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Weakness due to upper motor neuron dysfunction

MedGen UID:
866899
•Concept ID:
C4021255
•
Pathologic Function
Synonym: Paralysis due to lesions of the principle motor tracts
 
HPO: HP:0010549

Definition

Paralysis of voluntary muscles means loss of contraction due to interruption of one or more motor pathways from the brain to the muscle fibers. Although the word paralysis is often used interchangeably to mean either complete or partial loss of muscle strength, it is preferable to use paralysis or plegia for complete or severe loss of muscle strength, and paresis for partial or slight loss. Paralysis due to lesions of the principle motor tracts is related to a lesion in the corticospinal, corticobulbar or brainstem descending (subcorticospinal) neurons. [from HPO]

Professional guidelines

PubMed

Acute non-traumatic tetraparesis - Differential diagnosis.
Oliveira R, Ramalho Rocha F, Teodoro T, Oliveira Santos M
J Clin Neurosci 2021 May;87:116-124. Epub 2021 Mar 20 doi: 10.1016/j.jocn.2021.02.024. PMID: 33863518
Emerging understanding of the genotype-phenotype relationship in amyotrophic lateral sclerosis.
Goutman SA, Chen KS, Paez-Colasante X, Feldman EL
Handb Clin Neurol 2018;148:603-623. doi: 10.1016/B978-0-444-64076-5.00039-9. PMID: 29478603
Diagnostic investigation and multidisciplinary management in motor neuron disease.
Rocha JA, Reis C, Simões F, Fonseca J, Mendes Ribeiro J
J Neurol 2005 Dec;252(12):1435-47. doi: 10.1007/s00415-005-0007-9. PMID: 16362828

Recent clinical studies

Etiology

Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
The unfolding spectrum of inherited distal myopathies.
Milone M, Liewluck T
Muscle Nerve 2019 Mar;59(3):283-294. Epub 2018 Nov 28 doi: 10.1002/mus.26332. PMID: 30171629
Amyotrophic Lateral Sclerosis: An Update for 2018.
Oskarsson B, Gendron TF, Staff NP
Mayo Clin Proc 2018 Nov;93(11):1617-1628. Epub 2018 Jul 4 doi: 10.1016/j.mayocp.2018.04.007. PMID: 30401437
Cervicobrachial polymyositis.
Khadilkar SV, Gupta N, Yadav RS
J Clin Neuromuscul Dis 2014 Dec;16(2):59-68. doi: 10.1097/CND.0000000000000059. PMID: 25415516
Idiopathic brachial neuritis.
Sumner AJ
Neurosurgery 2009 Oct;65(4 Suppl):A150-2. doi: 10.1227/01.NEU.0000345355.59438.D1. PMID: 19927060

Diagnosis

Amyotrophic lateral sclerosis: a clinical review.
Masrori P, Van Damme P
Eur J Neurol 2020 Oct;27(10):1918-1929. Epub 2020 Jul 7 doi: 10.1111/ene.14393. PMID: 32526057Free PMC Article
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Int J Mol Sci 2019 Jun 10;20(11) doi: 10.3390/ijms20112818. PMID: 31185581Free PMC Article
Respiratory Failure in Amyotrophic Lateral Sclerosis.
Niedermeyer S, Murn M, Choi PJ
Chest 2019 Feb;155(2):401-408. Epub 2018 Jul 7 doi: 10.1016/j.chest.2018.06.035. PMID: 29990478
Amyotrophic Lateral Sclerosis: An Update for 2018.
Oskarsson B, Gendron TF, Staff NP
Mayo Clin Proc 2018 Nov;93(11):1617-1628. Epub 2018 Jul 4 doi: 10.1016/j.mayocp.2018.04.007. PMID: 30401437
Amyotrophic lateral sclerosis.
Hardiman O, Al-Chalabi A, Chio A, Corr EM, Logroscino G, Robberecht W, Shaw PJ, Simmons Z, van den Berg LH
Nat Rev Dis Primers 2017 Oct 5;3:17071. doi: 10.1038/nrdp.2017.71. PMID: 28980624

