Amyotrophic Lateral Sclerosis (ALS)

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Questions and Answers

Which of the following is the MOST accurate description of Amyotrophic Lateral Sclerosis (ALS)?

  • A chronic, non-progressive disorder affecting sensory neurons.
  • An acute, treatable condition leading to temporary motor impairment.
  • A genetic disorder primarily affecting cognitive functions without motor involvement.
  • A progressive, fatal neurodegenerative disorder with no known cure. (correct)

ALS affects cognitive function in nearly all diagnosed individuals.

False (B)

Describe the primary pathological process that leads to paralysis in individuals with ALS.

Degeneration and scarring of motor neurons in the lateral aspect of the spinal cord, brainstem, and cerebral cortex.

Mutations in the gene that produces the _________ enzyme have been linked with ALS, suggesting a potential role of oxidative stress in the disease.

<p>SOD1</p> Signup and view all the answers

Match each ALS characteristic with its description:

<p>Sporadic ALS = Most common form, affecting individuals typically between 40-60 years of age. Familial ALS = Inherited form of ALS, accounting for about 5-10% of cases. Bulbar ALS = Form of ALS where early symptoms involve motor neurons in the brainstem, affecting speech and swallowing.</p> Signup and view all the answers

Which of the following statements about the etiology of ALS is MOST accurate?

<p>The cause of ALS is largely unknown, but the disease is multi-factorial with both genetic and environmental risk factors. (B)</p> Signup and view all the answers

The risk of developing ALS decreases with age.

<p>False (B)</p> Signup and view all the answers

Name three potential environmental risk factors that have been associated with an increased risk of developing ALS.

<p>Smoking, environmental toxin exposure, and military service.</p> Signup and view all the answers

In the context of ALS, damage to _______ motor neurons typically results in symptoms such as spasticity, hyperreflexia, and a positive Babinski's sign.

<p>upper</p> Signup and view all the answers

Match each early ALS symptom with the body area it affects:

<p>Tripping = Legs Slurred speech = Mouth Dropping objects = Hands</p> Signup and view all the answers

Which diagnostic test is used to measure the electrical activity of muscles and can aid in ruling out other conditions that mimic ALS?

<p>EMG (B)</p> Signup and view all the answers

Treatments for ALS can cure the disease if administered early enough.

<p>False (B)</p> Signup and view all the answers

Identify two medications commonly prescribed to manage ALS symptoms or slow disease progression.

<p>Riluzole and Edaravone</p> Signup and view all the answers

In managing ALS, massage therapy can play a role, primarily aimed at preventing respiratory ______, a common cause of mortality in late-stage ALS.

<p>complications</p> Signup and view all the answers

Match each therapy with its primary aim in ALS treatment:

<p>Physical therapy = Maintain muscle strength and function. Speech therapy = Improve communication abilities. Nutritional support = Ensure adequate nutrient intake.</p> Signup and view all the answers

Which of the following is a key goal of massage therapy in ALS management?

<p>To prevent respiratory complications and maintain function of respiratory muscles. (A)</p> Signup and view all the answers

Ankle plantarflexion strength is typically lost before dorsiflexion strength in ALS.

<p>False (B)</p> Signup and view all the answers

Why is it important to routinely stretch contractures in individuals with ALS, and what precaution should be taken during the stretching process?

<p>To support the joints as there is minimal control of muscle activity in later stages.</p> Signup and view all the answers

In ALS patients, massage therapy targeting the abdominal muscles may assist in enhancing _________, especially when the diaphragm and intercostal muscles become weak.

<p>inspiration</p> Signup and view all the answers

Match each potential outcome with how massage can benefit ALS patients:

<p>Stiffness = Decrease stiffness. Pain = Decrease pain. Muscle Function = Promote muscle function.</p> Signup and view all the answers

Which cognitive function is generally NOT affected by ALS?

<p>Coordination (C)</p> Signup and view all the answers

Amyotrophic Lateral Sclerosis impacts primarily the upper motor neurons.

<p>False (B)</p> Signup and view all the answers

One way to diagnose ALS is by testing reflex responses.

<p>True (A)</p> Signup and view all the answers

State what percentage of ALS cases are inherited.

<p>5-10%</p> Signup and view all the answers

What percentage more common is ALS in men than women

<p>20%</p> Signup and view all the answers

Slurred speech can be an early sign of ALS. The medical term used for this symptom is __________.

<p>dysarthria</p> Signup and view all the answers

Fasciculations, or muscle twitching under the skin, is caused by a deterioration of the ______ motor neurons.

