1. Rapidly progressive amyotrophic lateral sclerosis initially masquerading as a demyelinating neuropathy.
- Author
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Ahdab R, Créange A, Saint-Val C, Farhat WH, and Lefaucheur JP
- Subjects
- Amyotrophic Lateral Sclerosis physiopathology, Axons physiology, Cerebral Cortex physiopathology, Demyelinating Diseases physiopathology, Diagnosis, Differential, Electric Stimulation, Electromyography, Electrophysiological Phenomena physiology, Female, Humans, Lower Extremity innervation, Lower Extremity physiopathology, Male, Middle Aged, Motor Neurons physiology, Muscle Weakness etiology, Nerve Degeneration physiopathology, Neural Conduction physiology, Neurologic Examination, Peripheral Nerves physiopathology, Spinal Nerve Roots physiology, Upper Extremity innervation, Upper Extremity physiopathology, Amyotrophic Lateral Sclerosis diagnosis, Demyelinating Diseases diagnosis
- Abstract
Rare cases of demyelinating neuropathy have been described in association with amyotrophic lateral sclerosis (ALS). We report two patients with typical ALS whose initial electroneuromyographic (ENMG) presentation could suggest the existence of a process of motor nerve fiber demyelination. However, subsequent ENMG examinations and the fatal course of the disease in a few months rather supported severe ongoing axonal degeneration at the origin of motor nerve conduction abnormalities. Repeated examinations could be required to distinguish between ENMG features of concomitant demyelinating neuropathy and rapidly progressive motor neuron loss in ALS., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)
- Published
- 2013
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