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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Main Authors: Timothy Fullam, Jeffrey Statland
Format: Article
Language:English
Published: MDPI AG 2021-05-01
Series:Brain Sciences
Subjects:
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.
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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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