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Updated: May 9, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
Rapidly progressive amyotrophic lateral sclerosis initially masquerading as a demyelinating neuropathy
R Ahdab1, A Créange, C Saint-Val
1EA 4391, faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France.
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.
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