALS (70%).
Those with initial speech and swallowing symptoms are classified as having bulbar-
onset ALS (25%).
Truncal-onset ALS is rare and may begin with the respiratory muscles (5%).
Symptom pattern variations and unknown causes make ALS difficult to identify and treat,
and the low incidence rate means that most nurses don’t commonly see patients with the
disease. A better grasp of clinical manifestations and diagnostic testing may lead to earlier
diagnosis and treatment. Nurses can lead this effort by increasing their knowledge of
symptom presentation, pharmacologic therapies, and interprofessional interventions.
The following case presentation illustrates disease onset, diagnosis, treatment, and
interprofessional care.
Case presentation
Kiran Singh* is a 73-year-old South Asian cis-man. A retired electrical engineer, he’s married
with two adult children living out of state. Mr. Singh, who’s 5’ 9”, weighs 138 pounds. His
past medical history includes coronary artery disease with three stents, chronic obstructive
pulmonary disease, immunoglobulin A nephropathy (in remission), hypertension,
hyperlipidemia, and heart failure.
Initially, Mr. Singh visits his primary care provider because of frequent bilateral lower leg
cramps. He describes the cramps as sudden, causing such severe pain that he can’t walk.
They resolve after 15 to 20 minutes. He says that calf massage and acetaminophen help
slightly with the pain. Lab tests show that Mr. Singh’s potassium levels are slightly below
normal. The provider prescribes oral potassium supplements and recommends that Mr. Singh
stay hydrated.
Four years later Mr. Singh has increasing, intermittent lower leg cramping that’s worse on
the left side. He notices that his left leg is much weaker than his right and uses a cane to
walk. In addition, Mr. Singh is losing weight; he now weighs 125 pounds. Because Mrs. Singh
is his main caretaker, a housekeeper found through elder care services performs light
housework and prepares meals. Mr. Singh still drives to local destinations, but his family
encourages him to stop. His primary care provider refers him to a neurologist.
The neurologist orders a series of tests, which rule out infection, cancer, and peripheral
arterial disease. Pulmonary function tests show that Mr. Singh’s forced vital capacity is 75%
(normal is 80% to 120%). Blood tests indicate high levels of creatinine kinase (CK), and
autoantibody tests are inconclusive. A biopsy of the muscle in Mr. Singh’s left calf reveals
intramuscular inflammation, with muscle atrophy and degenerative changes. Without a
definitive diagnosis, the neurologist refers the patient to a neuromuscular specialist.
By the time Mr. Singh meets with the neuromuscular specialist, the grip strength in his left
hand has grown weaker, he’s walking more slowly with a walker, and his voice is softer and
slightly slurred when he’s tired. His appetite is good, but he now weighs 115 pounds. He also
reports shortness of breath with exertion. The specialist’s physical examination shows clear
muscular atrophy of the left leg and hand compared to the right. To confirm a diagnosis, the
specialist orders nerve conduction studies and an electromyography (EMG). The EMG shows
abnormal results, including spontaneous electrical activity of the muscles even at rest. After
reviewing Mr. Singh’s history, progression, lab work, and abnormal EMG, the specialist
confirms a diagnosis of lower motor neuron ALS.
Diagnosis
Disease onset variations and initial signs and symptoms that mimic other neurodegenerative
diseases make ALS diagnosis difficult. No single diagnostic test exists, so diagnosis is by
exclusion. A thorough history and physical exam must be performed, and lab work, lumbar
puncture, and urinalysis help rule out other conditions. Pulmonary function tests to measure
lung volume, vital capacity, air flow, and gas exchange also should be conducted. If the
provider suspects neuromuscular disease, a muscle biopsy may be ordered. Conduction
studies and EMG can test for abnormal electrical activity of the muscles, resulting in a more
definitive diagnosis. (See Diagnosing ALS.)
Diagnosing ALS
Amyotrophic lateral sclerosis (ALS) is a diagnosis of exclusion. In addition to family and
health histories, the following exams and tests help rule out other conditions.
