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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
[Neurogenic electromyographic activity in a patient with non-paraneoplasic Lambert-Eaton syndrome]
B Lapergue1, J-P Lefaucheur, A Bourahoui
1Service de neurologie, hôpital Henri-Mondor, AP-HP, Université Paris XII, Créteil.
Introduction:
Lambert-Eaton myasthenic syndrome (LEMS) is a rare presynaptic disorder of the neuromuscular junction. Electrodiagnosis is characterized by compound muscle action potentials of small amplitude at rest, normalizing immediately after brief exercise or high-rate nerve stimulation. Needle electromyographic (EMG) activity is classically described as normal or myogenic.
Case:
We report the case of a young patient with a non-paraneoplastic LEMS in whom the initial electroneuromyographic examination showed neurogenic changes in needle EMG concomitant with typical features of presynaptic neuromuscular junction disorder at single and repetitive nerve stimulation.
Discussion:
Neurogenic EMG abnormalities were not previously described in patients with LEMS and could result from "functional" reversible denervation, depending on the presence of anti-calcium channel antibodies at axon terminals.
Conclusion:
Neurogenic features in needle EMG examination in case of subacute motor deficiency should not eliminate the possibility of a presynaptic neuromuscular junction disorder, such as LEMS.
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