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Amyotrophic Lateral Sclerosis — SCE Neurology MCQ

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EasyPeripheral Neuropathy & NeuromuscularAmyotrophic Lateral SclerosisSCE Neurology

A 65-year-old man presents with progressive weakness and wasting of both hands, fasciculations in the arms and legs, brisk reflexes throughout including an extensor plantar response bilaterally, and dysarthria. Sensation is intact. EMG shows widespread denervation and fasciculation potentials in bulbar, cervical, thoracic and lumbar segments. What is the most likely diagnosis?

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Correct answer: B — Motor neurone disease – amyotrophic lateral sclerosis

The combination of upper motor neurone signs (brisk reflexes, extensor plantar responses) and lower motor neurone signs (wasting, fasciculations) in multiple segments (bulbar, cervical, thoracic, lumbar) with preserved sensation is diagnostic of ALS. EMG showing widespread active denervation confirms the LMN component. A: Cervical myelopathy would not explain bulbar/thoracic/lumbar involvement. C: MMN is purely LMN and motor without UMN signs. D: MG causes fatigable weakness without wasting or fasciculations. E: Kennedy disease causes LMN signs only with gynecomastia and sensory neuropathy.

Reference: NICE NG42 MND (2016); El Escorial Criteria (Revised)