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ALS — MCCQE Part 1 MCQ

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ModerateWeaknessALSMCCQE Part 1

A man with established amyotrophic lateral sclerosis develops orthopnea, morning headaches, unrefreshing sleep and daytime somnolence. Seated oxygen saturation is 96%, but forced vital capacity has fallen and supine testing worsens. What is the most appropriate next management step?

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Correct answer: E — Offer non-invasive ventilation through the multidisciplinary ALS team

Orthopnea, morning headache, disturbed sleep and somnolence with declining vital capacity indicate respiratory-muscle weakness and nocturnal hypoventilation in ALS. Normal daytime pulse oximetry does not exclude this. Arrange prompt respiratory assessment and offer non-invasive ventilation through the multidisciplinary team, with interface fitting, secretion management, caregiver support and discussion of goals. Supplemental oxygen alone can worsen unrecognized carbon-dioxide retention and does not correct hypoventilation. Treatment should not wait for daytime respiratory failure. Bulbar dysfunction may affect tolerance but requires individualized support rather than automatic exclusion.

Reference: NICE Motor Neurone Disease Recommendations: https://www.nice.org.uk/guidance/ng42/chapter/recommendations