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Motor Neuron Disease — RACP Adult Medicine MCQ

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ModerateNeurologyMotor Neuron DiseaseRACP Adult Medicine

A 62-year-old man presents with progressive bulbar dysfunction (dysarthria, dysphagia), fasciculations, and upper and lower motor neuron signs in the limbs without sensory involvement. EMG shows widespread denervation. What is the most likely diagnosis?

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Correct answer: B — Motor neuron disease

Combined upper and lower motor neuron signs across multiple regions (bulbar, cervical, thoracic, lumbar) without sensory involvement is diagnostic of motor neuron disease (ALS) per the Gold Coast criteria. EMG showing widespread denervation supports the diagnosis. Riluzole and edaravone may modestly slow progression.

Reference: Gold Coast ALS Criteria 2020; eTG Neurology 2024