Abstract
Background
Abnormalities of the spinal cord were considered uncommon in progressive supranuclear
palsy (PSP), and therefore spinal symptoms were not included among PSP characteristic
features. However there have been some neuropathological reports of spinal cord lesions
in patients with PSP. The aim of our study was to find out if the possible lower motor
neuron involvement in PSP is reflected by electromyographic (EMG) and/or electroneurographic
(ENG) abnormalities.
Material
24 patients with clinically probable PSP (mean age 67.5 yrs; 66% males) were included in the study. The control group for ENG studies consisted
25 age matched healthy volunteers.
Methods
Nerve conduction studies in the ulnar, peroneal and sural nerves and EMG of the first
interosseus dorsal and tibial anterior muscles were performed.
Results
The only ENG abnormality observed was decreased compound muscle action potential (CMAP)
and sensory nerve action potential (SNAP) amplitudes in the ulnar nerve. Such decrease
was registered in 8.3% and 20% of PSP patients respectively. There was no significant
difference between the values of ENG parameters between PSP patients and the control
group. In EMG abnormalities suggesting chronic reinnervation were recorded in the
first interosseous dorsal (FID) muscle in 45.8%, and in the tibialis anterior (TA)
muscle in 37.5% of PSP patients. A significant correlation was found between the age
of PSP patients and their mean motor unit potential (MUP) amplitude in TA muscle (p=0.04) and also between the age of onset and MUP amplitude in both, the TA and FID
muscles (p=0.026 and p=0.03 respectively).
Conclusions
In PSP, neurogenic EMG abnormalities in skeletal muscles are present in nearly half
the patients suggesting a loss of motor neurons in the anterior horns of the spinal
cord which is in line with our histopathological findings. In contrast, electrophysiological
signs of neuropathy in peripheral nerves in PSP are very rare. Concluding, although
PSP is characterized by the pathological process in specific basal ganglia and brainstem
areas, our electromyographic study suggests the need for broadening the spectrum of
PSP for lower motor neurons degeneration.
Keywords
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Article info
Publication history
Published online: November 12, 2012
Accepted:
October 24,
2012
Received in revised form:
October 19,
2012
Received:
August 19,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
