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Motor Neurone Disease – Prognosis — SCE Neurology MCQ

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HardPeripheral Neuropathy & NeuromuscularMotor Neurone Disease – PrognosisSCE Neurology

A 50-year-old woman presents with a 6-month history of bilateral upper limb weakness, muscle wasting, and fasciculations. Reflexes are brisk throughout with extensor plantar responses. Tongue is wasted with fasciculations. Sensation and cognition are intact. She reports inappropriate emotional lability (pseudobulbar affect). What feature of this presentation is most concerning for poor prognosis in MND?

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Correct answer: B — Bulbar onset

Bulbar-onset MND (as suggested by tongue wasting and fasciculations) carries a worse prognosis than limb-onset disease, with median survival of approximately 2–3 years compared to 3–5 years for limb-onset. Other poor prognostic factors include older age at onset, low FVC at diagnosis, and rapid progression of disability. A: UMN signs alone do not predict prognosis. B: Pseudobulbar affect reflects bilateral UMN involvement but does not independently predict prognosis. D: Age >45 is a relative factor but bulbar onset is more significant. E: Female sex is not an independent poor prognostic factor.

Reference: NICE NG42 MND (2016); EFNS MND Guidelines (2012)