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Question 1
A 62-year-old presents with progressive asymmetric limb weakness, fasciculations, tongue atrophy, and brisk reflexes with Babinski signs. Needle EMG shows widespread active denervation and chronic reinnervation in bulbar, cervical, thoracic, and lumbosacral regions. Per the Awaji criteria, how is this patient classified?
Correct Answer:
Clinically definite ALS
Question 2
Which disease-modifying therapy for ALS is specifically indicated only in patients with confirmed SOD1 gene mutations?
Correct Answer:
Tofersen
Question 3
When is non-invasive ventilation (NIV) typically initiated in ALS based on pulmonary function testing?
Correct Answer:
When forced vital capacity (FVC) falls below 50% predicted or symptomatic orthopnea develops
Question 4
A 6-month-old hypotonic infant has tongue fasciculations, absent reflexes, and paradoxical breathing. Genetic testing confirms homozygous SMN1 deletion with 2 copies of SMN2. Which therapy provides one-time IV gene replacement and is FDA-approved for SMA patients under 2 years of age?
Correct Answer:
Onasemnogene abeparvovec
Question 5
A patient with Kennedy disease (spinobulbar muscular atrophy) presents with proximal weakness, gynecomastia, and perioral fasciculations. What is the underlying genetic mechanism?
Correct Answer:
CAG trinucleotide repeat expansion in the androgen receptor (AR) gene on Xq12
Question 1
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About this Exam

Prepare with the ABPMR Neuromuscular Medicine Practice Questions - ABPMR Neuromuscular Medicine Subspecialty Certifying Examination Exam practice quiz. This question bank includes 100 questions covering year-old, weakness, shows, diagnosis, and presents. Use it to review important concepts, identify knowledge gaps, and build confidence for the related exam, course, or assessment.

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ABPMR Neuromuscular Medicine Practice Questions - ABPMR Neuromuscular Medicine Subspecialty Certifying Examination Exam

This practice set contains 100 questions from the matching question bank and focuses on year-old, weakness, shows, diagnosis, and presents. Work through each question carefully, review the provided solutions, and revisit topics that need more study before your next attempt.

This is an independent study resource intended for practice and review; it is not an official examination or an endorsement by any organization named in the title.

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