Abstract
Introduction: Non-dystrophic myotonia (NDM) is caused by mutations in muscle chloride and sodium channels. Currently, there is no standardized instrument for documenting symptom frequency and severity in NDM. Methods: Subjects used an automated, interactive, telephone-based voice response diary (IVR) to record frequency and severity of stiffness, weakness, pain, and tiredness once a week for 8 weeks, after their baseline visits. Results: We describe the IVR and report data on 76 subjects for a total of 385 person-weeks. Overall there were 5.1 calls per subject. Forty-eight subjects called in 5 or more times, and 14 called in 8 times. Stiffness was both the most frequent and severe symptom. Warm-up and handgrip myotonia were associated with higher severity scores for stiffness. Conclusions: IVR is a convenient technology to allow patient reporting of repeated and real-time symptom frequency and severity, and it is presently being used in a trial of mexiletine in NDM.
Original language | English (US) |
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Pages (from-to) | 30-35 |
Number of pages | 6 |
Journal | Muscle and Nerve |
Volume | 44 |
Issue number | 1 |
DOIs | |
State | Published - Jul 1 2011 |
Keywords
- Ion channel gene defects
- Muscle disease
- Myotonia
- Nondystrophic myotonia
- Patient-reported outcome measures
ASJC Scopus subject areas
- Physiology
- Clinical Neurology
- Cellular and Molecular Neuroscience
- Physiology (medical)