open access

Vol 49, No 3 (2015)
Original research articles
Submitted: 2014-12-08
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Peripheral nerve involvement in myotonic dystrophy type 2 – similar or different than in myotonic dystrophy type 1?

Monika Nojszewska, Anna Łusakowska, Elżbieta Szmidt-Salkowska, Małgorzata Gaweł, Marta Lipowska, Anna Sułek, Wioletta Krysa, Marta Rajkiewicz, Andrzej Seroka, Katarzyna Kaczmarek, Anna M. Kamińska
DOI: 10.1016/j.pjnns.2015.04.008
·
Neurol Neurochir Pol 2015;49(3):164-170.

open access

Vol 49, No 3 (2015)
Original research articles
Submitted: 2014-12-08

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.

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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Keywords

Myotonic dystrophy, Myotonic dystrophy type 1, Myotonic dystrophy type 2, Nerve conduction studies, Polyneuropathy

About this article
Title

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

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 49, No 3 (2015)

Pages

164-170

DOI

10.1016/j.pjnns.2015.04.008

Bibliographic record

Neurol Neurochir Pol 2015;49(3):164-170.

Keywords

Myotonic dystrophy
Myotonic dystrophy type 1
Myotonic dystrophy type 2
Nerve conduction studies
Polyneuropathy

Authors

Monika Nojszewska
Anna Łusakowska
Elżbieta Szmidt-Salkowska
Małgorzata Gaweł
Marta Lipowska
Anna Sułek
Wioletta Krysa
Marta Rajkiewicz
Andrzej Seroka
Katarzyna Kaczmarek
Anna M. Kamińska

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