Journal
CLINICAL NEUROPHYSIOLOGY
Volume 132, Issue 12, Pages 2996-3001Publisher
ELSEVIER IRELAND LTD
DOI: 10.1016/j.clinph.2021.08.022
Keywords
Primary lateral sclerosis; Amyotrophic lateral sclerosis; Electromyography; Fasciculation potentials; Motor unit potentials
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Funding
- Fundacao para a Ciencia e Tecnologia [PTDC/CCICIF/4613/2020]
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Most PLS patients exhibit minor and stable EMG abnormalities, but those with more EMG abnormalities show a faster disease progression.
Objective: To characterize electromyographic (EMG) findings in patients with primary lateral sclerosis (PLS) during the disease course. Methods: In PLS patients we scored spontaneous activity and motor unit action potential (MUP) pattern on EMG. We compared patients according to lower (group A) and higher (group B) EMG scores. EMG studies were repeated at intervals longer than 11 months; two or three repeat studies were required for inclusion in the analysis. Results: We studied 22 patients. Fasciculation potentials were found in 13 and fibrillations/positive sharp waves (fibs/sw) in 3 patients. Both were stable over time. Most patients had MUP abnormalities (n = 17), with worsening in the lower limbs in patients with three evaluations (p = 0.010). Compared to group A (n = 12), patients of group B (n = 10) had a significant shorter disease duration (median 10.9 vs 15.2 years, p < 0.001), lower functional score at both first (39 vs 45, p = 0.034) and last (29 vs 38, p = 0.003) evaluations, and had a faster functional decline (0.19 vs 0.08, p = 0.004). Conclusions: Most PLS patients showed minor and stable EMG abnormalities, without progression to ALS. Patients with more EMG abnormalities have a faster progression. Significance: EMG abnormalities in most PLS patients are minor and stable. (C) 2021 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.
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