<p>lower</p> Signup and view all the answers

Match each treatment with its effect on daily function:

<p>Riluzole = Increase life expectancy. Edaravone = Reduced the decline of daily function</p> Signup and view all the answers

Match the ALS symptom with type of motor neuron:

<p>Spasticity = Upper neuron Muscle Cramps = Lower neuron</p> Signup and view all the answers

Which of the following is NOT a typical symptom of ALS?

<p>Sensory loss (C)</p> Signup and view all the answers

Which statement about the role genetics play in ALS is MOST accurate?

<p>Genetics may play a role in making people more susceptible. (A)</p> Signup and view all the answers

Smoking post menopause has no impact on ALS.

<p>False (B)</p> Signup and view all the answers

Genetic variation in Familial ALS is different than genetic variation in Sporadic ALS.

<p>False (B)</p> Signup and view all the answers

How does the location of nerve damage affect ALS symptoms

<p>Motor neuron damage impacts areas like hands, face, legs, and breathing. Different areas of body will be affected depending where nerve damage is located.</p> Signup and view all the answers

On average, how long do 80% of people with ALS live after diagnosis?

<p>2-5 years</p> Signup and view all the answers

In general, the risk of ALS increases with ______.

<p>age</p> Signup and view all the answers

The frontal cortex of the brain controls ________, which is not affected with those diagnosed with ALS.

<p>cognition</p> Signup and view all the answers

Match each ALS symptom with associated reflex change:

<p>Upper neuron damage = Hyperreflexia Lower neuron damage = Hyporeflexia</p> Signup and view all the answers

Match the type of ALS with typical location of motor neuron damage:

<p>Bulbar type = Corticobulbar area of brainstem</p> Signup and view all the answers

Flashcards

Amyotrophic Lateral Sclerosis (ALS)

Progressive neurodegenerative disorder with no cure, leading to gradual paralysis due to motor neuron degeneration in the spinal cord, brainstem, and cerebral cortex.

Unaffected Brain Areas in ALS

Areas of the brain controlling coordinated movements (cerebellum) and cognitive function (frontal cortex) are typically not affected by ALS.

SOD1 and ALS

Mutations of a gene that produces SOD1 enzyme, a powerful antioxidant, has been linked to ALS.

Hereditary ALS

5-10% of ALS cases are inherited from parent to child.

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Age and Sex Risk Factors for ALS

Most common between ages 40-60; before age 65, men are slightly more at risk than women; risk increases with age.

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Environmental Risk Factors for ALS

Exposure to lead, metals, chemicals, traumatic injuries, viral infections, and intense exertion.

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Sporadic ALS

Most common type of ALS, can affect anyone, between 40-60 years of age.

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Bulbar ALS

early stages motor neutron in corticobulbar area of brainstem are affected - affects mms of head, face, neck, these become paralyzed first

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Early speech/swallowing issues

Difficulty swallowing, slurred speech, and a 'thick' tongue can be early indicators

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Upper Motor Neuron Symptoms in ALS

Tight and stiff muscles (spasticity), hyperreflexia, and a positive Babinski's sign are indicators of upper motor neuron involvement

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Lower Motor Neuron Symptoms in ALS

Muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles (fasciculations) indicate lower motor neuron damage.

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Diagnostic Tests for ALS

EMG tests electrical activity of muscles, nerve conduction studies measure nerve impulse ability, MRI rules out tumors and herniated discs.

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Symptom Management in ALS

Designed to relieve symptoms and improve patient quality of life, including drugs to control drooling, spasticity, constipation, fatigue, pain, depression, and sleep issues.

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Medications for Treating ALS

Riluzole can increase life expectancy by 3-6 months, Edaravone reduces decline of function.

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Massage treatment outcomes for ALS

Maintain function of respiratory muscles, decrease stiffness, maintain ROM, prevent edema.

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Study Notes

  • ALS stands for Amyotrophic Lateral Sclerosis, also known as Lou Gehrig's Disease
  • CNS 400 FT 500 PT indicates this is part of central nervous system studies

What is ALS?