Physical exam
During the physical exam, look for the following:
Dysphagia
Emotional lability
Rigid muscles
Slurred speech
Unilateral muscle weakness and atrophy
Laboratory tests
Autoantibodies
Basic metabolic panel
B12 level
Complete blood cell count
Creatinine kinase
Heavy metals panel
Hepatitis
HIV (enzyme immunoassay and Western blot)
Lumbar puncture
Parathyroid hormone level
Thyroid hormone level
Thyroid-stimulating hormone level
Urinalysis
White blood cell count
Diagnostic tests
Computed tomography scan
Electromyography
Magnetic resonance imaging
Muscle Biopsy
Nerve conduction
Pulmonary function
Mr. Singh’s elevated CK (due to muscle atrophy), initial left leg muscle wasting and
dysfunction, as well as significant weight loss, all indicate a possible lower motor neuron ALS
diagnosis. Because the muscle biopsy and autoantibody tests aren’t definitive, inflammatory
and autoimmune diseases seem unlikely. Only after the EMG, which shows that the left calf
muscles don’t respond normally to electrical stimulus, does the provider diagnose ALS.
Treatment
Because no cure for ALS exists, treatment focuses on slowing disease progression and
managing symptoms. The only approved medications to slow disease progression are riluzole
(taken orally) and edaravone (administered via infusion). Riluzole is thought to work by
blocking the release and uptake of glutamate, a neurotransmitter that may cause increased
production of astrocytes that harm motor neurons. Edaravone, an antioxidant, is thought to
act as a neuroprotective agent by relieving the effects of oxidative stress on motor neurons.
Route of administration and cost frequently are the deciding factors for patients. Riluzole
costs up to $200 per month even with insurance coverage. Edaravone, which is administered
in 2-week cycles, costs $145,500 per month out-of-pocket, with varying insurance coverage.
Most ALS treatment focuses on symptom management. Weight loss and dysphagia are
addressed with dietary changes and occupational therapy. Percutaneous endoscopic
gastrostomy (PEG) tubes may be recommended to supplement nutritional needs. Studies
suggest that ALS in patients who are malnourished progresses faster than in those of normal
weight or who are slightly overweight. Dyspnea can be treated symptomatically with
morphine, but also with noninvasive ventilation (such as bi-level positive airway pressure
[BiPap]), cough assist machines, and suction. Noninvasive ventilation has been shown to
decrease exertion and slightly increase patient lifespan. (See Pharmacologic symptom
treatment.)
Pharmacologic symptom treatment
In addition to prescribing riluzole or edaravone to slow amyotrophic lateral sclerosis
progression, providers may prescribe the following medications to manage symptoms.
Medication Symptom
Acetaminophen
· Fever
· Mild-to-moderate pain
Antibiotics
· Pneumonia
· Urinary tract infections
Anticholinergics
· Glycopyrrolate
· Secretions (to dry them up)
· Upper respiratory congestion
Antispasmodics
· Tolterodine tartrate · Bladder spasms
Benzodiazepines
· Diazepam
· Lorazepam
· Anxiety
· Insomnia
· Muscle spasms
Eugeroics
· Modafinil · Fatigue
Expectorants
· Enemas
· Guaifenesin
· Congestion
· Secretions (to thin them)
· Laxatives
· Suppositories
· Constipation
Neurotoxin
· Botulinum toxin type A · Severe muscle spasms
Non-steroidal anti-inflammatory
drugs
· Ibuprofen
· Fever
· Mild-to-moderate pain
Opioids
· Morphine
· Dyspnea
· Moderate-to-severe pain
Sedatives
· Zolpidem · Insomnia
Skeletal muscle relaxants
· Baclofen · Muscle spasms
Topical
· Pramoxine-zinc oxide · Pressure injuries
Life with ALS
After his ALS diagnosis, Mr. Singh starts receiving care from a neurology specialist at an ALS
clinic. He’s diagnosed with mild-to-moderate stage ALS and starts taking riluzole. Mr. Singh
goes to the ALS clinic every 10 to 12 weeks and meets with an interprofessional treatment
team (neurologist, palliative care provider, nurse case manager, physical therapist,
occupational therapist, speech therapist, respiratory therapist, registered dietician, and
social worker). He receives physical, occupational, and speech therapy at home, and the
social worker refers him to the local ALS Association chapter for support. Mr. Singh also is
offered aide services, but he declines them because he’s still able to bathe himself with his
wife’s help.
