Prospective Validation of the New PLS Diagnostic Criteria From PLS Natural History Study: EMG and Neurofilament Analyses.
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Sorenson Eric, Heitzman Daragh, Lee Ikjae, Jang Grace, Elman Lauren, Habib Ali A, Wymer James, Hayat Ghazala, Goutman Stephen A, Fernandes J Americo M, Floeter Mary Kay, Ajroud-Driss Senda, Gwathmey Kelly, Kasarskis Edward, Kisanuki Yasushi Y, Lomen-Hoerth Catherine, Walk David, Johnston Wendy S, Diaz Frank, Maragakis Nicholas J, Paganoni Sabrina, Shah Jaimin, Oskarsson Bjorn, Zinman Lorne, Heiman-Patterson Terry, Jawdat Omar, Fournier Christina N, Pulley Michael T, Scelsa Stephen N, Shoesmith Christen, Simmons Zachary, Sherman Alexander V, Hoover Benjamin N, Yun Rebecca Y, Cheung Ken, Mitsumoto Hiroshi, PLS NHS Study Group
Аннотация
METHODS: Seventy-six PLS participants enrolled in this prospective natural history study. EMG studies, blood neurofilament light chain levels (NfLs), and demographic characteristics were obtained at baseline. At 1-year follow-up, repeat EMG studies were conducted to determine which participants fulfilled criteria for ALS. Baseline characteristics were then compared to determine features that predict reclassification.
RESULTS: Seventy participants completed 1-year follow-up. Five of the 70 were reclassified to ALS (7.1%). Those reclassified had higher trends in baseline blood NfL levels (91.4 vs. 34.0 pg/mL, p = 0.13) and shorter symptom duration (39 vs. 69 months in the PLS group, p = 0.15). Reclassification was noted in both probable and definite PLS participants. All cases with a symptomatic duration of less than 2 years retained the PLS phenotype (5 of 5). NfL levels over 90 pg/mL predicted reclassification with 94% specificity and 60% sensitivity. No other features predicted reclassification to ALS.
CONCLUSIONS: In our population, reclassification of PLS to ALS occurred at a low frequency at 1 year follow-up (7.1%). Baseline NfL was the strongest predictor in differentiating UMN dominant ALS from PLS at 1-year follow-up. Based on our data, we propose EMG and NfL criteria for enrollment in future PLS trials.