Assessment of Motor Neuron Disease
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Questions and Answers

Which assessment tool provides an 11-point global measure of functioning in individuals with ALS?

  • Functional Independence Measure (FIM)
  • ALS Functional Rating Scale (ALSFRS)
  • ALS Severity Scale (ALSSS)
  • Schwab and England Activities of Daily Living Scale (correct)

    • Beyond ALS-specific assessments, which scale has been utilized in clinical trials to measure the impact of ALS?

  • Fatigue Severity Scale (correct)
  • Barthel Index
  • Glasgow Coma Scale
  • Modified Ashworth Scale

    • According to the staging system described, what characteristics define a patient in stage 2 of ALS?

  • Mild deficits in function in three regions or a moderate to severe deficit in one region and mild or normal function in two other regions. (correct)
  • Functionally independent in ambulation, ADLs, and speech
  • Nonfunctional movement of at least two regions and moderate or nonfunctional movement of a third area
  • Needs assistance due to deficits in two or three regions

    • Which of the following is characteristic of patients in stage 3 of ALS?

    <p>Requires assistance due to deficits in two or three regions (D)</p> Signup and view all the answers

    Which of the following is associated with ALS stage 4?

    <p>Nonfunctional movement of at least two regions and moderate or nonfunctional movement of a third area. (B)</p> Signup and view all the answers

    In assessing a patient for Motor Neuron Disease (MND), which aspect of their personal history is MOST relevant?

    <p>Age (50-70) (B)</p> Signup and view all the answers

    Which cranial nerves are typically spared in Motor Neuron Disease (MND)?

    <p>Olfactory, Optic, Vestibulocochlear (D)</p> Signup and view all the answers

    What speech abnormality is MOST associated with bulbar symptoms in Motor Neuron Disease (MND)?

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

    What is a key characteristic of sensory examination findings in Motor Neuron Disease (MND)?

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

    In Motor Neuron Disease (MND), what is the MOST likely effect on a patient's muscle tone if the Upper Motor Neurons (UMN) are affected?

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

    During muscle testing for a patient with Motor Neuron Disease (MND) exhibiting UMN affection and mild symptoms, which type of muscle test is MOST appropriate?

    <p>Group muscle test (D)</p> Signup and view all the answers

    In assessing bulbar function in a patient with ALS, which of the following cranial nerves is NOT typically evaluated in depth?

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

    What MRI finding is indicative of Motor Neuron Disease (MND)?

    <p>Hyperintensity of the corticospinal tract (A)</p> Signup and view all the answers

    What aspect of respiratory function is best assessed using supine FVC?

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

    Why is skin inspection crucial in ALS management despite skin integrity rarely being a primary concern in late stages?

    <p>To assess contact points with various assistive devices (A)</p> Signup and view all the answers

    What is the primary focus of environmental assessment in ALS care?

    <p>Energy conservation and safety relative to the patient's current and future functional capabilities (B)</p> Signup and view all the answers

    When screening for possible cognitive impairments, what all aspects should be assessed in individuals with ALS?

    <p>Executive function, abstract reasoning, language comprehension and memory. (B)</p> Signup and view all the answers

    In evaluating postural alignment and balance for individuals with ALS, what aspect of postural control should be determined?

    <p>Static and dynamic postural alignment and body mechanics during self-care. (C)</p> Signup and view all the answers

    Which measure or test can be used to evaluate balance in individuals with ALS?

    <p>Timed Up and Go Test (TUG) (B)</p> Signup and view all the answers

    When assessing gait in individuals with ALS, what primary factors should be examined?

    <p>Gait stability, safety, and endurance. (A)</p> Signup and view all the answers

    What should be regularly examined regarding orthotic and assistive devices used by ALS patients?

    <p>Energy expenditure, alignment, fit, practicality, safety, and ease of use (B)</p> Signup and view all the answers

    What is the typical age of onset for Motor Neuron Disease (MND)?

    <p>Middle to old age (C)</p> Signup and view all the answers

    Which of the following is a characteristic of Lower Motor Neuron (LMN) affection?

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

    What is the syndrome associated with Lower Motor Neuron affection in the spinal cord, specifically affecting the Anterior Horn Cells (AHC)?

    <p>Progressive Muscular Atrophy (C)</p> Signup and view all the answers

    Which of the following symptoms is specifically associated with True Bulbar Palsy, a type of Lower Motor Neuron affection?

    <p>Absent palatal and pharyngeal reflexes (C)</p> Signup and view all the answers

    In Amyotrophic Lateral Sclerosis (ALS), what combination of signs is typically observed in the upper limbs?

