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- W2887994816 abstract "Objective: Motor unit number index (MUNIX) as a tool to study disease severity in Spinal Muscular Atrophy (SMA) Background: Motor unit number index (MUNIX) is a validated electrophysiological biomarker in motor neuron disease. It’s a three-step procedure, first maximum compound muscle action potential (CMAP) is recorded from muscle. Second, surface EMG interference pattern (SIP), 200μV each, 300ms epoch, 10–1000Hz filter setting recorded at minimal, moderate, and maximal voluntary effort. Finally, SIP CMAP area and power are used to calculate ideal case motor unit count (ICMUC). ICUMUC is plotted against SIP and MUNIX is derived. MUNIX divided by CMAP is motor unit size index (MUSIX). Design/Methods: Genetic confirmed SMA type 2 subjects scheduled to receive Spinraza® underwent Hammersmith Motor function Scale Expanded (HMFSE) and MUNIX (Synergy, Natus Inc. Middleton, WI) of right abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. Study subjects were coached to incrementally activate APB and ADM to record twenty SIP epochs per muscle. Each muscle recording was done under five minutes by board certified pediatric electromyographer (S.V.). No sedation or analgesia was needed. Results: Nine subjects (5 girls, 4 boys) mean age 9.4±2.9 years (range 4–12 years) enrolled. Mean ± S.D APB and ADM CMAP (5.5±2.2mV, 2.6±2.4mV), MUNIX (73±38.4, 27.4±28.1, normal >80) and MUSIX (98.4±51.3μV, 138.6±82.5μV, normal Conclusions: APB and ADM muscle MUNIX studies are feasible in SMA type 2 children and correlate with disease severity on motor function testing (HMFSE). We plan to preform longitudinal MUNIX studies in SMA children receiving intrathecal Spinraza® and validate MUNIX as a potential biomarker for disease activity. Disclosure: Dr. Forte has nothing to disclose. Dr. Verma has nothing to disclose. Dr. Shah has nothing to disclose." @default.
- W2887994816 created "2018-08-22" @default.
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- W2887994816 date "2018-04-10" @default.
- W2887994816 modified "2023-09-23" @default.
- W2887994816 title "Motor Unit Number Index (MUNIX) in Spinal Muscular Atrophy (SMA) (P4.461)" @default.
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