Interpretation of electrodiagnostic findings in sporadic progressive muscular atrophy.
OBJECTIVE
We present the electrophysiologic data at baseline of 37 patients who were included in our prospective study on sporadic adult-onset progressive muscular atrophy (PMA). The aim was to correlate electrophysiological signs of lower motor neuron (LMN) loss with clinical signs of LMN loss, and to determine the prognostic value of the distribution of electrophysiological abnormalities in patients who presented clinically with only lower motor neuron signs.


METHODS
Thirty-seven patients, who met our inclusion criteria for a prospective study on sporadic adult-onset PMA, underwent extensive standardized electrophysiological examination at baseline, consisting of concentric needle EMG in three regions (cervical, thoracic and lumbosacral) and standardized nerve conduction studies.


RESULTS
Denervation on needle EMG was found in 88 % of clinically affected and in 40 % of clinically unaffected limb regions. All patients with a segmental or distal phenotype at baseline who developed generalized weakness had denervation in the thoracic region. Motor nerve conduction abnormalities were found in a substantial number of nerves and included reduced CMAP amplitude, increased distal motor latency, decreased motor conduction velocity, and F-wave abnormalities. Signs of demyelination and sensory nerve conduction abnormalities were rare.


CONCLUSIONS
Our electrophysiological data in patients recently diagnosed with sporadic progressive muscular atrophy are consistent with widespread LMN loss. Progression in patients with a segmental or distal onset of PMA may be likely if denervation is found in clinically unaffected regions, including the thoracic region.
Visser
J
J
Dept. of Neurology, Academic Medical Center, University of Amsterdam, 22700, 1100 DE, Amsterdam, The Netherlands. j.visser@amc.uva.nl
de Visser
M
M
Van den Berg-Vos
R M
RM
Van den Berg
L H
LH
Wokke
J H J
JH
de Jong
J M B V
JM
Franssen
H
H
D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000503:physiopathology; D018450:Disease Progression; D004568:Electrodiagnosis / Q000379:methods*; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000503:physiopathology*; D009046:Motor Neurons / Q000473:pathology*; D018908:Muscle Weakness / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000294:innervation / Q000503:physiopathology; D009134:Muscular Atrophy, Spinal / Q000175:diagnosis* / Q000503:physiopathology*; D009410:Nerve Degeneration / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology; D010525:Peripheral Nerves / Q000503:physiopathology; D011237:Predictive Value of Tests; D011446:Prospective Studies