    <p>Weakness, wasting, and fasciculations along with hypertonia and hyperreflexia (B)</p> Signup and view all the answers

    What findings from nerve conduction studies would suggest Motor Neuron Disease?

    <p>Preservation of sensory responses with normal or reduced motor amplitudes (D)</p> Signup and view all the answers

    What is the syndrome associated with Upper Motor Neuron affection in the brain stem or cerebral hemisphere?

    <p>Pseudo-bulbar Palsy (B)</p> Signup and view all the answers

    What findings from a needle EMG (Intramuscular) would suggest Motor Neuron Disease?

    <p>Active denervation demonstrated by fibrillation potentials and chronic sharp waves. (B)</p> Signup and view all the answers

    Flashcards

    Fatigue Severity Scale

    A scale used to assess fatigue severity in clinical trials for ALS.

    Functional Independence Measure (FIM)

    A tool used to document functional status in clinical trials assessing independence in daily activities.

    Schwab and England Scale

    An 11-point scale measuring activities of daily living (ADL) function from 100% to 0% in ALS patients.

    ALS Functional Rating Scale (ALSFRS)

    A specific assessment tool for evaluating function in patients with ALS, measuring various abilities.

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    ALS severity scale stages

    Five stages of ALS severity, from mild (independent) to death, based on functional deficits.

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    Cranial Nerve Testing

    Assessing cranial nerves to understand bulbar involvement extent.

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    Respiratory Function Assessment

    Evaluating respiratory symptoms, muscle function, and capacity using spirometry.

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    Supine FVC

    Forced Vital Capacity measured while lying down; better for diaphragm assessment.

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    Maximal Inspiratory Pressure (MIP)

    A measure of respiratory function to detect early respiratory insufficiency.

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    Skin Integrity in ALS

    Skin problems are rare in late-stage ALS; inspect for device contact points.

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    Cognitive Assessment in ALS

    Examine executive function, memory, and reasoning; screen for depression.

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    Balance Tests

    Use measures like Berg Balance Scale to assess postural control in ALS patients.

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    Gait Assessment

    Documenting gait stability, safety, and endurance with timed trials.

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    T2 Weighted Imaging

    MRI technique highlighting hyperintensity in brain regions relevant to corticospinal tracts.

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    Corticospinal Tract

    Pathway connecting the brain to the spinal cord for motor control, typically assessed in neurological examinations.

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    Upper Motor Neuron (UMN) Signs

    Indications of nerve damage located in the brain or spinal cord, like hypertonia and spasticity.

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    Lower Motor Neuron (LMN) Signs

    Indications of nerve damage outside the brain and spinal cord, characterized by hypotonia and muscle weakness.

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    Dysarthria

    Speech disorder due to muscle weakness, resulting in slurred or unclear speech.

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    Manual Muscle Testing (MMT)

    Clinical evaluation technique assessing muscle strength and endurance through physical resistance tests.

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    Assessment of Bulbar Function

    Evaluation of the cranial nerves that control swallowing and speech, often affected in ALS.

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    Visual Analogue Scale (VAS)

    A measurement tool for patients to rate their pain intensity on a scale, often a line or a chart.

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    Motor Neuron Disease (MND)

    A degenerative disease affecting the motor system, mostly in older adults.

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    Upper Motor Neuron (UMN) affection

    Type of MND involving the upper motor neurons, leading to symptoms like spasticity.

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    Lower Motor Neuron (LMN) affection

    Type of MND affecting lower motor neurons, causing weakness and muscle atrophy.

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    Amyotrophic Lateral Sclerosis (ALS)

    A type of MND with combined symptoms of both UMN and LMN.

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    Spastic Paraplegia

    Occurs when UMN damage is below the cervical region, causing leg stiffness.

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    Bulbar Palsy

    Result of UMN affection in the brain stem, affecting speech and swallowing.

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    Fibrillation potentials

    Electrical signals indicating active denervation found in needle EMG of LMN.

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    Nerve conduction studies

    Tests showing preserved sensory responses but abnormal motor amplitudes in MND.

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    Study Notes

    Assessment of Motor Neuron Disease

    • Motor Neuron Disease (MND) is a degenerative disease with a gradual onset and progressive course, affecting only the motor system.
    • The typical age of onset is middle to old age, with males more frequently affected than females.
    • The course of the disease is gradual and progressive.
    • Symptoms are usually bilateral and depend on the affected area.

    Types of Motor Neuron Affection

    • Upper Motor Neuron (UMN) affection:

      • In the spinal cord, UMN affection can cause bilateral symptoms, potentially leading to spastic paraplegia (below the cervical region) or spastic quadriplegia (at the level of the cervical region).
      • In the brain stem or cerebral hemisphere, affected individuals experience pseudo-bulbar palsy symptoms, including bulbar symptoms, quadriplegia, exaggerated palatal and pharyngeal reflexes, emotional and mood changes, and an exaggerated jaw reflex (if the lesion is above the pons).

