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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

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
Rotator drift: A sign of upper motor neuron leg weakness.
Weaver DF
Clin Neurol Neurosurg 2020 Oct;197:106084. Epub 2020 Jul 13 doi: 10.1016/j.clineuro.2020.106084. PMID: 32683196
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
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

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
Pathophysiology and Diagnosis of ALS: Insights from Advances in Neurophysiological Techniques.
van den Bos MAJ, Geevasinga N, Higashihara M, Menon P, Vucic S
Int J Mol Sci 2019 Jun 10;20(11) doi: 10.3390/ijms20112818. PMID: 31185581Free 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
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.
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

Tetramethylpyrazine nitrone improves motor dysfunction and pathological manifestations by activating the PGC-1α/Nrf2/HO-1 pathway in ALS mice.
Wen J, Li S, Zheng C, Wang F, Luo Y, Wu L, Cao J, Guo B, Yu P, Zhang G, Li S, Sun Y, Yang X, Zhang Z, Wang Y
Neuropharmacology 2021 Jan;182:108380. Epub 2020 Nov 2 doi: 10.1016/j.neuropharm.2020.108380. PMID: 33152451
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
Rehabilitation techniques to maximize spasticity management.
Logan LR
Top Stroke Rehabil 2011 May-Jun;18(3):203-11. doi: 10.1310/tsr1803-203. PMID: 21642058
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
Multifocal motor neuropathy.
Nobile-Orazio E
J Neuroimmunol 2001 Apr 2;115(1-2):4-18. doi: 10.1016/s0165-5728(01)00266-1. PMID: 11282149

Prognosis

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
Skeletal Muscle in ALS: An Unappreciated Therapeutic Opportunity?
Scaricamazza S, Salvatori I, Ferri A, Valle C
Cells 2021 Mar 2;10(3) doi: 10.3390/cells10030525. PMID: 33801336Free PMC Article
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
Perspectives of Kennedy's disease.
Finsterer J
J Neurol Sci 2010 Nov 15;298(1-2):1-10. Epub 2010 Sep 16 doi: 10.1016/j.jns.2010.08.025. PMID: 20846673
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
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
Monomelic amyotrophy.
Gourie-Devi M, Suresh TG, Shankar SK
Arch Neurol 1984 Apr;41(4):388-94. doi: 10.1001/archneur.1984.04050160050015. PMID: 6703940
Sensations of heaviness.
Gandevia SC, McCloskey DI
Brain 1977 Jun;100(2):345-54. doi: 10.1093/brain/100.2.345. PMID: 884488

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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