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ALS – Cognitive Screening with ECAS — SCE Neurology MCQ

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HardPeripheral Neuropathy & NeuromuscularALS – Cognitive Screening with ECASSCE Neurology

A patient with Waldenstrom macroglobulinaemia develops months of cranial neuropathies, radicular pain and cognitive slowing despite stable systemic markers. MRI shows smooth cauda-equina and cranial-nerve enhancement. Which investigation package best establishes the suspected neurological complication?

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Correct answer: B — Neuraxis MRI and CSF cytology, flow and MYD88 testing

Explanation lettering: E = shown as B · D = shown as C · C = shown as D · B = shown as E

A cannot exclude direct CNS infiltration when systemic disease appears stable. B investigates inflammatory demyelination. C may assess systemic disease but cannot establish leptomeningeal Waldenstrom infiltration. D evaluates paraproteinaemic peripheral neuropathy, not cranial, radicular and cognitive CNS disease. E is correct: Bing-Neel syndrome requires neuraxis imaging and direct CSF assessment with cytology, flow and molecular clonality support such as MYD88 L265P; blood contamination must be minimised.

Reference: Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome: https://pmc.ncbi.nlm.nih.gov/articles/PMC5210231/