Primary Lateral Sclerosis Natural History Study: Primary Lateral Sclerosis Functional Rating Scale and Other Outcomes Assessment.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Lee Ikjae, Jang Grace, Cheung Ying Kuen Ken, Sherman Alexander V, Johnston Wendy S, Diaz Frank, Fernandes J Americo M, Habib Ali A, Maragakis Nicholas J, Paganoni Sabrina, Burke Katherine, Oskarsson Bjorn, Shah Jaimin, Zinman Lorne, Floeter Mary Kay, Ajroud-Driss Senda, Gwathmey Kelly, Heiman-Patterson Terry, Jawdat Omar, Kasarskis Edward J, Kisanuki Yaz Y, Wymer James, Fournier Christina, Hayat Ghazala, Heitzman Daragh, Lomen-Hoerth Catherine, Pulley Michael T, Scelsa Stephen N, Walk David, Goutman Stephen A, Shoesmith Christen, Simmons Zachary, Sorenson Eric, Elman Lauren, Harms Matthew B, Hoover Benjamin N, Yun Rebecca Y, Santella Regina M, Mitsumoto Hiroshi, PLS NHS Study Group
Аннотация
METHODS: The PLS NHS is a prospective, longitudinal, multicenter study of people living with PLS in different diagnostic categories: early (disease duration <2 years); probable (2-4 years); and definite PLS (4-15 years). PLSFRS scores and other functional outcome measures were collected at baseline, 3-, 6-, 9-, and 12-month follow-up visits. Baseline characteristics were compared between the groups. The slopes of the PLSFRS and other functional outcome measures over 12 months were examined in the overall cohort and subgroups using linear mixed-effect models. The associations between baseline characteristics and the rate of PLSFRS decline were analyzed with linear regression models.
RESULTS: A total of 76 participants were included: early (n = 6); probable (n = 26); and definite (n = 44) PLS. Baseline PLSFRS total scores were highest in the early PLS group, followed by the probable and definite PLS groups. In the overall cohort, the PLSFRS total score declined by 0.33 points/month (95% confidence interval [0.27-0.39], adjusted p < 0.05). The rate of decline was steepest in the early PLS group, followed by the probable and definite PLS groups. Baseline neurofilament light chain level was associated with the rate of PLSFRS decline over 1 year (p = 0.001).
INTERPRETATION: In PLS, the rate of functional decline, as measured by the PLSFRS total score, is faster during the early phase of the disease. Neurofilament light might serve as a prognostic biomarker in PLS. ANN NEUROL 2026;99:418-428.