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OBJECTIVE: We sought to assess cortical function in amyotrophic lateral sclerosis (ALS) using noninvasive neural signal recording. METHODS: Resting-state magnetoencephalography was used to measure power fluctuations in neuronal oscillations from distributed cortical parcels in 24 patients with ALS and 24 healthy controls. A further 9 patients with primary lateral sclerosis and a group of 15 asymptomatic carriers of genetic mutations associated with ALS were also studied. RESULTS: Increased functional connectivity, particularly from the posterior cingulate cortex, was demonstrated in both patient groups compared to healthy controls. Directionally similar patterns were also evident in the asymptomatic genetic mutation carrier group. CONCLUSION: Increased cortical functional connectivity elevation is a quantitative marker that reflects ALS pathology across its clinical spectrum, and may develop during the presymptomatic period. The amelioration of pathologic magnetoencephalography signals might be a marker sensitive enough to provide proof-of-principle in the development of future neuroprotective therapeutics.

Original publication

DOI

10.1212/WNL.0000000000005333

Type

Journal article

Journal

Neurology

Publication Date

17/04/2018

Volume

90

Pages

e1418 - e1424

Keywords

Adult, Aged, Amyotrophic Lateral Sclerosis, Brain Mapping, C9orf72 Protein, Cerebral Cortex, Cognition Disorders, Electrocardiography, Female, Humans, Magnetoencephalography, Male, Middle Aged, Mutation, Nerve Net, Neuropsychological Tests, Rest, Superoxide Dismutase-1, Young Adult