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Grown Up.
A Newsletter For Those Who Care For
ADOLESCENTS, ADULTS, and AGING ADULTS
Volume 16, Issue 6
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
June 2011
Editor-in-Chief: Mary Myers Dunlap, MAEd, RN Upper motor neurons are involved in the initiation and control of voluntary movements and the maintenance of B E H A V I O R A L O B J E C T I V E S
muscle tone. When damaged or lost, the limbs become A F T E R R E A D I N G T H I S N E W S L E T T E R T H E L E A R N E R spastic or stiff, and over-activity of tendon reflexes, such as knee and ankle jerks, typically occurs. Lower motor neurons are tconnecting t 1. Describe the pathophysiology, etiology, and clinical
t bringing tfrom the out to t Loss or damage of lower motor neurons results in twitching and atrophy of the muscles. Both sets of motor neurons are 2. Discuss management of ALS, including implications for
required for optimal control of muscles. When there are disruptions in these signals, the muscles do not work 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. exaggerated reflexes including an overactive gag reflex. A positiv the big toe extends paralyzed. ALS is a terminal illness with upward and other toes spread out, also indicates upper motor neuron damage. Manifestations of lower motor neuron degeneration include muscle weakness and atrophy, muscle half of ALS patients die within 3 years of 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 Jean-Martin Charcot. Lou Gehrig first brought national and that cannot be attributed to other causes. international attention to the disease when he abruptly retired from the New York Yankees in 1939. Today, ETIOLOGY
approximately 5,000 new cases of ALS are diagnosed each Genetics accounts for 5–10% of cases of familial ALS year in the United States. ALS usually occurs between 40 (FALS) in the United States. A recessive dominant trait is and 70 years of age, with the incidence increasing with each responsible for FALS, meaning only one parent carries the decade. The average age of onset is age 55. The male to gene. Children of patients with this type of ALS, have a 50% female ratio is 1.3 to 1.5 but approaches 1 to 1 at ages older chance of developing the disease. In 90 – 95% of cases, than 70 years. The disease has no racial, socioeconomic, or ALS occurs sporadically. Although no cause and effect has been found, U.S. veterans and laborers engaged in This newsletter will describe the pathophysiology, agricultural work, factory work, heavy manual labor, and etiology, and clinical manifestations of ALS. Management of welding are groups of people who appear to develop ALS ALS will be discussed, including implications for the Current research suggests that glutamate, the most important neurotransmitter for healthy brain function, PATHOPHYSIOLOGY
accumulates to toxic levels at the synapses with ALS, ALS causes progressive degeneration of the upper and causing degeneration of neurons. Neurotransmitters are the lower motor neurons. Motor neurons are those cells in the chemicals which allow the transmission of signals from one brain, brain stem, and spinal cord that control voluntary neuron to the next across synapses. Excessive levels of movements of muscles. Upper motor neurons reside in the glutamate can over stimulate motor neurons and cause them motor cortex of the brain and send electrical impulses to the lower motor neurons in the brain stem and spinal cord. Copyright 2011 Growing Up With Us, Inc. All rights reserved. Page 1 of 4
CLINICAL MANIFESTATIONS
DIAGNOSIS
ALS involves a slow, chronic progression of symptoms. The patient usually adapts to the initial, gradual changes The onset of symptoms varies with each patient. Regardless in muscle strength and endurance associated with ALS of the part of the body first affected by ALS, symptoms before consulting a physician / neurologist. Often these spread to other parts of the body as the disease progresses. symptoms are initially attributed to aging. However, over Occasionally, the symptoms remain confined to one limb for 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 There is no definitive test for diagnosing ALS. A onset" ALS. One of the first symptoms 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 requiring manual dexterity, such as buttoning a shirt, writing, involvement) and muscle weakness, wasting, twitching, and or turning a key in a lock. Patients with the leg-onset form cramps (lower neuron involvement). Diagnostic tests may experience awkwardness when walking or running. Or frequently include blood studies, electromyography (EMG), they may notice incidents of tripping or stumbling, often with nerve conduction velocities (NCV), x-rays, muscle and/or a "dropped foot" which drags gently along the ground. Early nerve biopsy, CT scan, and magnetic resonance imaging in the disease one limb is usually involved, and later in the (MRI). Often times, these tests rule out other disorders that course of the illness the other limbs become involved. It’s mimic symptoms of ALS, such as myasthenia gravis, important to note that upper limb symptoms do not mean multiple sclerosis, and multifocal motor neuropathy (MMN). upper motor neuron involvement, and that involvement of the Genetic testing may be conducted if there is a family history legs is not synonymous with lower motor neuron loss or About 25% of ALS cases are "bulbar onset”, in which the PATIENT CARE PRIORITIES