    • Lower Motor Neuron (LMN) affection:

      • LMN affection can occur in the spinal cord or cranial nerve nuclei of the brain stem, resulting in progressive muscular atrophy.
      • Symptoms include muscle wasting, hypotonia (decreased muscle tone), weakness, hyporeflexia (reduced reflexes), and fasciculations (muscle twitching).
      • Symptoms initially appear in the upper limbs, later progressing to the lower limbs.
      • In the Cranial nerve nuclei, true bulbar palsy can emerge, characterized by bulbar symptoms, absent palatal and pharyngeal reflexes, and wasted tongue with fasciculations.

    • Combined UMN and LMN affection:

      • Also known as Amyotrophic Lateral Sclerosis (ALS), this involves a combination of UMN and LMN symptoms.
      • In the upper limb, combined affection presents as weakness, wasting, and fasciculations (LMN), along with hypertonia and hyperreflexia (UMN). This combination is often referred to as tonic atrophy.
      • In the lower limb, weakness with UMN signs is found.

    Diagnosis of Motor Neuron Disease (MND)

    • Nerve conduction studies: These show preservation of sensory responses, with normal or reduced motor amplitudes.
    • Needle EMG: Demonstrates active denervation, shown by fibrillation potentials and chronic sharp waves in multiple myotomes.
    • MRI (Brain/Spine): Shows hyperintensity in corticospinal tracts (specifically the posterior limb of the internal capsule and centrum semiovale).

    Assessment of MND

    • History: Includes personal information (name, age, sex, habits, onset, course, duration of symptoms, medications, past medical history, trauma, family history, and chief complaints).

    • Examination:

      • General: Assess respiration, neurological status
      • Non-essential: Mental status (emotional liability, mood changes, and pseudo-bulbar palsy), evaluation of cranial nerves (all except 1, 2, 8, ocular nerves, sensory parts of V and VII); assess speech for dysarthria and dysphonia (bulbar symptoms).
      • Essential: Sensory examination is normal, Motor examination includes inspection (muscle wasting, fasciculations), assessment of muscle tone (hypertonia in UMN, hypotonia in LMN).

    • *Muscle testing: Patients may fatigue easily, therefore, appropriate tests (group or functional) may be used, dependent on the level of severity.

    • Reflexes: Reflex assessment is performed based on the type of affection.

    • Coordination: Postural stability, reactive, anticipatory, and adaptive postural controls are assessed. ALS-specific balance tests are not available. Various general balance tests (Berg Balance Scale, Timed Up and Go Test, Functional Reach Test) are employed.

    • Gait: Examination looks at the duration of gait (within time period/distance), stability, safety, endurance, energy expenditure, practicality, and ease of use of assistive devices.

    • Cognition: Assess executive function, language comprehension, memory, and abstract reasoning. Common co-morbidities like depression and anxiety are screened for.

    • Sensory Examination: General sensory examination is usually normal.

    • Bulbar evaluation: Assessment of cranial nerves V, VII, IX, X, and XII assesses the extent of bulbar involvement (important for swallowing and speech).

    • Respiratory function: Examine respiratory symptoms, breathing pattern, chest expansion, respiratory sounds, cough effectiveness, Forced Vital Capacity (FVC), and maximal inspiratory pressure (MIP) using spirometry.

    • Integument: Skin integrity is usually not an issue.

    • Environmental assessment: Focuses on energy conservation, safety, and current/future functional capabilities.

    • Additional assessment: The Fatigue Severity Scale and other scales or measures may be used across various stages of ALS, for different functional issues.

    Stages of ALS

    • Stage 1 (Mild): Recent diagnosis, functionally independent in ambulation, ADLs, and speech.
    • Stage 2 (Moderate): Mild deficits in function in one or more regions. Moderate to severe deficit in one region with mild/normal function in others.
    • Stage 3 (Severe): Need for assistance in two or more regions (e.g., walking, transferring, upper extremity activities, dysarthria/dysphagia).
    • Stage 4 (Very Severe): Nonfunctional movement in two or more regions, nonfunctional impairment in a third region.
    • Stage 5 (Terminal): Death.

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    Assessment of Motor Neuron Disease PDF

    Description

    This quiz explores the characteristics and types of Motor Neuron Disease (MND), a progressive condition primarily affecting motor systems. It discusses the differences between Upper and Lower Motor Neuron affections and their associated symptoms. Test your knowledge on this degenerative disease affecting motor function.

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