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Stroke Rehabilitation – SMART Goal Setting — SCE Neurology MCQ

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HardNeurorehabilitationStroke Rehabilitation – SMART Goal SettingSCE Neurology

A 29-year-old with genetically confirmed DOK7 congenital myasthenic syndrome has limb-girdle fatigability, preserved eye movements and no thymic abnormality. Pyridostigmine has worsened her weakness. Which disease-directed treatment is most appropriate?

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Reveal the answer and explanation

Correct answer: C — Introduce oral salbutamol under neuromuscular supervision

Explanation lettering: B = shown as A · D = shown as B · A = shown as C · E = shown as D · C = shown as E

A is correct: DOK7 congenital myasthenic syndrome often worsens or fails to respond to acetylcholinesterase inhibition, whereas beta-2 agonists such as salbutamol can produce marked sustained benefit. B compounds the known problem. C targets complement-mediated autoimmune generalised myasthenia, not DOK7 disease. D is not the defining effective strategy and can have cardiac and seizure toxicity. E has no role in a genetic neuromuscular-junction disorder.

Reference: DOK7 congenital myasthenic syndrome: https://www.ncbi.nlm.nih.gov/books/NBK1168/