open access

Vol 47, No 1 (2013)
ARTYKUŁ ORYGINALNY
Submitted: 2012-04-23
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Relationships between clinical data and quantitative EMG findings in facioscapulohumeral muscular dystrophy

Małgorzata Dorobek, Elżbieta Szmidt-Sałkowska, Katarzyna Rowińska-Marcińska, Małgorzata Gaweł, Irena Hausmanowa-Petrusewicz
DOI: 10.5114/ninp.2013.32936
·
Neurol Neurochir Pol 2013;47(1):8-17.

open access

Vol 47, No 1 (2013)
ARTYKUŁ ORYGINALNY
Submitted: 2012-04-23

Abstract

Background and purpose

In recently published reports, electrophysiological findings were analysed, in some facioscapulohumeral muscular dystrophy (FSHD) cases without genetic disease confirmation. In several reports, some electrophysiological findings were described, not specific for myopathy. The aim of study was to analyse electrophysiological findings in a genetically homogeneous FSHD group to find possible relationships between electromyography (EMG) abnormalities and clinical symptoms.

Material and methods

37 patients with genetically proven FSHD (23 men and 14 women) aged 7–58 years (mean 28.8 years) were studied. Electromyographic examinations were done according to a uniform scheme for FSHD. Quantitative EMG examination was performed in vastus lateralis, tibialis anterior, deltoid and biceps brachii muscles.

Results

There was no correlation between clinical features and electrophysiological findings. EMG confirmed myopathic changes in all patients with most advanced changes in tibialis anterior and deltoid muscles. Some of these changes were unspecific for myopathy and the degree of their intensity differed in particular muscles. The most advanced changes were observed in the tibialis anterior and deltoid muscles. The usefulness of the size index for myopathic processes assessment was confirmed. Analysis of so-called outliers for motor unit activity potential parameters did not show any new data for evaluation of the myopathic process. Myopathic changes in our material were not as advanced as those described in classical dystrophies. Histopathological examinations of skeletal muscle were normal in about 1/3 of patients.

Conclusions

We established that myopathic changes are clearly present in FSHD, with different degrees of intensity, most pronounced in tibialis anterior and deltoid muscles. There was no correlation between electrophysiological findings and clinical features. The size index provided the highest motor unit potential diagnostic sensitivity in FSHD.

Abstract

Background and purpose

In recently published reports, electrophysiological findings were analysed, in some facioscapulohumeral muscular dystrophy (FSHD) cases without genetic disease confirmation. In several reports, some electrophysiological findings were described, not specific for myopathy. The aim of study was to analyse electrophysiological findings in a genetically homogeneous FSHD group to find possible relationships between electromyography (EMG) abnormalities and clinical symptoms.

Material and methods

37 patients with genetically proven FSHD (23 men and 14 women) aged 7–58 years (mean 28.8 years) were studied. Electromyographic examinations were done according to a uniform scheme for FSHD. Quantitative EMG examination was performed in vastus lateralis, tibialis anterior, deltoid and biceps brachii muscles.

Results

There was no correlation between clinical features and electrophysiological findings. EMG confirmed myopathic changes in all patients with most advanced changes in tibialis anterior and deltoid muscles. Some of these changes were unspecific for myopathy and the degree of their intensity differed in particular muscles. The most advanced changes were observed in the tibialis anterior and deltoid muscles. The usefulness of the size index for myopathic processes assessment was confirmed. Analysis of so-called outliers for motor unit activity potential parameters did not show any new data for evaluation of the myopathic process. Myopathic changes in our material were not as advanced as those described in classical dystrophies. Histopathological examinations of skeletal muscle were normal in about 1/3 of patients.

Conclusions

We established that myopathic changes are clearly present in FSHD, with different degrees of intensity, most pronounced in tibialis anterior and deltoid muscles. There was no correlation between electrophysiological findings and clinical features. The size index provided the highest motor unit potential diagnostic sensitivity in FSHD.

Get Citation

Keywords

myopathy, facioscapulohumeral muscular dystrophy, electromyography, motor unit potential, size index

About this article
Title

Relationships between clinical data and quantitative EMG findings in facioscapulohumeral muscular dystrophy

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 47, No 1 (2013)

Pages

8-17

DOI

10.5114/ninp.2013.32936

Bibliographic record

Neurol Neurochir Pol 2013;47(1):8-17.

Keywords

myopathy
facioscapulohumeral muscular dystrophy
electromyography
motor unit potential
size index

Authors

Małgorzata Dorobek
Elżbieta Szmidt-Sałkowska
Katarzyna Rowińska-Marcińska
Małgorzata Gaweł
Irena Hausmanowa-Petrusewicz

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