Oligosymptomatic Forms of Myotonic Dystrophy Type 2

Česká verzia

Authors: Z. Ambler
Authors place of work: Neurologická klinika LF UK a FN Plzeň
Published in the journal: Cesk Slov Neurol N 2012; 75/108(2): 237-239
Category: Case Report

Summary

Myotonic dystrophy type 2 (DM2) typically presents in adult life with lower extremity proximal muscle weakness, variously expressed myotonia and cataract being the most common symptoms. On electromyography (EMG) a broad spectrum of spontaneous activity with myotonic discharges can be found and motor unit action potentials may show myopathic pattern. Both clinical and EMG features in DM2 may be greatly variable. We present two cases of DM2 with proximal muscle weakness, spontaneous EMG activity but no myotonic discharges and no characteristic myopathic EMG pattern.

Key words:
myotonic dystrophy type 2 – myopathy – myotonia

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