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1. For a diagnosis of amyotrophic lateral sclerosis (ALS) based on the El Escorial criteria, patients should have both upper motor neuron (UMN) and lower motor neuron (LMN) signs. Which of the following is correct regarding UMN and LMN signs of ALS?

A. UMN signs include muscle atrophy B. LMN signs include muscle spasticity C. LMN signs include fasciculations D. UMN signs include decreased reflexes E. Unsure

2. Amyotrophic lateral sclerosis (ALS) is a diagnosis that depends on different sources of information and exclusion of other diagnoses. Which of the following is NOT involved in diagnosing ALS using the El Escorial criteria?

A. Electromyography B. Signs or symptoms in more than 1 body region C. Disease progression over time D. Presence of dementia E. Unsure

3. The ALSFRS-R scoring system is commonly used to follow the change in daily physical function over time in patients with amyotrophic lateral sclerosis (ALS). Which of the following statements is true about the ALSFRS-R?

A. This scoring system is easily administered and can be answered by the patient and their caregiver B. This scoring system is very sensitive to small changes in daily function over time C. This scoring system is rarely used as an outcome in clinical trials D. The total score is expected to increase over time as the disease progresses E. Unsure

4. Following stringent clinical trial inclusion criteria, which of the following would exclude him from participating in the phase III edaravone trials?

A. FVC < 85% predicted B. ALS diagnosed > 2 years ago C. ALSFRS-R score > 24 (score of ≥ 2 on each of 12 items) D. Probable ALS per revised El Escorial criteria E. Unsure

5. TF is approved for edaravone and you will provide the initial patient education. He will be receiving the maintenance infusions after the first cycle via home infusion. Which of the following is NOT correct regarding edaravone therapy in ALS?

A. Edaravone has been shown to prolong survival by approximately 3 months in patients with ALS B. Edaravone is administered by intravenous (IV) infusion for 10 days of each 28-day cycle C. Patients often receive an implanted port for IV access to administer edaravone D. The majority of patients receiving edaravone are also taking riluzole E. Unsure

6. TF mentions that the team at his ALS clinic recommended he participate in the National ALS Registry. He wonders if this is helpful and would like your opinion. Which of the following statements is NOT true regarding the National ALS Registry?

A. The registry was created by an act of Congress and signed into law in 2008 to study this rare disease B. The information gathered through the registry is vital to better understand ALS and improve care for patients with ALS C. A patient with ALS must be part of a clinical trial to enroll in the registry D. Patients with ALS, once enrolled, will complete a questionnaire about their personal history to help identify risk factors for developing ALS E. Unsure

7. LC is a 74-year-old female recently diagnosed with ALS. She will be taking riluzole but decided against edaravone because of the time required for infusions and transportation. She has a history of elevated liver enzymes (< 2 times upper limit of normal) of unknown cause. Which of the following is the most appropriate recommendation for monitoring riluzole therapy?

A. Obtain baseline liver function tests and repeat frequently upon initiating therapy (e.g., monthly for 3 months) if abnormal at baseline B. Obtain baseline liver function tests and ask the patient to monitor for jaundice and easy bruising C. Monitor liver function tests once yearly after riluzole therapy is initiated D. Riluzole is contraindicated in this patient E. Unsure

8. A patient calls the specialty pharmacy requesting to talk to a pharmacist about edaravone. The patient reports a rash, which coincides with his edaravone infusions. He denies shortness of breath, throat swelling, or expansion of the rash with subsequent doses. He strongly favors continuing edaravone rather than discontinuing it due to the potential benefits on disease progression. Which of the following is the most appropriate plan for his edaravone therapy?

A. Recommend he discontinue edaravone B. Recommend to his provider adjusting from edaravone 60 mg per dose to 30 mg per dose C. Recommend he continue edaravone without changes D. Recommend to his provider prescribing an antihistamine and corticosteroid for prior to edaravone doses E. Unsure

9. Which of the following vaccines is least important for patients diagnosed with amyotrophic lateral sclerosis (ALS)?

A. Pneumococcal conjugate vaccine 13-valent B. Recombinant zoster vaccine C. Pneumococcal polysaccharide vaccine 23-valent D. Seasonal influenza vaccine E. Unsure

10. A patient with bulbar-onset amyotrophic lateral sclerosis (ALS) will soon get a gastronomy tube placed. You review her medication list for appropriate administration via the gastronomy tube. Which of the following medications should NOT be administered via gastronomy tube?

