Download Grown Up...©

Grown Up...
©
A Newsletter For Those Who Care For
ADOLESCENTS, ADULTS, and AGING ADULTS
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Volume 16, Issue 6
June 2011
Editor-in-Chief: Mary Myers Dunlap, MAEd, RN
BEHAVIORAL OBJECTIVES
AFTER READING THIS NEWSLETTER THE LEARNER
WILL BE ABLE TO:
1.
Describe the pathophysiology, etiology, and clinical
manifestations of ALS.
2.
Discuss management of ALS, including implications for
the healthcare provider.
Amyotrophic lateral sclerosis (ALS), or Lou Gehrig's
disease, is a progressive, chronic neuromuscular disease
that attacks nerve cells and pathways in the brain and spinal
cord causing muscles to deteriorate. The affected nerves
cells are those responsible for providing electrical stimulation
to the muscles, which allows for voluntary movement.
Without the necessary electrical
stimulation, the muscles begin to
atrophy and eventually become
paralyzed. ALS is a terminal illness with
death usually resulting in respiratory
infections or complications. More than
half of ALS patients die within 3 years of
the first symptoms and 90% within 5
years.
ALS was first described in 1869 by
Jean-Martin Charcot. Lou Gehrig first brought national and
international attention to the disease when he abruptly
retired from the New York Yankees in 1939. Today,
approximately 5,000 new cases of ALS are diagnosed each
year in the United States. ALS usually occurs between 40
and 70 years of age, with the incidence increasing with each
decade. The average age of onset is age 55. The male to
female ratio is 1.3 to 1.5 but approaches 1 to 1 at ages older
than 70 years. The disease has no racial, socioeconomic, or
ethnic boundaries.
This newsletter will describe the pathophysiology,
etiology, and clinical manifestations of ALS. Management of
ALS will be discussed, including implications for the
healthcare provider.
PATHOPHYSIOLOGY
ALS causes progressive degeneration of the upper and
lower motor neurons. Motor neurons are those cells in the
brain, brain stem, and spinal cord that control voluntary
movements of muscles. Upper motor neurons reside in the
motor cortex of the brain and send electrical impulses to the
lower motor neurons in the brain stem and spinal cord.
Upper motor neurons are involved in the initiation and
control of voluntary movements and the maintenance of
muscle tone. When damaged or lost, the limbs become
spastic or stiff, and over-activity of tendon reflexes, such as
knee and ankle jerks, typically occurs. Lower motor neurons
are the motor neurons connecting the brainstem and spinal
cord to muscle fibers, bringing the nerve impulses from the
upper motor neurons out to the muscles. Loss or damage of
lower motor neurons results in weakness, twitching and
atrophy of the muscles. Both sets of motor neurons are
required for optimal control of muscles. When there are
disruptions in these signals,
the muscles do not work
properly. Eventually, the
ability to control voluntary
movement can be lost.
Indications of upper motor
neuron involvement include
tight and stiff muscles
(spasticity) and
exaggerated reflexes (hyperreflexia) including an overactive
gag reflex. A positive Babinski's sign, the big toe extends
upward and other toes spread out, also indicates upper
motor neuron damage. Manifestations of lower motor neuron
degeneration include muscle weakness and atrophy, muscle
cramps, fleeting twitches of muscles that can be seen under
the skin (fasciculations) and depressed tendon reflexes. To
be diagnosed with ALS, patients must have signs and
symptoms of both upper and lower motor neuron damage
that cannot be attributed to other causes.
ETIOLOGY
Genetics accounts for 5–10% of cases of familial ALS
(FALS) in the United States. A recessive dominant trait is
responsible for FALS, meaning only one parent carries the
gene. Children of patients with this type of ALS, have a 50%
chance of developing the disease. In 90 – 95% of cases,
ALS occurs sporadically. Although no cause and effect has
been found, U.S. veterans and laborers engaged in
agricultural work, factory work, heavy manual labor, and
welding are groups of people who appear to develop ALS
more often than the general population.
Current research suggests that glutamate, the most
important neurotransmitter for healthy brain function,
accumulates to toxic levels at the synapses with ALS,
causing degeneration of neurons. Neurotransmitters are the
chemicals which allow the transmission of signals from one
neuron to the next across synapses. Excessive levels of
glutamate can over stimulate motor neurons and cause them
to die.
