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El Escorial Criteria – ALS Diagnostic Certainty — SCE Neurology MCQ

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HardPeripheral Neuropathy & NeuromuscularEl Escorial Criteria – ALS Diagnostic CertaintySCE Neurology

Progressive motor impairment with upper- and lower-motor-neurone signs in multiple regions and supportive EMG meets contemporary diagnostic criteria after mimics are excluded. What diagnosis should be communicated?

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Correct answer: B — Motor neurone disease under contemporary Gold Coast diagnostic criteria

The best answer is “Motor neurone disease under contemporary Gold Coast diagnostic criteria”. Motor neurone disease diagnosis requires progressive motor impairment with upper and lower motor-neurone dysfunction and exclusion of important mimics; multidisciplinary supportive care should begin early. The alternatives “Multifocal motor neuropathy under conduction-block criteria, after specialist assessment”, “Cervical myeloradiculopathy confined to one spinal level”, “Kennedy disease with androgen-receptor expansion, within the relevant UK pathway”, “Inclusion body myositis with finger-flexor weakness, after excluding important mimics” are clinically adjacent possibilities, but they do not match the defining chronology, localisation, physiology, investigation result or UK management sequence in this stem.

Reference: NICE NG42 motor neurone disease recommendations: https://www.nice.org.uk/guidance/ng42/chapter/Recommendations