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A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis

Cervical spondylotic amyotrophy (CSA) is a rare clinical condition characterized by weakness and atrophy of the upper limb with minimal to no associated sensory deficits. The detection of the disease is based on clinical features at presentation, neurological examination, electrophysiological studie...

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Autores principales: Idiculla, Pretty Sara, Govindarajan, Raghav
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7548943/
https://www.ncbi.nlm.nih.gov/pubmed/33082770
http://dx.doi.org/10.1159/000509684
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author Idiculla, Pretty Sara
Govindarajan, Raghav
author_facet Idiculla, Pretty Sara
Govindarajan, Raghav
author_sort Idiculla, Pretty Sara
collection PubMed
description Cervical spondylotic amyotrophy (CSA) is a rare clinical condition characterized by weakness and atrophy of the upper limb with minimal to no associated sensory deficits. The detection of the disease is based on clinical features at presentation, neurological examination, electrophysiological studies, and imaging. The proposed pathophysiological mechanisms include selective damage to the ventral root or anterior horn cells of the spinal cord. Depending on the muscle groups that are involved, CSA is broadly classified into a proximal type and a distal type. The clinical profiles of patients with CSA and ALS have a very close resemblance to each other, especially at the early stages of the disease. Cervical spine magnetic resonance imaging (MRI) may show T2 hyperintensity in both proximal and distal types. Electromyogram demonstrates denervation potentials and reduced motor unit potentials in the affected muscles. The conservative management is often the first-line modality, and those who fail to respond to conservative treatment have severe muscular atrophy and weakness, and distal-type CSA are considered potential candidates for surgery. We present the case of a 57-year-old female who presented with a 1-year history of left-hand weakness and wasting with no sensory deficits. She denied any involvement of her other hand or bilateral lower limbs, and she was referred to our clinic with the potential diagnosis of amyotrophic lateral sclerosis (ALS). An elaborate history, physical examination, electrophysiological studies, and imaging assisted us in reaching the diagnosis of CSA, 1 year after the onset of symptoms.
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spelling pubmed-75489432020-10-19 A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis Idiculla, Pretty Sara Govindarajan, Raghav Case Rep Neurol Single Case − General Neurology Cervical spondylotic amyotrophy (CSA) is a rare clinical condition characterized by weakness and atrophy of the upper limb with minimal to no associated sensory deficits. The detection of the disease is based on clinical features at presentation, neurological examination, electrophysiological studies, and imaging. The proposed pathophysiological mechanisms include selective damage to the ventral root or anterior horn cells of the spinal cord. Depending on the muscle groups that are involved, CSA is broadly classified into a proximal type and a distal type. The clinical profiles of patients with CSA and ALS have a very close resemblance to each other, especially at the early stages of the disease. Cervical spine magnetic resonance imaging (MRI) may show T2 hyperintensity in both proximal and distal types. Electromyogram demonstrates denervation potentials and reduced motor unit potentials in the affected muscles. The conservative management is often the first-line modality, and those who fail to respond to conservative treatment have severe muscular atrophy and weakness, and distal-type CSA are considered potential candidates for surgery. We present the case of a 57-year-old female who presented with a 1-year history of left-hand weakness and wasting with no sensory deficits. She denied any involvement of her other hand or bilateral lower limbs, and she was referred to our clinic with the potential diagnosis of amyotrophic lateral sclerosis (ALS). An elaborate history, physical examination, electrophysiological studies, and imaging assisted us in reaching the diagnosis of CSA, 1 year after the onset of symptoms. S. Karger AG 2020-09-18 /pmc/articles/PMC7548943/ /pubmed/33082770 http://dx.doi.org/10.1159/000509684 Text en Copyright © 2020 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Single Case − General Neurology
Idiculla, Pretty Sara
Govindarajan, Raghav
A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis
title A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis
title_full A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis
title_fullStr A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis
title_full_unstemmed A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis
title_short A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis
title_sort case of cervical spondylotic amyotrophy mimicking amyotrophic lateral sclerosis
topic Single Case − General Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7548943/
https://www.ncbi.nlm.nih.gov/pubmed/33082770
http://dx.doi.org/10.1159/000509684
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