Copyright © 2011 Growing Up With Us, Inc. All rights reserved.
Page 1 of 4
CLINICAL MANIFESTATIONS
ALS involves a slow, chronic progression of symptoms.
The onset of symptoms varies with each patient. Regardless
of the part of the body first affected by ALS, symptoms
spread to other parts of the body as the disease progresses.
Occasionally, the symptoms remain confined to one limb for
a long period of time or for the whole
length of the illness. This is known as
monomelic amyotrophy. The majority
of people with ALS experience "limb
onset" ALS. One of the first symptoms
is usually unexplained weakness in a
limb. The first symptoms may involve
the arms, in which patients may
experience difficulty with tasks
requiring manual dexterity, such as buttoning a shirt, writing,
or turning a key in a lock. Patients with the leg-onset form
may experience awkwardness when walking or running. Or
they may notice incidents of tripping or stumbling, often with
a "dropped foot" which drags gently along the ground. Early
in the disease one limb is usually involved, and later in the
course of the illness the other limbs become involved. It’s
important to note that upper limb symptoms do not mean
upper motor neuron involvement, and that involvement of the
legs is not synonymous with lower motor neuron loss or
damage.
About 25% of ALS cases are "bulbar onset”, in which the
oral muscles degenerate. These patients first notice difficulty
speaking clearly or swallowing. Speech may become
slurred, nasal in character, or quieter than normal. Other
symptoms include hoarseness, difficulty swallowing
(dysphagia), and loss of tongue mobility. Bulbar palsy is one
of the most distressing features of motor neuron disease. It
causes weakness of the tongue, pharynx, and facial muscles
and leads to loss of salivary control. The patient has difficulty
with eating and may choke and drool.
A smaller proportion of patients experience "respiratory
onset" ALS where the intercostal muscles that support
breathing are affected first. Death can occur within months
with this type of onset. Early respiratory or bulbar symptoms
and increasing age are poor prognostic indicators.
Many patients with ALS experience involuntary emotional
expression disorder (IEED), also known as pseudobulbar
affect or emotional lability. IEED is characterized by
episodes of uncontrollable laughter, crying or smiling, that
are inappropriate and independent of the patient’s mood.
IEED is attributable to degeneration of upper motor neurons
resulting in exaggeration of motor expressions of emotion.
Mood swings, anxiety, and depression may also occur. As
the patient watches muscle strength decline, becomes
increasingly dependent on others, and faces their own
mortality, feelings of depression commonly increase and
suicidal thoughts may emerge.
Rarely is there cognitive involvement associated with
ALS. Bladder or bowel function, as well as the senses of
vision, touch, hearing, taste, and smell, are not affected. As
the disease progresses more muscle groups become
involved. However, with ALS, all muscles aren’t involved.
For example, the cardiac muscles, as well as the smooth
muscles (those in the hollow parts of the body, such as in
the stomach, intestines, blood vessels and the bladder),
aren't involved in ALS.
DIAGNOSIS
The patient usually adapts to the initial, gradual changes
in muscle strength and endurance associated with ALS
before consulting a physician / neurologist. Often these
symptoms are initially attributed to aging. However, over
time the patient or a family member realizes that frequent
tripping or falling, the inability to hold a full cup of coffee or
dress oneself, and/or slowed speech is likely something
more than aging.
There is no definitive test for diagnosing ALS. A
neurologic exam, assessment of patient symptoms, and
family history are important parts of the diagnosis. Both
upper and lower neuron impairment must be present for the
diagnosis of ALS to be made, including tight and stiff
muscles and exaggerated reflexes (upper neuron
involvement) and muscle weakness, wasting, twitching, and
cramps (lower neuron involvement). Diagnostic tests
frequently include blood studies, electromyography (EMG),
nerve conduction velocities (NCV), x-rays, muscle and/or
nerve biopsy, CT scan, and magnetic resonance imaging
(MRI). Often times, these tests rule out other disorders that
mimic symptoms of ALS, such as myasthenia gravis,
multiple sclerosis, and multifocal motor neuropathy (MMN).
Genetic testing may be conducted if there is a family history
of ALS.
