Peripheral Nerve Axon Involvement in Myotonic Dystrophy Type 1, Measured Using the Automated Nerve Excitability Test.
- Author:
Jong Seok BAE
1
;
Sang Gin KIM
;
Jeong Cheol LIM
;
Eun Joo CHUNG
;
Oeung Kyu KIM
Author Information
- Publication Type:Original Article
- Keywords: myotonic dystrophy; neuropathy; threshold tracking; axonal excitability; nerve excitability test
- MeSH: Axons; Diabetes Mellitus; Humans; Membrane Potentials; Myotonic Dystrophy; Peripheral Nerves; Peripheral Nervous System Diseases; Sarcosine; Thiocarbamates
- From:Journal of Clinical Neurology 2011;7(2):90-95
- CountryRepublic of Korea
- Language:English
- Abstract: BACKGROUND AND PURPOSE: Primary involvement of the peripheral nerves in myotonic dystrophy type I (MyD1) is controversial. We investigated whether the involvement of peripheral nerves is a primary event of MyD1 or secondary to another complication such as diabetes mellitus (DM). METHODS: The subjects comprised 12 patients with MyD1, 12 with DM and no peripheral nerve involvement, and 25 healthy volunteers. We measured multiple excitability indices in the median motor axons. The strength-duration time constant was calculated from the duration-charge curve, the threshold electrotonus and current-threshold relationships were calculated from the sequential subthreshold current, and the recovery cycle was derived from double suprathreshold stimulation. RESULTS: The depolarizing and hyperpolarizing threshold electrotonus were significantly reduced and exhibited increased refractoriness in the MyD1 group compared with the DM and control groups. The SDTC, superexcitability, and subexcitability were not significantly altered in the MyD1 group. CONCLUSIONS: The MyD1 group exhibited a depolarized axonal membrane potential. The significant differences in peripheral nerve excitability between the MyD1 group and the DM and normal control groups suggest that peripheral neuropathy is a primary event in MyD1 rather than a secondary complication of DM.