  • ALS is a progressive, fatal neurodegenerative disorder with no cure
  • It involves gradual paralysis due to degeneration and scarring of motor neurons in the lateral aspect of the spinal cord, brainstem, and cerebral cortex
  • Upper motor neurons extend from the brain to the brainstem and spinal cord synapse with lower motor neurons
  • Lower motor neurons send nerve impulses to contract muscles
  • Motor neurons gradually break down and die, leading to muscle weakness and atrophy, resulting in paralysis
  • The areas of the brain controlling coordinated movements (cerebellum) and cognition (frontal cortex) are not affected
  • About 80% of people die within 2-5 years of diagnosis, but some can live 10+ years

Cognitive Function in ALS

  • 30 to 50% of people with ALS experience cognitive and/or behavioral difficulties that can affect thinking and personality
  • Manifestations include inattention, difficulty in speech, facial or reading comprehension, verbal communication issues, obsessive behavior, apathy, or depressive behavior
  • It's important to discern if symptoms are due to biological disease and to consult with professionals for cognitive assessment, as specialized tests exist for ALS/FTD

Etiology of ALS

  • The cause is unknown, and there is no cure
  • It is multifactorial and based on changes in a person's genes
  • ALS is 20% more common in men than women
  • Mutations of the gene that produces the SOD1 enzyme may be linked to ALS
  • SOD1 is a powerful antioxidant that protects the body from damage caused by free radicals

Risk Factors

  • 5-10% of ALS cases are hereditary, passed from parent to child, giving children a 50/50 chance of developing the disease
  • The risk of ALS increases with age, and it is most common between ages 40 and 60
  • Before age 65, men are slightly more at risk than women, but this difference disappears after age 70
  • Genetic studies show similarities in familial and sporadic ALS genetic variations, implying susceptibility

Environmental Risk Factors

  • Smoking is more significant for women, especially post-menopause
  • Environmental toxin exposure, possibly to lead, may be a factor, but research is ongoing
  • Military service is indicated as a risk, potentially due to exposure to metals, chemicals, traumatic injuries, viral infections, or intense exertion

Types of ALS

  • Sporadic ALS is the most common type, affecting anyone, particularly those between 40-60 years old
  • Familial ALS is passed from parent to child in 5-10% of cases
  • Bulbar ALS accounts for 30% of cases and affects motor neurons in the corticobulbar area of the brainstem, paralyzing muscles in the head, face, and neck first

Symptoms and Early Signs

  • Early signs of ALS Include tripping, dropping things
  • Symptoms include slurred or "thick" speech, difficulties in swallowing
  • Symptoms include weight loss, decreased muscle tone
  • Symptoms include shortness of breath, and increased or decreased reflexes
  • Symptoms include uncontrollable periods of laughing or crying

Symptoms of ALS

  • Upper motor neuron symptoms include no sensory deficit, speaking problems, and breathing problems
  • Upper motor neuron symptoms include eating problems, anxiety and depression, and dementia/cognitive changes
  • Upper motor neuron signs involve tight and stiff muscles (spasticity), hyperreflexia, and a positive Babinski's sign
  • Lower motor neuron signs include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles visible under the skin (fasciculations)

Diagnosis of ALS

  • Diagnosing ALS involves EMG to test the electrical activity of muscles at rest for diagnosis or rule out
  • Diagnosing ALS involves a nerve conduction study measures nerve ability to send impulses
  • Diagnosing ALS involves MRI to check for spinal cord tumors or herniated discs in the neck causing symptoms
  • Diagnosing ALS involves blood and urine tests to eliminate other potential conditions
  • Diagnosing ALS involves a muscle biopsy can provide a differential diagnosis of muscle diseases

Medical Treatment of ALS

  • Treatments are designed to relieve symptoms and improve the quality of life
  • Riluzole is taken orally and may increase life expectancy by 3-6 months
  • Edaravone is administered intravenously and reduces the decline of daily function
  • Other medications control drooling (anticholinergic), spasticity (baclofen, diazepam), constipation, fatigue, pain, depression, sleep issues, and uncontrolled outbursts of laughing or crying

Other Therapies for ALS

  • Breathing care is used
  • Physical therapy is used
  • Occupational therapy is used
  • Speech therapy is used
  • Nutritional support is used
  • Psychological and social support is used

Massage Treatment for ALS

  • Prevent respiratory complications like respiratory failure which are final stages of ALS.
  • Spasticity contributes to muscle weakness can be helped. Consistent slow stretching decreases tone to benifit
  • Cramps respond to daily stretching.
  • Ankle dorsiflexion is lost before loss of strength in plantarflexion
  • Hamstring strength appears to correlate with walking and strength decrease and parallel to loss of walking.
  • Contractures should be routinely stretched taking care to support the joints as there is minimal control of muscle activity in late stages
  • Abdominal muscles to help increase inspiration when diaphragmatic and intercostal muscles become weak.

Massage Treatment Outcomes

  • Maintaining function of respiratory muscles
  • Decreasing stiffness, maintaining ROM, and preventing or decreasing contractures
  • Promoting relaxation and improving mood
  • Maintaining joint health
  • Preventing or reducing edema
  • Preventing deep vein thrombosis
  • Promoting muscle function
  • Decreasing pain

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