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Median Nerve-Neurophysiological Index Correlates With the Survival of Patients With Amyotrophic Lateral Sclerosis.

ABSTRACT: Objective: This study aims to explore the association between median nerve-neurophysiological index (NI) and survival of patients with amyotrophic lateral sclerosis (ALS). Methods: A retrospective case series with a prospective follow-up study was performed in 238 patients with ALS. Their clinical profiles and NI were recorded. Kaplan-Meier curves and Cox regression were adopted to perform survival analysis. Results: The median survival time of all ALS cases was 33.0 months. Multivariate analysis showed that older age of onset, shorter diagnostic delay, higher ?ALSFRS-R, and faster progression {NI ? 2.15; hazard ratio [HR] = 1.543 [95% confidence interval (CI), 1.136-2.094]} were associated with short survival. NI was correlated with ALSFRS-R at baseline (r s = 0.3153; p < 0.0001) and ALSFRS-R at different time points of follow-up (r s = 0.5127; p < 0.0001). The higher NI slope of decline (> 0.25) showed shorter survival compared with the lower group (? 0.25; 34.0 vs. 52.0 months; p = 0.0003). A predictive model was constructed based on the age of onset, diagnostic delay, median nerve NI, and ?ALSFRS-R. The higher predictive score (> 14) showed significantly shorter survival compared with the lower group (? 14; HR = 3.907, 95% CI, 2.857-5.342). Conclusion: Median nerve NI and its slope of decline were predictive of survival of ALS.

SUBMITTER: Xu LQ

PROVIDER: S-EPMC7642643 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Median Nerve-Neurophysiological Index Correlates With the Survival of Patients With Amyotrophic Lateral Sclerosis.

Xu Liu-Qing LQ Hu Wei W Guo Qi-Fu QF Lai Lu-Lu LL Xu Guo-Rong GR Chen Wan-Jin WJ Wang Ning N Zhang Qi-Jie QJ

Frontiers in neurology 20201022

Objective: This study aims to explore the association between median nerve-neurophysiological index (NI) and survival of patients with amyotrophic lateral sclerosis (ALS). Methods: A retrospective case series with a prospective follow-up study was performed in 238 patients with ALS. Their clinical profiles and NI were recorded. Kaplan-Meier curves and Cox regression were adopted to perform survival analysis. Results: The median survival time of all ALS cases was 33.0 months. ...[more]

PMID: 33193014

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Project description:ImportanceDegeneration of neuromuscular junctions and axons is considered an important aspect of the pathomechanism of amyotrophic lateral sclerosis (ALS). However, a mechanism including the role of transactive response DNA-binding protein 43 (TDP-43) in axons has not been pathologically clarified.ObjectiveTo identify and characterize the histopathology of peripheral axons in the skeletal muscle of patients with ALS.Design, setting, and participantsThis study comprised 2 parts: a postmortem case-control study and a retrospective population-based cohort study with a minimum of 1 year of follow-up. Patients in the cohort study were enrolled from January 1, 2004, to September 30, 2019. The postmortem study included patients with sporadic ALS (SALS) with TDP-43 pathology and control patients with non-ALS disease. The cohort study enrolled patients without a family history of ALS or other neuromuscular disease and those not diagnosed with a muscle disease at biopsy. Patients were excluded if their clinical records were not screened after biopsy, if they were diagnosed with a muscular disease, and if they were harboring known causative genes of ALS. Data were collected between September 2019 and June 2021 and analyzed in June 2021.ExposuresMuscle biopsy or postmortem muscle tissue examination.Main outcomes and measuresClinical information and muscle pathological characteristics.ResultsA total of 10 patients with autopsy-confirmed SALS (mean [SD] age at death, 76.1 [8.5] years; 8 men [80%]) exhibited axonal phosphorylated TDP-43 (pTDP-43)-positive accumulations in intramuscular nerve bundles; the 12 control patients without ALS did not. Among the 114 patients in the cohort study (mean [SD] age, 62.3 [16.1] years; 76 men [67%]), 71 patients (62.3%) exhibited intramuscular nerve bundles; 43 (37.7%) did not. Among those who exhibited pTDP-43-positive intramuscular nerve bundles, 33 patients (22 men [66.7%]; mean [SD] age, 65.2 [15.6] years) were later diagnosed with ALS. The other 38 patients (26 men [68.4%]; mean [SD] age, 59.3 [18.0] years) showed no pTDP-43-positive bundles and did not develop ALS. Among those without evident nerve bundles (28 men [65.1%]; mean [SD] age, 61.3 [15.3] years), 3 were later diagnosed with ALS. Among patients with ALS in the biopsy cohort, 9 with pTDP-43-positive bundles showed only lower motor neuron symptoms at biopsy.Conclusions and relevanceResults of this dual case-control and retrospective cohort study suggest that axonal pTDP-43 accumulations may be characteristic for patients with ALS. As such findings precede clinical fulfillment of the Gold Coast criteria, TDP-43 in nerve bundles may be a novel diagnostic biomarker for ALS.

Dataset Information

Median Nerve-Neurophysiological Index Correlates With the Survival of Patients With Amyotrophic Lateral Sclerosis.

Publications

Median Nerve-Neurophysiological Index Correlates With the Survival of Patients With Amyotrophic Lateral Sclerosis.

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