ALS (Lou Gehrig’s Disease)

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Questions and Answers

Which of the following is NOT a characteristic of ALS?

  • Progressive loss of motor function
  • Muscle weakness
  • Degeneration of motor neurons in the CNS and PNS
  • Sensory problems (correct)

Which of the following factors is NOT considered a potential etiological factor for ALS?

  • Excessive glutamate
  • Oxidative stress
  • Hereditary factors
  • Bacterial infection (correct)

Bulbar pathology in ALS is characterized by impairments primarily affecting which of the following functions?

  • Bowel and bladder control
  • Speaking and swallowing (correct)
  • Vision and hearing
  • Balance and coordination

Which of the following is NOT typically associated with UMN impairments in ALS?

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

Which of the following is a characteristic LMN impairment seen in ALS?

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

A patient with ALS reports experiencing shortness of breath when lying down. Which respiratory symptom is this most indicative of?

<p>Orthopnea (D)</p> Signup and view all the answers

In ALS, the disease typically spreads initially within:

<p>The same spinal cord segments (D)</p> Signup and view all the answers

A patient with ALS is experiencing increasing impairments, moderate activity limitations, and participation restrictions. According to the staging of ALS, which stage is this patient likely in?

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

Which of the following factors generally suggests a faster progression of ALS?

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

Which of the following is LEAST likely to be associated with a faster progression of ALS?

<p>Initial presentation with limb weakness (B)</p> Signup and view all the answers

Which of the following outcome measures is specifically designed to assess functional limitations in individuals with ALS?

<p>ALS Functional Rating Scale (C)</p> Signup and view all the answers

Which intervention approach is MOST appropriate for a patient in the early stages of ALS?

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

In the middle to late stages of ALS, the primary goal of intervention shifts towards:

<p>Compensating for impairments and modifying activities (D)</p> Signup and view all the answers

Which type of intervention is appropriate throughout all stages of ALS?

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

Which intensity level is most appropriate for exercise prescription in patients with ALS?

<p>Moderate to low intensity (C)</p> Signup and view all the answers

Resistance exercises are appropriate for patients with ALS, provided that the muscle strength is at least:

<p>3/5 MMT (A)</p> Signup and view all the answers

Which is a symptom LEAST likely to be related to overuse in ALS patients?

<p>Decreased muscle cramping (B)</p> Signup and view all the answers

A patient with ALS reports increased muscle cramping and fasciculations after a recent increase in their exercise intensity. What is the most likely explanation for these symptoms?

<p>Overuse (D)</p> Signup and view all the answers

Disuse atrophy is a consequence of which?

<p>Reduced physical activity (D)</p> Signup and view all the answers

Moderate and low intensity exercises are prescribed for ALS to prevent:

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

What is the primary focus of interventions in the late stages of ALS?

<p>Managing symptoms and maximizing quality of life (C)</p> Signup and view all the answers

Which of the following interventions would be MOST appropriate for a patient with bulbar pathology in ALS?

<p>Strategies for managing drooling and swallowing difficulties (B)</p> Signup and view all the answers

A patient with ALS is experiencing difficulty with fine motor tasks such as buttoning a shirt. This impairment is MOST likely related to:

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

Which of the following symptoms would LEAST likely indicate respiratory involvement in a patient with ALS?

<p>Hyperventilation (D)</p> Signup and view all the answers

What is the purpose of performing AROM and stretching exercises for a patient with ALS?

<p>To maintain joint mobility and prevent contractures (C)</p> Signup and view all the answers

Which of the following is the LEAST important component of an exercise prescription for patients with ALS?

<p>High intensity exercises (D)</p> Signup and view all the answers

After a diagnosis of ALS, the average survival rate is approximately:

<p>5 years (C)</p> Signup and view all the answers

Which of the following is typically preserved in individuals with ALS?

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

Upon examination, a patient with ALS demonstrates decreased reflexes, weakness, and atrophy in the intrinsic hand muscles. These findings are MOST consistent with:

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

Which of the following BEST describes the primary goal of preventative interventions in patients with ALS?

<p>To prevent secondary complications and maintain function (B)</p> Signup and view all the answers

A patient in the late stage of ALS is primarily bedridden and requires assistance with most activities. Which of the following outcome measures would be MOST appropriate for assessing their functional status?

<p>ALS Functional Rating Scale (C)</p> Signup and view all the answers

Which objective finding is least associated with UMN pathology in ALS?

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

Which treatment strategy is LEAST appropriate for managing disuse atrophy in a patient with late-stage ALS?

<p>Aquatic exercises with maximal resistance (C)</p> Signup and view all the answers

A patient with ALS reports difficulty breathing while lying flat in bed. The PTA should suspect:

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

Which of the following activities would be MOST appropriate for a patient with ALS in the EARLY stages of the disease?

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

Flashcards

What is ALS?

A progressive neurodegenerative disease involving the degeneration and loss of motor neurons in the CNS and PNS, leading to muscle weakness without sensory problems. Survival rate is approximately 5 years.

Etiology of ALS?

The cause is often unknown, but can include genetic factors, oxidative stress, and excessive glutamate.

Who does ALS affect?

