Motor unit number index and neurophysiological index as candidate biomarkers of presymptomatic motor neuron loss in amyotrophic lateral sclerosis
| Autor: | Agessandro Abrahao, Nadia Iandoli de Oliveira Braga, Lorne Zinman, Marcio Luiz Escorcio-Bezerra, Acary Souza Bulle Oliveira, Gilberto Mastrocola Manzano, Karlo Faria Nunes |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Male medicine.medical_specialty Physiology Muscle Fibers Skeletal Action Potentials Cell Count Muscle Strength Dynamometer Lower motor neuron 03 medical and health sciences Cellular and Molecular Neuroscience 0302 clinical medicine Physical medicine and rehabilitation Physiology (medical) medicine Humans Prospective Studies Amyotrophic lateral sclerosis Aged Motor Neurons Hand Strength business.industry Electrodiagnosis Disease progression Amyotrophic Lateral Sclerosis Outcome measures Motor unit number Motor neuron Neurophysiology Middle Aged medicine.disease 030104 developmental biology medicine.anatomical_structure Treatment Outcome Disease Progression Biomarker (medicine) Female Neurology (clinical) business 030217 neurology & neurosurgery Biomarkers |
| Zdroj: | Musclenerve. 58(2) |
| ISSN: | 1097-4598 |
| Popis: | INTRODUCTION Our objective was to determine the utility of motor unit number index (MUNIX) and neurophysiological index (NI) as surrogate biomarkers of disease progression in limbs without clinical signs of lower motor neuron (LMN) involvement from patients with slowly progressive amyotrophic lateral sclerosis (ALS). METHODS Patients with slowly progressive ALS and at least 1 clinically unaffected limb were prospectively enrolled. Clinical signs of LMN loss and results from hand-held dynamometer (HHD), revised ALS Functional Rating Scale (ALSFRS-R), mean-MUNIX (from 3 different muscles), and NI were longitudinally recorded. RESULTS Eighteen patients with 43 presymptomatic muscles were evaluated. Twenty-seven muscles remained clinically unaffected during study, with stable ALSFRS-R subscores and HHD measures. However, a significant decline in mean-MUNIX and NI was detected. DISCUSSION Mean-MUNIX and NI were more sensitive than clinical measures at detecting LMN loss in presymptomatic limbs from patients with slowly progressive ALS. Therefore, these electrophysiological biomarkers should be included in early study phases as meaningful outcome measures. Muscle Nerve 58: 204-212, 2018. |
| Databáze: | OpenAIRE |
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