The case manager coordinates services and equipment (including a wheelchair and
commode) delivery for Mr. Singh. The family attends team meetings together via Zoom, and
Mrs. Singh and their children advocate for Mr. Singh.
Initially, Mr. Singh wants to try edaravone infusions to slow ALS progression and prolong his
life. With some difficulty, the case manager receives insurance approval. However, the
clinical protocol requires Mr. Singh to receive infusions in the clinic every day for 2 weeks
before starting home infusions. Trips back and forth to the clinic have become difficult for the
patient, so after speaking with the specialist and palliative care provider, he decides not to
start edaravone treatment. Instead, he opts to preserve his quality of life and comfort.
Mr. Singh continues riluzole but the disease progresses swiftly. His voice weakens and he
begins to experience dysphagia and increased dyspnea. After the speech therapist conducts
a swallow study, the dietician recommends that Mr. Singh’s food be pureed and that he
supplements his meals with Ensure. Pulmonary function tests show that the patient’s forced
vital capacity has dropped to 60%. The neurology specialist orders BiPap, cough assist, and
suction machines. The respiratory therapist teaches Mr. and Mrs. Singh how to use them.
The team is concerned with the patient’s rapid weight loss. They speak with him about his
diet and possible PEG tube placement. As a devout Hindu and strict vegetarian, Mr. Singh
declines the PEG tube. He says he still has a good appetite and doesn’t want such an
invasive intervention. However, the team dietician and his daughters urge him to relax his
dietary restrictions to increase his caloric intake, which he agrees to. Mr. Singh had restricted
his salt and fat intake to manage his hypertension and cardiac disease, but now that he has
advancing ALS, he must consume as many calories as possible.
While reviewing advance directives, Mr. Singh tells the neurology specialist that he wants a
full code, with all resuscitative measures, and he makes Mrs. Singh his healthcare proxy. The
Singh daughters voice concern about making Mrs. Singh healthcare proxy because she has
difficulty expressing herself in English, but Mr. Singh insists. Driving also is addressed. Mr.
Singh feels that his independence is being taken away but recognizes the safety issues. He
agrees to give up driving.
Mr. Singh can still ambulate with a walker, but only for short distances and with great effort.
He’s also still continent but is using urinals and can only walk to the bathroom with help from
a family member or aide. Observing the patient’s sharp decline and advanced symptoms, the
neurology specialist and palliative care provider recommend hospice services. Although
reluctant at first, with family support and education, Mr. Singh agrees.
End-of-life care
Three weeks after starting hospice services, while Mr. Singh is having tea with his wife, he
feels a sudden urge to urinate. When he reaches for his urinal, he falls to the floor and hits
his head. He doesn’t lose consciousness, but he sustains a laceration and a black eye. In
addition, Mr. Singh becomes extremely short of breath with any effort, so the provider
prescribes low-dose morphine to decrease dyspnea.
Mr. Singh begins to have bouts of severe constipation, a common occurrence among ALS
patients. He’s anxious about this and follows a strict bowel regimen, taking laxatives and
stool softeners daily. Suppositories and enemas are administered if no bowel movement
occurs. Eventually, constipation becomes such an issue that the hospice nurses must dis-
impact the patient weekly.
Fortunately, Mr. Singh has only infrequent complaints of pain, most of which are caused by
hemorrhoids and anal fissures resulting from constipation. They’re treated with pramoxine-
zinc oxide and morphine. Occasionally, Mr. Singh reports intermittent back and leg pain,
which is relieved with repositioning.
