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Anti-Hu Paraneoplastic – Poor Immunotherapy Response — SCE Neurology MCQ

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HardNeuro-oncologyAnti-Hu Paraneoplastic – Poor Immunotherapy ResponseSCE Neurology

A 65-year-old woman with known anti-Hu positive paraneoplastic sensory neuronopathy associated with small cell lung cancer develops progressive cerebellar ataxia despite cancer treatment. What is the expected response to immunotherapy?

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Correct answer: D — Poor — anti-Hu paraneoplastic syndromes typically respond poorly to immunotherapy as the neuronal damage is largely irreversible

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

Anti-Hu (ANNA-1) associated paraneoplastic syndromes (sensory neuronopathy, encephalomyelitis, cerebellar degeneration) have a generally poor response to immunotherapy because the neuronal damage is predominantly cytotoxic T-cell mediated and largely irreversible by the time of diagnosis. Treatment focuses on managing the underlying malignancy (which may slow progression) and supportive care. In contrast, antibodies targeting cell-surface antigens (anti-NMDAR, anti-LGI1, anti-CASPR2) generally respond better to immunotherapy as the mechanism is antibody-mediated and potentially reversible. A/B: Response is typically poor. D: Immunotherapy is not contraindicated but has limited efficacy. E: Response correlates more with antibody type than histology.

Reference: EAN Paraneoplastic Syndromes Guidelines (2021)