PATIENT CARE PRIORITIES
Riluzole (Rilutek), which reduces the presynaptic release
of glutamate and protects neurons by reducing excitotoxicity,
is the only FDA approved drug for ALS patients. Treatment
should be initiated as early as poss ible after the patient has
been diagnosed. Liver function should be closely monitored,
since it causes an elevation in liver enzymes. Other
medications may be prescribed according to the patient’s
symptoms, such as Baclofen (Lioresal) to relieve stiffness in
the limbs and Tizanidine, a muscle relaxant, for treatment of
muscle spasms.
Meeting the nutritional
needs of patients with ALS is
priority. Patients with ALS have a
high risk of malnutrition. Drooling
and thick mucus can cause choking
and interfere with eating. Suctioning
is a key intervention. The patient should be fed small,
frequent high-calorie meals. The head of the bed should be
elevated 30 minutes after meals to help prevent possible
aspiration. As the disease progresses, nutritional support is
commonly provided by a gastrostomy tube.
There is no cure for ALS. Patients are cared for
symptomatically, and supportive management is directed at
preventing complications of immobility, including skin
breakdown and deep vein thrombosis. Most patients with
ALS are cared for at home. Patient and family teaching, as
well as support, are essential roles of the healthcare
professional.
Growing Up With Us, Inc.
PO Box 481810 • Charlotte, NC • 28269
Phone: (919) 489-1238 Fax: (919) 321-0789
Editor-in-Chief: Mary M. Dunlap MAEd, RN
E-mail: editor@growingupwithus.com
Website: www.growingupwithus.com
GUWU Testing Center
www.growingupwithus.com/quiztaker/
Copyright © 2011 Growing Up With Us, Inc. All rights reserved.
Page 2 of 4
Name:_____________________________________________________
Date:___________________________________
Employee ID#:____________________________________________
Unit:____________________________________
POPULATION/AGE-SPECIFIC EDUCATION POST TEST
GROWN UP...
Caring For Adolescents, Adults, and Aging Adults
June 2011
Competency: Demonstrates Age-Specific Competency by correctly answering 9 out
of 10 questions related to Amytrophic Lateral Sclerosis (ALS).
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
1. One of the first symptoms of ALS is often:
a.
b.
c.
d.
dysphagia.
emotional lability.
muscle cramps and wasting.
unexplained weakness in a limb.
2. ALS eventually leads to atrophy of the involuntary muscles.
a. True
b. False
3. ALS involves eventual degeneration of the brain and meninges.
a. True
b. False
4. Which statement is true about ALS? It:
a.
b.
c.
d.
involves both the upper and lower motor neurons.
is an acute condition.
affects women more often than men.
has an average age of onset at age 45.
5. Indicators of lower neuron involvement in ALS include:
a.
b.
c.
d.
muscle cramps and fasciculations.
tight and stiff muscles.
positive Babinski.
exaggerated reflexes.
Copyright © 2011 Growing Up With Us, Inc. All rights reserved.
Page 3 of 4
Name:_____________________________________________________
Date:___________________________________
Employee ID#:____________________________________________
Unit:____________________________________
POPULATION/AGE-SPECIFIC EDUCATION POST TEST
GROWN UP...
Caring For Adolescents, Adults, and Aging Adults
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
6. Current research indicates which of the following accumulates to toxic levels with ALS?
a.
b.
c.
d.
Serotonin
Histamine
Dopamine
Glutamate
7. Symptoms of ALS typically include which of the following?
a.
b.
c.
d.
Depression
Cognitive loss
Bowel and bladder dysfunction
Loss of the senses
8. The only FDA approved drug for ALS is Riluzole (Rilutek).
a. True
b. False
9. Which statement is NOT true about diagnosing ALS? ALS:
a.
b.
c.
d.
has a hereditary component in some cases.
symptoms are often initially attributed to aging by the patient.
is often diagnosed by ruling out other conditions.
affects those under 50 years old most commonly.
10. After the appearance of the first symptoms of ALS, life expectancy rarely exceeds how many
years?
a.
b.
c.
d.
1
3
5
7
Copyright © 2011 Growing Up With Us, Inc. All rights reserved.
Page 4 of 4