Abstract
Lower motor neuron lesions are not among the characteristic features of multiple system
atrophy (MSA), although electromyography (EMG) and autopsy studies revealed peripheral
neuron abnormalities in some cases of MSA.
The aim of the study was to evaluate subclinical involvement of the peripheral neuron
in MSA using EMG and electroneurography (ENG).
Material
48 patients with clinically probable MSA (mean age 60.6 years; 67% males) were included in the study and divided into subgroups, with predominant
cerebellar (MSA-C) and parkinsonian signs (MSA-P).
Methods
ENG in ulnar, peroneal and sural nerves and EMG of the first interosseus dorsal and
tibial anterior muscles were performed.
Results
Abnormal ENG in one nerve was recorded in 20.8% of patients, and in two nerves in
another 20.8% of patients. The most frequent and significant findings were decreased
compound motor action potential amplitudes in the ulnar nerve in the overall MSA group
as well as in the MSA-P type as compared to controls. Abnormalities suggesting reinnervation
was observed in 43 of 96 examined muscles (44.7%). In individual cases, neurogenic
features were recorded in one muscle in 31.2% of patients and in two muscles in 29.1%
of patients.
Conclusions
Subclinical axonopathy in MSA is not frequent and is more pronounced in MSA with predominant
parkinsonian signs. In MSA, neurogenic EMG abnormalities in muscles are more frequent
than peripheral nerve lesions and as evidenced by increased motor unit potential amplitudes,
could be considered a sign of anterior horn cell involvement and a hallmark of the
“continuum” of neurodegeneration in MSA.
Keywords
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Article info
Publication history
Published online: May 30, 2012
Accepted:
May 4,
2012
Received in revised form:
March 30,
2012
Received:
February 1,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.