ALS typically affects males over the age of 50.

Bulbar Pathology

A type of ALS affecting cranial nerves, leading to difficulties in speaking and chewing (dysarthria, dysphagia), drooling (sialorrhea), and emotional lability (pseudobulbar affect).

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UMN Impairments in ALS

Muscle weakness, spasticity, hyperreflexia, clonus, abnormal timing, fatigue, loss of dexterity, and dyssynergic movement.

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LMN Impairments in ALS

Hyporeflexia, decreased/absent reflexes, atrophy, fasciculations, cramping, and weakness.

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Respiratory Pathology in ALS

Ventilatory muscle weakness, dyspnea (shortness of breath), orthopnea (SOB when lying down), hypoventilation, and ineffective cough.

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Progression of ALS?

ALS spreads first within the same spinal cord segments.

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Stages of ALS?

Early: Few impairments. Middle: Increasing impairments. Late: Severe impairments.

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Factors Influencing ALS Progression

Older onset (over 40), bulbar onset, dyspnea at diagnosis, psychological stress, and severe involvement at diagnosis.

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Outcome Measures for ALS

Gait speed, FIM (Functional Independence Measure), BERG/POMA/TUG (balance assessments), Fatigue Severity Scale, and ALS Functional Rating Scale.

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Types of Interventions for ALS

Restorative (early stages), compensatory (middle and late stages), and preventative (all stages).

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Exercise Prescription for ALS

Moderate and low intensity exercise, AROM and stretching, resistance only >3/5 MMT, and submaximal aerobic exercise.

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Symptoms of Overuse in ALS

Exhaustion, pain, more muscle cramping, and more muscle fasciculations (vibration).

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Disuse atrophy

Reduced physical activity leads to contractions in.

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Study Notes

  • Lou Gehrig’s disease, known as ALS, involves the degeneration and loss of motor neurons in both the central and peripheral nervous systems.
  • ALS is characterized by muscle weakness without sensory impairment.
  • The survival rate for individuals with ALS is approximately 5 years.

Etiology

  • The causes of ALS are idiopathic and hereditary.
  • Other potential factors include oxidative stress and excessive glutamate levels.

Affected Population

  • ALS typically affects males over the age of 50.

Bulbar Pathology

  • 20-30% of ALS cases manifest as bulbar pathology, impacting speech and chewing.
  • Bulbar pathology affects cranial nerves, leading to dysarthria, dysphagia, sialorrhea (drooling), and pseudobulbar affect.

UMN Impairments

  • Muscle weakness is a key symptom of UMN impairment.
  • Spasticity and hyperreflexia are observed in UMN impairments.
  • Clonus and abnormal timing are characteristic of UMN impairments.
  • Fatigue, loss of dexterity, and dyssynergic movements are also associated with UMN impairments.

LMN Impairments

  • LMN impairments include hyporeflexia, or decreased/absent reflexes.
  • Atrophy and fasciculations are indicative of LMN impairments.
  • Cramping and weakness are also symptoms of LMN impairments.

Respiratory System Pathology

  • Ventilatory muscle weakness affects the respiratory system.
  • Dyspnea (shortness of breath) is a common respiratory symptom.
  • Orthopnea (shortness of breath when lying down) occurs.
  • Hypoventilation and ineffective cough are respiratory complications.

ALS Progression

  • ALS initially spreads within the same spinal cord segments.

Stages of ALS

  • Early Stage: Characterized by few impairments with minimal to no activity limitations or participation restrictions.
  • Middle Stage: Involves an increasing number of impairments and minimal to moderate activity limitations and participation restrictions.
  • Late Stage: Marked by severe impairments, activity limitations, and participation restrictions.

Factors Influencing ALS Progression

  • Older age of onset (over 40) is associated with faster progression.
  • Bulbar onset leads to quicker progression compared to limb onset.
  • Dyspnea diagnosis accelerates the disease.
  • Psychological stress can speed up the progression.
  • Severe involvement at diagnosis indicates faster progression.

Outcome Measures

  • Gait speed is a key measure to assess ALS.
  • The Functional Independence Measure (FIM) is used.
  • BERG Balance Scale, POMA, and Timed Up and Go (TUG) tests are utilized.
  • The Fatigue Severity Scale measures fatigue levels.
  • The ALS Functional Rating Scale is a specific tool for ALS assessment.

Types of Interventions

  • Restorative interventions, like strengthening unaffected muscles, are used in early stages.
  • Compensatory interventions, such as modifying activities and tasks, are applied in middle and late stages.
  • Preventative interventions, including ventilation techniques to avoid secondary complications, are implemented in all stages.

Exercise Prescription

  • Moderate and low-intensity exercises are recommended.
  • Active Range of Motion (AROM) exercises and stretching are beneficial.
  • Resistance exercises are appropriate only if MMT (Manual Muscle Testing) is >3/5.
  • Aerobic exercise should be performed at submaximal levels.

Symptoms of Overuse

  • Symptoms of overuse include exhaustion, pain, increased muscle cramping, and increased muscle fasciculations (vibrations).

Disuse Atrophy

  • Reduced physical activity leads to contractions in disuse atrophy.

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