Vol 49, No 3 (2015)

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Peripheral nerve involvement in myotonic dystrophy type 2 – similar or different than in myotonic dystrophy type 1?

Monika Nojszewska1, Anna Łusakowska1, Elżbieta Szmidt-Salkowska1, Małgorzata Gaweł1, Marta Lipowska1, Anna Sułek2, Wioletta Krysa2, Marta Rajkiewicz2, Andrzej Seroka1, Katarzyna Kaczmarek1, Anna M. Kamińska1
DOI: 10.1016/j.pjnns.2015.04.008
Neurol Neurochir Pol 2015;49(3):164-170.

Abstract

Introduction

Multisystem manifestations of myotonic dystrophies type 1 (DM1) and 2 (DM2) are well known. Peripheral nerve involvement has been reported in DM1 but not in genetically confirmed DM2. The aim of our study was to assess peripheral nerve involvement in DM2 using nerve conduction studies and to compare these results with findings in DM1.

Methods

We prospectively studied patients with genetically confirmed DM2 (n=30) and DM1 (n=32). All patients underwent detailed neurological examination and nerve conduction studies.

Results

Abnormalities in electrophysiological studies were found in 26.67% of patients with DM2 and in 28.13% of patients with DM1 but the criteria of polyneuropathy were fulfilled in only 13.33% of patients with DM2 and 12.5% of patients with DM1. The polyneuropathy was subclinical, and no correlation was found between its presence and patient age or disease duration.

Conclusions

Peripheral nerves are quite frequently involved in DM2, but abnormalities meeting the criteria of polyneuropathy are rarely found. The incidence of peripheral nerve involvement is similar in both types of myotonic dystrophy.

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Neurologia i Neurochirurgia Polska