In vivo assessment of muscle membrane properties in myotonic dystrophy.

Tan, S Veronica; Z'Graggen, Werner Josef; Boërio, Delphine; Turner, Christopher; Hanna, Michael G; Bostock, Hugh (2016). In vivo assessment of muscle membrane properties in myotonic dystrophy. Muscle & nerve, 54(2), pp. 249-257. John Wiley & Sons 10.1002/mus.25025

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INTRODUCTION Myotonia in myotonic dystrophy types 1 (DM1) and 2 (DM2) is generally attributed to reduced chloride-channel conductance. We used muscle velocity recovery cycles (MVRCs) to investigate muscle membrane properties in DM1 and DM2, using comparisons with myotonia congenita (MC). METHODS MVRCs and responses to repetitive stimulation were compared between patients with DM1 (n = 18), DM2 (n = 5), MC (n = 18), and normal controls (n = 20). RESULTS Both DM1 and DM2 showed enhanced late supernormality after multiple conditioning stimuli, indicating delayed repolarization as in MC. Contrary to MC, however, DM1 showed reduced early supernormality after multiple conditioning stimuli, and weak DM1 patients also showed abnormally slow latency recovery after repetitive stimulation. CONCLUSIONS These findings support the presence of impaired chloride conductance in both DM1 and DM2. The early supernormality changes indicate that sodium currents were reduced in DM1, whereas the weakness-associated slow recovery after repetitive stimulation may provide an indication of reduced Na(+) /K(+) -ATPase activation. Muscle Nerve 54: 249-257, 2016.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurosurgery

UniBE Contributor:

Z'Graggen, Werner Josef


600 Technology > 610 Medicine & health




John Wiley & Sons




Nicole Söll

Date Deposited:

08 Mar 2017 11:46

Last Modified:

09 Sep 2017 18:44

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

channelopathy; chloride channel; excitability; membrane potential; myotonic dystrophy; sodium channel; sodium-potassium pump; velocity recovery cycle




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