Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia
Following the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). Differentiation of thes...
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Format: | Article |
Language: | English |
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MDPI AG
2021-05-01
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Series: | Brain Sciences |
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Online Access: | https://www.mdpi.com/2076-3425/11/5/611 |
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author | Timothy Fullam Jeffrey Statland |
author_facet | Timothy Fullam Jeffrey Statland |
author_sort | Timothy Fullam |
collection | DOAJ |
description | Following the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). Differentiation of these disorders in the early phases of disease remains challenging. While no single clinical or diagnostic tests is specific, there are several developing biomarkers and neuroimaging technologies which may help distinguish PLS from HSP and UMNdALS. Recent consensus diagnostic criteria and use of evolving technologies will allow more precise delineation of PLS from other upper motor neuron disorders and aid in the targeting of potentially disease-modifying therapeutics. |
first_indexed | 2024-03-10T11:33:42Z |
format | Article |
id | doaj.art-712c3fa82a2c42869f25733888b97527 |
institution | Directory Open Access Journal |
issn | 2076-3425 |
language | English |
last_indexed | 2024-03-10T11:33:42Z |
publishDate | 2021-05-01 |
publisher | MDPI AG |
record_format | Article |
series | Brain Sciences |
spelling | doaj.art-712c3fa82a2c42869f25733888b975272023-11-21T19:05:07ZengMDPI AGBrain Sciences2076-34252021-05-0111561110.3390/brainsci11050611Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic ParaplegiaTimothy Fullam0Jeffrey Statland1Department of Neurology, University of Kansas Medical Center, Kansas, KS 66160, USADepartment of Neurology, University of Kansas Medical Center, Kansas, KS 66160, USAFollowing the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). Differentiation of these disorders in the early phases of disease remains challenging. While no single clinical or diagnostic tests is specific, there are several developing biomarkers and neuroimaging technologies which may help distinguish PLS from HSP and UMNdALS. Recent consensus diagnostic criteria and use of evolving technologies will allow more precise delineation of PLS from other upper motor neuron disorders and aid in the targeting of potentially disease-modifying therapeutics.https://www.mdpi.com/2076-3425/11/5/611primary lateral sclerosisamyotrophic lateral sclerosishereditary spastic paraplegia |
spellingShingle | Timothy Fullam Jeffrey Statland Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia Brain Sciences primary lateral sclerosis amyotrophic lateral sclerosis hereditary spastic paraplegia |
title | Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia |
title_full | Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia |
title_fullStr | Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia |
title_full_unstemmed | Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia |
title_short | Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia |
title_sort | upper motor neuron disorders primary lateral sclerosis upper motor neuron dominant amyotrophic lateral sclerosis and hereditary spastic paraplegia |
topic | primary lateral sclerosis amyotrophic lateral sclerosis hereditary spastic paraplegia |
url | https://www.mdpi.com/2076-3425/11/5/611 |
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