After his fall, Mr. Singh becomes bed- and chairbound and dependent for all activities of daily
living. He begins to develop Stage 1 pressure injuries on his coccyx, which the hospice
nurses treat with pramoxine-zinc oxide. To prevent further skin breakdown, the hospice
nurse orders a hospital bed with an alternating pressure air mattress and a manual hydraulic
lift. The local ALS association loans Mr. Singh a powered wheelchair.
To help the family cope with the increased care, the social worker recommends an in-home
health agency to provide aides 8 hours a day, 5 days a week. The aides help to reposition Mr.
Singh every few hours, bath and change him, and perform light housekeeping and laundry.
They use a gait belt to help the patient stand and pivot to his wheelchair.
Mr. Singh can no longer feed himself, so his wife purees his food and feeds him. Thickened
fluids allow him to drink by himself with a straw. Despite the risk for aspiration related to
increasing dysphagia, Mr. Singh chooses to keep eating and drinking for comfort and
continues to refuse the PEG tube.
Psychosocial and spiritual issues become more important to Mr. Singh. Confined to a hospital
bed, he’s anxious and depressed, and he’s embarrassed that others are bathing, cleaning,
and feeding him. Mr. Singh is religious and stoic, rarely talking about his emotions. However,
he begins sharing his concerns and end-of-life wishes with his daughters and the hospice
social worker. When offered hospice chaplaincy services, he declines. The social worker
contacts Mr. Singh’s Hindu priest, who provides spiritual support.
Eventually, Mr. Singh asks one of his daughters if God will forgive him if he can’t perform his
usual religious observances. He says he’s afraid and doesn’t want to suffer. His daughter
reassures him that God will forgive him, and that she and her sister will take care of their
mother and look after his affairs. She also assures him that they understood his wishes and
will carry them out.
Thus comforted, Mr. Singh meets with the hospice team and his family and makes his
daughters his healthcare proxies. He provides detailed guidance for his family on the Hindu
funeral rites he wants performed, and he changes his code status, opting not to be
resuscitated, intubated, or taken to the hospital. Mr. Singh declines I.V. therapies and
artificial feeding. The provider prescribes lorazepam for anxiety and insomnia, which helps
the patient sleep.
Outcome
Mr. Singh’s ALS progresses rapidly. Nine months after his diagnosis, he aspirates and
becomes less responsive and eventually comatose. He dies a day later in his home with
hospice services.
Be a strong advocate
ALS care, which primarily focuses on palliation and helping patients adapt to physical
changes, requires a wide range of resources and detailed coordination. Nurses play a critical
role in identifying symptoms, making treatment recommendations, coordinating care, and
educating patients and families. Nurse-led interprofessional care is essential for patient
comfort and should begin as early as possible after diagnosis to ensure appropriate physical,
psychosocial, and spiritual support. Local ALS associations also can provide support,
equipment, and other resources.
Ongoing culturally sensitive conversations with patients and families about quality of life and
advanced directives can help ensure treatment options and the patient’s wishes are
periodically reviewed as the disease progresses. As seen in this case presentation, code
status, treatment choices, and psychosocial and spiritual needs can change throughout the
course of the disease. With increased education and awareness, nurses can serve as strong
advocates and leaders on interprofessional teams caring for patients with ALS.
*Names are fictitious.
ALS resources and equipment
Caring for patients with amyotrophic lateral sclerosis (ALS) requires a variety of
equipment and resources.
ALS
symptoms
Recommended
durable medical
equipment
Suggested
interprofessional
care
ALS
complications
Motor
· Ambulation
difficulty
· Loss of grip
strength
(initially one-
sided, then
progressive
· Muscle
weakness and
wasting
(initially one-
sided, then
progressive)
· Adaptive equipment
(for dressing, feeding,
etc.)
