NCS Neuromuscular Disorders 3

Question 1

A patient with myasthenia gravis develops acute respiratory failure requiring intubation (myasthenic crisis). After airway stabilization, which intervention provides the most rapid immunomodulation?

Accepted Answer

Plasmapheresis or intravenous immunoglobulin (IVIG)

Answer

Plasmapheresis (removes AChR antibodies) and IVIG both act within days to weeks — the fastest immunomodulatory options for myasthenic crisis; azathioprine takes months, and pyridostigmine alone cannot manage crisis.

Question 2

Physical therapy for a child with Duchenne muscular dystrophy (DMD) should prioritize:

Accepted Answer

Stretching to prevent contractures, functional mobility training, and respiratory support education

Answer

DMD management focuses on preventing contractures through stretching, maintaining ambulatory function, adaptive equipment, and preparing for respiratory support — aggressive or eccentric strengthening can accelerate muscle fiber damage.

Question 3

Non-invasive positive pressure ventilation (NIPPV) is typically initiated in ALS when FVC falls to:

Accepted Answer

Below 50% predicted or when symptoms of nocturnal hypoventilation are present

Answer

ALS practice guidelines recommend initiating NIPPV when FVC drops below 50% predicted or when patients report symptoms of nocturnal hypoventilation; early initiation prolongs survival and improves quality of life.

Question 4

A patient with biopsy-confirmed inclusion body myositis (IBM) presents for rehabilitation. Which management approach is most appropriate?

Accepted Answer

Low-intensity aerobic exercise and occupational therapy for adaptive equipment

Answer

IBM is uniquely resistant to all immunosuppressive therapies; management focuses on low-intensity aerobic exercise to maintain function and adaptive equipment/occupational therapy for finger flexor and quadriceps weakness.

Question 5

Nusinersen (Spinraza) treats spinal muscular atrophy through which mechanism?

Accepted Answer

Modifies SMN2 pre-mRNA splicing to include exon 7, producing full-length functional SMN protein

Answer

Nusinersen is an antisense oligonucleotide that redirects splicing of SMN2 to include exon 7, generating more full-length SMN protein and compensating for the absent SMN1 gene.

(NCS) Neurologic Clinical Specialist Practice Test

(NCS) Neurologic Clinical Specialist Practice Test

NCS Neuromuscular Disorders 3