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Motor neurone disease — SCE Neurology MCQ

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ModerateNeuromuscularMotor neurone diseaseSCE Neurology

A 62-year-old man has progressive hand weakness, cramps and weight loss. Examination shows tongue fasciculations, brisk jaw jerk, wasting of the first dorsal interossei and brisk leg reflexes with extensor plantars. Sensory examination is normal. MRI cervical spine shows no cord compression. What is the most likely diagnosis?

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Correct answer: C — Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis is the best answer. Combined upper and lower motor neurone signs in multiple regions with normal sensation support amyotrophic lateral sclerosis. Cervical myelopathy would correlate with compression and sensory signs; multifocal motor neuropathy lacks UMN signs; myasthenia is fatigable without wasting; IBM has a characteristic myopathic pattern. Clinical pearl: Normal sensory findings in a progressive mixed UMN/LMN syndrome are highly suggestive.

Reference: NICE NG42; ABN MND guidance