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Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia

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Title: Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia
Authors: Timothy Fullam, Jeffrey Statland
Source: Brain Sciences, Vol 11, Iss 5, p 611 (2021)
Publisher Information: MDPI AG, 2021.
Publication Year: 2021
Collection: LCC:Neurosciences. Biological psychiatry. Neuropsychiatry
Subject Terms: primary lateral sclerosis, amyotrophic lateral sclerosis, hereditary spastic paraplegia, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
More Details: 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.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 2076-3425
Relation: https://www.mdpi.com/2076-3425/11/5/611; https://doaj.org/toc/2076-3425
DOI: 10.3390/brainsci11050611
Access URL: https://doaj.org/article/712c3fa82a2c42869f25733888b97527
Accession Number: edsdoj.712c3fa82a2c42869f25733888b97527
Database: Directory of Open Access Journals
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  Data: Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia
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  Data: Brain Sciences, Vol 11, Iss 5, p 611 (2021)
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  Data: 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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      – SubjectFull: hereditary spastic paraplegia
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