Therapy

Amyotrophic Lateral Sclerosis: An Update for 2018.
Oskarsson B, Gendron TF, Staff NP
Mayo Clin Proc 2018 Nov;93(11):1617-1628. Epub 2018 Jul 4 doi: 10.1016/j.mayocp.2018.04.007. PMID: 30401437
Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis.
Rutkove SB
Neurotherapeutics 2015 Apr;12(2):384-93. doi: 10.1007/s13311-014-0331-9. PMID: 25582382Free PMC Article
Cassava food toxins, konzo disease, and neurodegeneration in sub-Sahara Africans.
Tshala-Katumbay D, Mumba N, Okitundu L, Kazadi K, Banea M, Tylleskär T, Boivin M, Muyembe-Tamfum JJ
Neurology 2013 Mar 5;80(10):949-51. doi: 10.1212/WNL.0b013e3182840b81. PMID: 23460617Free PMC Article
Multifocal motor neuropathy: the diagnostic spectrum and response to treatment.
Slee M, Selvan A, Donaghy M
Neurology 2007 Oct 23;69(17):1680-7. doi: 10.1212/01.wnl.0000277697.55288.d0. PMID: 17954783
Botulinum treatment of spasticity: why is it so difficult to show a functional benefit?
Sheean GL
Curr Opin Neurol 2001 Dec;14(6):771-6. doi: 10.1097/00019052-200112000-00015. PMID: 11723387

Prognosis

The Role of Human Pluripotent Stem Cells in Amyotrophic Lateral Sclerosis: From Biological Mechanism to Practical Implications.
Ceccarelli L, Verriello L, Pauletto G, Valente M, Spadea L, Salati C, Zeppieri M, Ius T
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Facial onset sensory and motor neuronopathy.
Zheng Q, Chu L, Tan L, Zhang H
Neurol Sci 2016 Dec;37(12):1905-1909. Epub 2016 Jul 29 doi: 10.1007/s10072-016-2686-7. PMID: 27473302
Cassava food toxins, konzo disease, and neurodegeneration in sub-Sahara Africans.
Tshala-Katumbay D, Mumba N, Okitundu L, Kazadi K, Banea M, Tylleskär T, Boivin M, Muyembe-Tamfum JJ
Neurology 2013 Mar 5;80(10):949-51. doi: 10.1212/WNL.0b013e3182840b81. PMID: 23460617Free PMC Article
Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study.
Chiò A, Calvo A, Moglia C, Mazzini L, Mora G; PARALS study group
J Neurol Neurosurg Psychiatry 2011 Jul;82(7):740-6. Epub 2011 Mar 14 doi: 10.1136/jnnp.2010.235952. PMID: 21402743
Brown-Vialetto-Van Laere syndrome.
Sathasivam S
Orphanet J Rare Dis 2008 Apr 17;3:9. doi: 10.1186/1750-1172-3-9. PMID: 18416855Free PMC Article

Clinical prediction guides

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.
Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244
Gamma Loop Dysfunction as a Possible Neurophysiological Mechanism of Arthrogenic Muscle Inhibition: A Narrative Review of the Literature.
Konishi Y, Yoshii R, Ingersoll CD
J Sport Rehabil 2022 Aug 1;31(6):736-741. Epub 2022 Jan 25 doi: 10.1123/jsr.2021-0232. PMID: 35078149
Imbalanced Corticospinal and Reticulospinal Contributions to Spasticity in Humans with Spinal Cord Injury.
Sangari S, Perez MA
J Neurosci 2019 Oct 2;39(40):7872-7881. Epub 2019 Aug 14 doi: 10.1523/JNEUROSCI.1106-19.2019. PMID: 31413076Free PMC Article
Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
The evaluation of "spasticity".
Ashby P, Mailis A, Hunter J
Can J Neurol Sci 1987 Aug;14(3 Suppl):497-500. doi: 10.1017/s0317167100037987. PMID: 3315151

Recent systematic reviews

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.
Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244

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