## Abstract We report a patient with autosomal‐dominant amyotrophic lateral sclerosis (ALS) and a sequence variation in the __SOD1__ promoter region, located in the conserved TATA box motif (TATAAA→TGTAAA). Functional promoter studies of this variant in an in vitro system showed moderate reduction
Analysis of continuous diaphragm electromyographic signal: Results from a patient with amyotrophic lateral sclerosis
✍ Scribed by Hans D. Katzberg; Daniel F. Barros; Bernard Widrow; Charles S. Cho; Yuen T. So
- Publisher
- John Wiley and Sons
- Year
- 2011
- Tongue
- English
- Weight
- 523 KB
- Volume
- 43
- Category
- Article
- ISSN
- 0148-639X
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✦ Synopsis
Abstract
Introduction: Analysis of continuous diaphragm electromyography (dEMG) has not been well studied. We describe a system of analyzing continuous dEMG using implanted electrodes. Methods: dEMG signal was acquired via two pairs of electrodes near the diaphragm motor points. Raw bursts of dEMG signal were compared to externally captured electrocardiogram (ECG) using adaptive filtering in order to remove cardiac contamination. Differential energy levels were used to identify each dEMG burst, and average amplitude and area values from both hemidiaphragms were aggregated and averaged for the duration of the recording. Results: A 64‐year‐old patient with amyotrophic lateral sclerosis underwent three serial dEMG studies every 6 months. An average of three tracings were collected per visit, and all had excellent intertest reliability (κ > 0.8). Average dEMG area correlated with forced vital capacity and mean inspiratory pressure (r^2^ > 0.9). Conclusions: The approach described represents a comprehensive method for capturing and analyzing continuous diaphragm EMG signal. Muscle Nerve, 2011
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