oral muscles degenerate. These patients first notice difficulty Riluzole (Rilutek), which reduces the presynaptic release speaking clearly or swallowing. Speech may become of glutamate and protects neurons by reducing excitotoxicity, slurred, nasal in character, or quieter than normal. Other is the only FDA approved drug for ALS patients. Treatment symptoms include hoarseness, difficulty swallowing should be initiated as early as poss ible after the patient has (dysphagia), and loss of tongue mobility. Bulbar palsy is one been diagnosed. Liver function should be closely monitored, of the most distressing features of motor neuron disease. It since it causes an elevation in liver enzymes. Other causes weakness of the tongue, pharynx, and facial muscles medications may be prescribed according to the patient’s and leads to loss of salivary control. The patient has difficulty symptoms, such as Baclofen (Lioresal) to relieve stiffness in the limbs and Tizanidine, a muscle relaxant, for treatment of A smaller proportion of patients experience "respiratory onset" ALS where tmuscles 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 or emotional lability. IEED is characterized by is a key intervention. The patient should be fed small, episodes of uncontrollable laughter, crying or smiling, that frequent high-calorie meals. The head of the bed should be are inappropriate and independent of the patient’s mood. elevated 30 minutes after meals to help prevent possible IEED is attributable to degeneration of upper motor neurons aspiration. As the disease progresses, nutritional support is resulting in exaggeration of motor expressions of emotion. commonly provided by a gastrostomy tube. Mood swings, anxiety, and depression may also occur. As the patient watches muscle strength decline, becomes There is no cure for ALS. Patients are cared for increasingly dependent on others, and faces their own symptomatically, and supportive management is directed at mortality, feelings of depression commonly increase and preventing complications of immobility, including skin breakdown and deep vein thrombosis. Most patients with Rarely is there cognitive involvement associated with ALS are cared for at home. Patient and family teaching, as ALS. Bladder or bowel function, as well as the senses of well as support, are essential roles of the healthcare vision, touch, hearing, taste, and smell, are not affected. As the disease progresses more muscle groups become Growing Up With Us, Inc.
involved. However, with ALS, all muscles aren’t involved. PO Box 481810 • Charlotte, NC • 28269 For example, the cardiac muscles, as well as the smooth Phone: (919) 489-1238 Fax: (919) 321-0789
muscles (those in the hollow parts of the body, such as in Editor-in-Chief: Mary M. Dunlap MAEd, RN
E-mail: editor@growingupwithus.com
the stomach, intestines, blood vessels and the bladder), Website: www.growingupwithus.com
GUWU Testing Center
Copyright 2011 Growing Up With Us, Inc. All rights reserved. Page 2 of 4
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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. dysphagia. b. emotional lability. c. muscle cramps and wasting. d. unexplained weakness in a limb. 2. ALS eventually leads to atrophy of the involuntary muscles. 3. ALS involves eventual degeneration of the brain and meninges. 4. Which statement is true about ALS? It: a. involves both the upper and lower motor neurons. b. is an acute condition. c. affects women more often than men. d. has an average age of onset at age 45. 5. Indicators of lower neuron involvement in ALS include: a. muscle cramps and fasciculations. b. tight and stiff muscles. c. positive Babinski. d. exaggerated reflexes. Copyright 2011 Growing Up With Us, Inc. All rights reserved. Page 3 of 4
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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. Serotonin b. Histamine c. Dopamine d. Glutamate 7. Symptoms of ALS typically include which of the following? a. Depression b. Cognitive loss c. Bowel and bladder dysfunction d. 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. has a hereditary component in some cases. b. symptoms are often initially attributed to aging by the patient. c. is often diagnosed by ruling out other conditions. d. affects those under 50 years old most commonly. 10. After the appearance of the first symptoms of ALS, life expectancy rarely exceeds how many Copyright 2011 Growing Up With Us, Inc. All rights reserved. Page 4 of 4

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