Cortical Excitability as a Prognostic and Phenotypic Stratification Biomarker in Amyotrophic Lateral Sclerosis.
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.
Ranieri Federico, Senerchia Gianmaria, Bonan Luigi, Casali Stefania, Cabona Corrado, Cantone Mariagiovanna, De Marchi Fabiola, Diamanti Luca, Doretti Alberto, Fini Nicola, Filosto Massimiliano, Fortuna Andrea, Iovino Aniello, Iuzzolino Valentina Virginia, Lanza Giuseppe, Lunetta Christian, Maderna Luca, Mandrioli Jessica, Mazzini Letizia, Musumeci Gabriella, Nuredini Andi, Sorarù Gianni, Toriello Antonella, Ticozzi Nicola, Todisco Massimiliano, Vacchiano Veria, Zinno Lucia, Silani Vincenzo, Rossi Simone, Di Lazzaro Vincenzo, Dubbioso Raffaele, MND Study Group of the Italian Neurological Society (SIN) and the Neurostimulation‐Neuromodulation Study Group of the Italian Society of Clinical Neurophysiology (SINC)
Аннотация
METHODS: In this multicenter retrospective study, 743 amyotrophic lateral sclerosis patients from 16 tertiary centers in Italy were analyzed. The MEP:CMAP ratio, recorded from upper limb muscles, was categorized as hyperexcitable, normal, or hypoexcitable. Phenotypes included progressive muscular atrophy (or lower motor neuron), flail arm/leg, classic, bulbar, patient with predominant upper motor neuron signs (or pyramidal), and primary lateral sclerosis. Disease stage was assessed using King's staging. Survival was analyzed using Kaplan-Meier curves and Cox regression models.
RESULTS: The MEP:CMAP ratio differed significantly across phenotypes (p < 0.0001), with hyperexcitability predominating in lower motor neuron, flail, classic, and bulbar forms, and hypoexcitability in pyramidal and primary lateral sclerosis. Hypoexcitability increased in advanced King's stages (p < 0.0001). Hyperexcitable patients had shorter survival (p = 0.004), including when tested within 1 year of onset (p = 0.006). Cox regression identified the MEP:CMAP ratio as an independent survival predictor (HR 1.84, 95% CI 1.12-3.03, p = 0.016).
INTERPRETATION: This real-world study supports the clinical value of the MEP:CMAP ratio as a scalable biomarker of cortical excitability in amyotrophic lateral sclerosis, with prognostic relevance across phenotypes and disease stages. ANN NEUROL 2025;98:801-813.