Journal of neurology · 2026
Progressive disability but survival advantage in anti-Hu/ANNA1 paraneoplastic neurological syndromes.
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DiMauro Kimberly A, Thompson Nicolas R, Lapin Brittany, Pennell Nathan A, Stevens Glen, Shook Steven, Cohen Jeffrey A, Kunchok Amy
Аннотация
BACKGROUND: Type 1 antineuronal nuclear or "Hu" antibody (ANNA1/Hu-IgG) is associated with paraneoplastic neurological syndromes (PNS), and most often, small cell lung carcinoma (SCLC).
OBJECTIVES: To evaluate factors associated with disability and survival in ANNA1/Hu-IgG PNS and to compare the survival of patients with SCLC (with and without ANNA1/Hu-IgG PNS).
METHODS: This was a single-center cohort study of 45 patients with ANNA1/Hu-IgG PNS. Kaplan-Meier methods were used to estimate time to mortality, wheelchair dependence, and modified Rankin Scale (mRS) score > 2. Cox proportional hazards models and logistic regression were used to estimate hazard ratios (HRs) and odds ratios (ORs), respectively. Secondary analyses compared patients with ANNA1/Hu-IgG PNS to a cohort with SCLC. Survival was evaluated using Cox proportional hazards models, adjusting for age, sex, cancer stage, and treatment.
RESULTS: Disability and survival were associated with clinical phenotype at onset, with limbic encephalitis having a greater hazard of mRS > 2 (HR = 6.84, 95% CI = 2.12-22.04, P = 0.001) and greater odds of death (HR = 2.44, 95% CI = 1.05-5.66, P = 0.039). Among patients with SCLC, the patients with ANNA1/Hu-IgG PNS + SCLC (n = 26) compared to SCLC-only (n = 1513) had a 41% lower hazard of death, adjusting for age, sex, stage, and cancer treatment (HR = 0.59, 95% CI = 0.37-0.96, P = 0.033).
CONCLUSION: In ANNA1/Hu-IgG PNS, faster progression of disability and greater risk of mortality is associated with limbic encephalitis at onset, suggesting a potentially modifiable factor for early intervention. Patients with SCLC and ANNA1/Hu-IgG PNS survived longer than patients with SCLC without PNS, suggesting a survival advantage of paraneoplastic autoimmunity.
OBJECTIVES: To evaluate factors associated with disability and survival in ANNA1/Hu-IgG PNS and to compare the survival of patients with SCLC (with and without ANNA1/Hu-IgG PNS).
METHODS: This was a single-center cohort study of 45 patients with ANNA1/Hu-IgG PNS. Kaplan-Meier methods were used to estimate time to mortality, wheelchair dependence, and modified Rankin Scale (mRS) score > 2. Cox proportional hazards models and logistic regression were used to estimate hazard ratios (HRs) and odds ratios (ORs), respectively. Secondary analyses compared patients with ANNA1/Hu-IgG PNS to a cohort with SCLC. Survival was evaluated using Cox proportional hazards models, adjusting for age, sex, cancer stage, and treatment.
RESULTS: Disability and survival were associated with clinical phenotype at onset, with limbic encephalitis having a greater hazard of mRS > 2 (HR = 6.84, 95% CI = 2.12-22.04, P = 0.001) and greater odds of death (HR = 2.44, 95% CI = 1.05-5.66, P = 0.039). Among patients with SCLC, the patients with ANNA1/Hu-IgG PNS + SCLC (n = 26) compared to SCLC-only (n = 1513) had a 41% lower hazard of death, adjusting for age, sex, stage, and cancer treatment (HR = 0.59, 95% CI = 0.37-0.96, P = 0.033).
CONCLUSION: In ANNA1/Hu-IgG PNS, faster progression of disability and greater risk of mortality is associated with limbic encephalitis at onset, suggesting a potentially modifiable factor for early intervention. Patients with SCLC and ANNA1/Hu-IgG PNS survived longer than patients with SCLC without PNS, suggesting a survival advantage of paraneoplastic autoimmunity.