A. Aspirin chewable tablet B. Oxybutynin immediate-release tablet C. Riluzole tablet D. Duloxetine delayed-release capsule E. Unsure

Maintenance is scheduled for 8/7/2023 7:00 AM EST - 8:00 AM EST.

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1. For a diagnosis of amyotrophic lateral sclerosis (ALS) based on the El Escorial criteria, patients should have both upper motor neuron (UMN) and lower motor neuron (LMN) signs. Which of the following is correct regarding UMN and LMN signs of ALS?

2. Amyotrophic lateral sclerosis (ALS) is a diagnosis that depends on different sources of information and exclusion of other diagnoses. Which of the following is NOT involved in diagnosing ALS using the El Escorial criteria?

3. The ALSFRS-R scoring system is commonly used to follow the change in daily physical function over time in patients with amyotrophic lateral sclerosis (ALS). Which of the following statements is true about the ALSFRS-R?

4. Following stringent clinical trial inclusion criteria, which of the following would exclude him from participating in the phase III edaravone trials?

5. TF is approved for edaravone and you will provide the initial patient education. He will be receiving the maintenance infusions after the first cycle via home infusion. Which of the following is NOT correct regarding edaravone therapy in ALS?

6. TF mentions that the team at his ALS clinic recommended he participate in the National ALS Registry. He wonders if this is helpful and would like your opinion. Which of the following statements is NOT true regarding the National ALS Registry?

9. Which of the following vaccines is least important for patients diagnosed with amyotrophic lateral sclerosis (ALS)?

10. A patient with bulbar-onset amyotrophic lateral sclerosis (ALS) will soon get a gastronomy tube placed. You review her medication list for appropriate administration via the gastronomy tube. Which of the following medications should NOT be administered via gastronomy tube?

Evaluation Questions

11. How confident are in your monitoring choice for your patient LC?

12. How confident are in your treatment choice for your patient on edaravone?

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Maintenance is scheduled for 8/7/2023 7:00 AM EST - 8:00 AM EST.

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1. For a diagnosis of amyotrophic lateral sclerosis (ALS) based on the El Escorial criteria, patients should have both upper motor neuron (UMN) and lower motor neuron (LMN) signs. Which of the following is correct regarding UMN and LMN signs of ALS?

2. Amyotrophic lateral sclerosis (ALS) is a diagnosis that depends on different sources of information and exclusion of other diagnoses. Which of the following is NOT involved in diagnosing ALS using the El Escorial criteria?

3. The ALSFRS-R scoring system is commonly used to follow the change in daily physical function over time in patients with amyotrophic lateral sclerosis (ALS). Which of the following statements is true about the ALSFRS-R?

4. Following stringent clinical trial inclusion criteria, which of the following would exclude him from participating in the phase III edaravone trials?

5. TF is approved for edaravone and you will provide the initial patient education. He will be receiving the maintenance infusions after the first cycle via home infusion. Which of the following is NOT correct regarding edaravone therapy in ALS?

6. TF mentions that the team at his ALS clinic recommended he participate in the National ALS Registry. He wonders if this is helpful and would like your opinion. Which of the following statements is NOT true regarding the National ALS Registry?

9. Which of the following vaccines is least important for patients diagnosed with amyotrophic lateral sclerosis (ALS)?

10. A patient with bulbar-onset amyotrophic lateral sclerosis (ALS) will soon get a gastronomy tube placed. You review her medication list for appropriate administration via the gastronomy tube. Which of the following medications should NOT be administered via gastronomy tube?

Evaluation Questions

11. How confident are in your monitoring choice for your patient LC?

12. How confident are in your treatment choice for your patient on edaravone?

« Return to Activity

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