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. 2021 Aug 12;3(2):e000164. doi: 10.1136/bmjno-2021-000164

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© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

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Nusinersen does not result in overt improvement of CMAP decrement during RNS but does improve CMAP amplitude. (A) Cross-sectional analysis of CMAP decrement demonstrated no significant difference in assessments after 14 months of nusinersen (14 months of nusinersen treatment: −11.9%±8.3%, n=15, nine ambulatory) vs assessments prior to treatment (−14.2%±11.5% decrement, n=17, seven ambulatory) (unpaired t-test, p=0.5202). (B) Similarly, longitudinal assessment of a subset of participants (n=8, three ambulatory) demonstrated that CMAP amplitude decrement following 14 months of treatment (−13.9%±6.7%) was similar to baseline (−16.9%±13.4%) (paired t-test, p=0.5863). (C) Cross-sectional analysis of CMAP amplitude demonstrated no significant difference after 14 months of treatment (4.3±2.4 mV, n=15, nine ambulatory) vs assessments prior to treatment (4.3±3.4 mV, n=17, seven ambulatory) (unpaired t-test, p=0.9871). (D) In contrast, CMAP amplitude was significantly improved with nusinersen when analysed in a subset of participants (n=8, three ambulatory) that underwent assessment prior to treatment (2.6±2.0 mV) and after 14 months of nusinersen (4.2±2.8 mV) (paired t-test, p=0.0383). CMAP, compound muscle action potential; RNS, repetitive nerve stimulation.