· Bedside table
·
Cane/walker/wheelchair
· Commode
· Gait belt
· Grab bars
· Powered wheelchair
· Shower chair
· Splints/orthotics
· Urinal/bedpan
· Chaplaincy
· Home health
aides
· Hospice care
team
· Housekeeper
· Neurology
provider/ALS clinic
· Nursing
· Occupational
therapist
· Palliative care
team
· Physical therapist
· Registered
· Activities of daily
living
· Anxiety
· Aspiration
pneumonia
· Depression
· Falls
· Nutritional
compromise
· Pain
· Respiratory
compromise
Respiratory
· Decreased
vital capacity
· Dysarthria
· Dyspnea
· Inability to
cough
effectively
· BiPap/CPap machine
· Cough assist machine
· Hospital bed
· Mechanical ventilation
· Suction machine
· Voice
banking/communication
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HOME JOURNAL )CNE CLINICAL TOPICS )RESOURCES )ADVISOR SERIES )COMMUNITY )MAGNET® & PATHWA
*
· Respiratory
failure
banking/communication
device
· Registered
dietician
· Respiratory
therapist
· Social worker
· Speech therapist
· Skin compromise
· Spiritual unrest
· Ventilator-
associated
pneumonia
Weight loss
· Anorexia
· Cachexia
· Dysphagia
· Ensure or other dietary
supplement
· Gastronomy tube
· Liquid thickeners
Skin
· Pressure
injuries
· Hospital bed with air
mattress
· Manual hydraulic lift
with sling
· Wound care supplies
BiPap = bilevel positive airway pressure, CPap = continuous positive airway pressure
Kaveri M. Roy is a distinguished teaching assistant professor and TEAL Fellow at
the MGH Institute of Health Professions in Boston, Massachusetts.
References
ALS Association. Understanding ALS. als.org/understanding-als
ALS News Today. Diagnosis of ALS. alsnewstoday.com/als-diagnosis
Bello-Haas VD. Physical therapy for individuals with amyotrophic lateral sclerosis: Current
insights. Degener Neurol Neuromuscl Dis. 2018;8:45-54. doi:10.2147/DNND.S146949
Burgos R, Bretón I, Cereda E, et. al. ESPEN guideline clinical nutrition in neurology. Clin
Nutr. 2018;37(1):354-96. doi:10.1016/j.clnu.2017.09.003
Hogden A, Foley G, Henderson RD, James N, Aoun SM. Amyotrophic lateral sclerosis:
Improving care with a multidisciplinary approach. J Multidiscip Healthc. 2017;10:205-15.
doi:10.2147/JMDH.S134992
Larson TC, Kaye W, Mehta P, Horton DK. Amyotrophic lateral sclerosis mortality in the United
States, 2011-2014. Neuroepidemiology. 2018;51(1-2):96-103. doi:10.1159/000488891
Long R, Havics B, Zembillas M, Kelly J, Amundson M. Elucidating the end-of-life experience
of persons with amyotrophic lateral sclerosis. Holist Nurs Pract. 2019;33(1):3-8.
doi:10.1097/HNP.0000000000000301
Majmudar S, Wu J, Paganoni S. Rehabilitation in amyotrophic lateral sclerosis: Why it
matters. Muscle Nerve. 2014;50(1):4-13. doi:10.1002/mus.24202
Mazón M, Vázquez Costa JF, Ten-Esteve A, Martí-Bonmatí L. Imaging biomarkers for the
diagnosis and prognosis of neurodegenerative diseases. The example of amyotrophic lateral
sclerosis. Front Neurosci. 2018;12:784. doi:10.3389/fnins.2018.00784
Roy KM. Frequently asked questions about palliative care. Am Nurse Today.2020;15(7).
myamericannurse.com/frequently-asked-questions-about-palliative-care
Sanchez B, Rutkove SB. Electrical impedance myography and its applications in
neuromuscular disorders. Neurotherapeutics. 2017;14(1):107-18. doi:10.1007/s13311-016-
0491-x
Schultz J. Disease-modifying treatment of amyotrophic lateral sclerosis. Am J Manag Care.
2018;24(15 suppl):S327-35.
Vacca VM. Amyotrophic lateral sclerosis: Nursing care and considerations. Nursing.
2020;50(6):32-9. doi:10.1097/01.nurse.0000662348.31823.44
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