Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis

Objective: Since motor nerve conduction slowing can occur due to loss of large axons, we investigate the conduction slowing profile in amyotrophic lateral sclerosis (ALS) and identify the limits beyond which the diagnosis of exclusive axonal loss is unlikely. Methods: First, using linear regression...

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Main Authors: Nizar Souayah, Ankit Pahwa, Mustafa Jaffry, Tejas Patel, Abu Nasar, Zhao Zhong Chong, Howard W. Sander
Format: Article
Language:English
Published: Elsevier 2023-08-01
Series:Heliyon
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2405844023056086
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author Nizar Souayah
Ankit Pahwa
Mustafa Jaffry
Tejas Patel
Abu Nasar
Zhao Zhong Chong
Howard W. Sander
author_facet Nizar Souayah
Ankit Pahwa
Mustafa Jaffry
Tejas Patel
Abu Nasar
Zhao Zhong Chong
Howard W. Sander
author_sort Nizar Souayah
collection DOAJ
description Objective: Since motor nerve conduction slowing can occur due to loss of large axons, we investigate the conduction slowing profile in amyotrophic lateral sclerosis (ALS) and identify the limits beyond which the diagnosis of exclusive axonal loss is unlikely. Methods: First, using linear regression analysis, we established the range of motor conduction slowing in 76 chronic inflammatory demyelinating polyneuropathy (CIDP) patients. Demyelinating range confidence intervals were defined by assessing conduction velocity (CV), distal latency (DML), and F-wave latency (F) in relation to distal compound muscle action potential (CMAP) amplitude of median, ulnar, fibular, and tibial nerves. Results were subsequently validated in 38 additional CIDP patients. Then, the newly established demyelination confidence intervals were used to investigate the profile of conduction slowing in 95 ALS patients. Results: CV slowing, prolonged DML, and abnormal F were observed in 22.2%, 19.6%, and 47.1% of the studied nerves respectively in ALS patients. When slowing occurred, it affected more than one segment of the motor nerve, suggesting that CMAP amplitude dependent conduction slowing caused by an exclusive loss of large axons is the main mechanism of slowing. No ALS patient had more than 2 nerves with CV slowing in the confidence interval defined by the regression equations or the American Academy of Neurology (AAN) research criteria for CIDP diagnosis. Conclusions: The presence of more than two motor nerves with CV slowing in the demyelinating range defined by the regression analysis or AAN criteria in ALS patients suggests the contribution of acquired demyelination or other additional mechanisms exist in the electrodiagnostic profile of ALS.
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spelling doaj.art-dac7032a0ad841ab95c2a352505469fe2023-08-30T05:51:12ZengElsevierHeliyon2405-84402023-08-0198e18400Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosisNizar Souayah0Ankit Pahwa1Mustafa Jaffry2Tejas Patel3Abu Nasar4Zhao Zhong Chong5Howard W. Sander6Department of Neurology, Rutgers University, New Jersey Medical School, 90 Bergen Street DOC 8100, Newark, NJ, 07101, USA; Corresponding author.SMR Consulting, 407 Elmwood Avenue, Sharon Hill, PA, 19079, USADepartment of Neurology, Rutgers University, New Jersey Medical School, 90 Bergen Street DOC 8100, Newark, NJ, 07101, USADepartment of Neurology, Rutgers University, New Jersey Medical School, 90 Bergen Street DOC 8100, Newark, NJ, 07101, USADepartment of Neurology, Rutgers University, New Jersey Medical School, 90 Bergen Street DOC 8100, Newark, NJ, 07101, USADepartment of Neurology, Rutgers University, New Jersey Medical School, 90 Bergen Street DOC 8100, Newark, NJ, 07101, USADepartment of Neurology, NYU Grossman School of Medicine, New York, NY, 10016, USAObjective: Since motor nerve conduction slowing can occur due to loss of large axons, we investigate the conduction slowing profile in amyotrophic lateral sclerosis (ALS) and identify the limits beyond which the diagnosis of exclusive axonal loss is unlikely. Methods: First, using linear regression analysis, we established the range of motor conduction slowing in 76 chronic inflammatory demyelinating polyneuropathy (CIDP) patients. Demyelinating range confidence intervals were defined by assessing conduction velocity (CV), distal latency (DML), and F-wave latency (F) in relation to distal compound muscle action potential (CMAP) amplitude of median, ulnar, fibular, and tibial nerves. Results were subsequently validated in 38 additional CIDP patients. Then, the newly established demyelination confidence intervals were used to investigate the profile of conduction slowing in 95 ALS patients. Results: CV slowing, prolonged DML, and abnormal F were observed in 22.2%, 19.6%, and 47.1% of the studied nerves respectively in ALS patients. When slowing occurred, it affected more than one segment of the motor nerve, suggesting that CMAP amplitude dependent conduction slowing caused by an exclusive loss of large axons is the main mechanism of slowing. No ALS patient had more than 2 nerves with CV slowing in the confidence interval defined by the regression equations or the American Academy of Neurology (AAN) research criteria for CIDP diagnosis. Conclusions: The presence of more than two motor nerves with CV slowing in the demyelinating range defined by the regression analysis or AAN criteria in ALS patients suggests the contribution of acquired demyelination or other additional mechanisms exist in the electrodiagnostic profile of ALS.http://www.sciencedirect.com/science/article/pii/S2405844023056086Amyotrophic lateral sclerosisChronic inflammatory demyelinating polyneuropathyConduction slowingNeuropathyCMAP
spellingShingle Nizar Souayah
Ankit Pahwa
Mustafa Jaffry
Tejas Patel
Abu Nasar
Zhao Zhong Chong
Howard W. Sander
Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis
Heliyon
Amyotrophic lateral sclerosis
Chronic inflammatory demyelinating polyneuropathy
Conduction slowing
Neuropathy
CMAP
title Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis
title_full Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis
title_fullStr Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis
title_full_unstemmed Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis
title_short Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis
title_sort electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis
topic Amyotrophic lateral sclerosis
Chronic inflammatory demyelinating polyneuropathy
Conduction slowing
Neuropathy
CMAP
url http://www.sciencedirect.com/science/article/pii/S2405844023056086
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