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Peripheral nerve involvement in myotonic dystrophy type 2 – similar or different than in myotonic dystrophy type 1?
- Department of Neurology, Medical University of Warsaw, 8 Kondratowicza str, 03-242 Warsaw, Poland
- Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland
open access
Abstract
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.
MethodsWe prospectively studied patients with genetically confirmed DM2 (n=30) and DM1 (n=32). All patients underwent detailed neurological examination and nerve conduction studies.
ResultsAbnormalities 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.
ConclusionsPeripheral 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
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.
MethodsWe prospectively studied patients with genetically confirmed DM2 (n=30) and DM1 (n=32). All patients underwent detailed neurological examination and nerve conduction studies.
ResultsAbnormalities 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.
ConclusionsPeripheral 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.
Keywords
Myotonic dystrophy, Myotonic dystrophy type 1, Myotonic dystrophy type 2, Nerve conduction studies, Polyneuropathy
Title
Peripheral nerve involvement in myotonic dystrophy type 2 – similar or different than in myotonic dystrophy type 1?
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
164-170
Page views
278
Article views/downloads
420
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