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Upper motor neuron signs

MedGen UID:
148190
•Concept ID:
C0749870
•
Sign or Symptom

Professional guidelines

PubMed

AWAJI criteria are not always superior to the previous criteria: A meta-analysis.
Jang JS, Bae JS
Muscle Nerve 2015 Jun;51(6):822-9. Epub 2015 Mar 31 doi: 10.1002/mus.24575. PMID: 25597926
Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders.
Overell JR, Hsieh ST, Odaka M, Yuki N, Willison HJ
Cochrane Database Syst Rev 2007 Jan 24;2007(1):CD004761. doi: 10.1002/14651858.CD004761.pub2. PMID: 17253522Free PMC Article
Closed spinal dysraphism: a review on diagnosis and treatment in infancy.
Cornette L, Verpoorten C, Lagae L, Plets C, Van Calenbergh F, Casaer P
Eur J Paediatr Neurol 1998;2(4):179-85. doi: 10.1016/s1090-3798(98)80017-2. PMID: 10726589

Recent clinical studies

Etiology

Upper motor neuron signs in primary lateral sclerosis and hereditary spastic paraplegia.
Santos Silva C, Correia Rodrigues C, Fortuna Baptista M, Oliveira Santos M, Gromicho M, Carvalho V, Correia Guedes L, de Carvalho M
Muscle Nerve 2024 Jul;70(1):152-156. Epub 2024 Apr 30 doi: 10.1002/mus.28100. PMID: 38687249
Neurological disease in xeroderma pigmentosum: prospective cohort study of its features and progression.
Garcia-Moreno H, Langbehn DR, Abiona A, Garrood I, Fleszar Z, Manes MA, Morley AMS, Craythorne E, Mohammed S, Henshaw T, Turner S, Naik H, Bodi I, Sarkany RPE, Fassihi H, Lehmann AR, Giunti P
Brain 2023 Dec 1;146(12):5044-5059. doi: 10.1093/brain/awad266. PMID: 38040034Free PMC Article
The flail-arm syndrome: the influence of phenotypic features.
Gromicho M, Santos MO, Pinto S, Swash M, Carvalho M
Amyotroph Lateral Scler Frontotemporal Degener 2023 Aug;24(5-6):383-388. Epub 2023 Jan 4 doi: 10.1080/21678421.2022.2164205. PMID: 36600571
Fiber-specific white matter analysis reflects upper motor neuron impairment in amyotrophic lateral sclerosis.
Ogura A, Kawabata K, Watanabe H, Choy SW, Bagarinao E, Kato T, Imai K, Masuda M, Ohdake R, Hara K, Nakamura R, Atsuta N, Nakamura T, Katsuno M, Sobue G
Eur J Neurol 2022 Feb;29(2):432-440. Epub 2021 Oct 31 doi: 10.1111/ene.15136. PMID: 34632672
Practical approach to the diagnosis of adult-onset leukodystrophies: an updated guide in the genomic era.
Lynch DS, Wade C, Paiva ARB, John N, Kinsella JA, Merwick Á, Ahmed RM, Warren JD, Mummery CJ, Schott JM, Fox NC, Houlden H, Adams ME, Davagnanam I, Murphy E, Chataway J
J Neurol Neurosurg Psychiatry 2019 May;90(5):543-554. Epub 2018 Nov 22 doi: 10.1136/jnnp-2018-319481. PMID: 30467211Free PMC Article

Diagnosis

Degenerative Cervical Myelopathy: A Concept Review and Clinical Approach.
Gallagher DO, Taghlabi KM, Bondar K, Saifi C
Clin Spine Surg 2024 Feb 1;37(1):1-8. Epub 2023 Jun 19 doi: 10.1097/BSD.0000000000001463. PMID: 38285428
Practical approach to the diagnosis of adult-onset leukodystrophies: an updated guide in the genomic era.
Lynch DS, Wade C, Paiva ARB, John N, Kinsella JA, Merwick Á, Ahmed RM, Warren JD, Mummery CJ, Schott JM, Fox NC, Houlden H, Adams ME, Davagnanam I, Murphy E, Chataway J
J Neurol Neurosurg Psychiatry 2019 May;90(5):543-554. Epub 2018 Nov 22 doi: 10.1136/jnnp-2018-319481. PMID: 30467211Free PMC Article
Progressive Muscular Atrophy.
Liewluck T, Saperstein DS
Neurol Clin 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005. PMID: 26515620
Giant axonal neuropathy.
Hentati F, Hentati E, Amouri R
Handb Clin Neurol 2013;115:933-8. doi: 10.1016/B978-0-444-52902-2.00052-7. PMID: 23931822
Mitochondriopathies.
Finsterer J
Eur J Neurol 2004 Mar;11(3):163-86. doi: 10.1046/j.1351-5101.2003.00728.x. PMID: 15009163

Therapy

Adolescent/Adult-Onset Leukodystrophy with MTHFR Deficiency - A Treatable Cause.
Kumar NH, Mounika KJ, Sundarachary NV
Neurol India 2023 Mar-Apr;71(2):326-328. doi: 10.4103/0028-3886.375409. PMID: 37148062
Progressive Muscular Atrophy.
Liewluck T, Saperstein DS
Neurol Clin 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005. PMID: 26515620
Is IVIg therapy warranted in progressive lower motor neuron syndromes without conduction block?
Simon NG, Ayer G, Lomen-Hoerth C
Neurology 2013 Dec 10;81(24):2116-20. Epub 2013 Nov 8 doi: 10.1212/01.wnl.0000437301.28441.7e. PMID: 24212395Free PMC Article
Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS.
Gordon PH, Cheng B, Katz IB, Mitsumoto H, Rowland LP
Neurology 2009 Jun 2;72(22):1948-52. doi: 10.1212/WNL.0b013e3181a8269b. PMID: 19487653
Chronic motor neuropathies: diagnosis, therapy, and pathogenesis.
Kornberg AJ, Pestronk A
Ann Neurol 1995 May;37 Suppl 1:S43-50. doi: 10.1002/ana.410370706. PMID: 8968216

Prognosis

Degenerative Cervical Myelopathy: A Concept Review and Clinical Approach.
Gallagher DO, Taghlabi KM, Bondar K, Saifi C
Clin Spine Surg 2024 Feb 1;37(1):1-8. Epub 2023 Jun 19 doi: 10.1097/BSD.0000000000001463. PMID: 38285428
The flail-arm syndrome: the influence of phenotypic features.
Gromicho M, Santos MO, Pinto S, Swash M, Carvalho M
Amyotroph Lateral Scler Frontotemporal Degener 2023 Aug;24(5-6):383-388. Epub 2023 Jan 4 doi: 10.1080/21678421.2022.2164205. PMID: 36600571
Clinical and electrophysiological findings in C9ORF72 ALS.
Sista SRS, Shelly S, Oskarsson B, Rubin DI, Martinez-Thompson JM, Parra-Cantu C, Staff NP, Laughlin RS
Muscle Nerve 2022 Sep;66(3):270-275. Epub 2022 Jul 11 doi: 10.1002/mus.27665. PMID: 35727129
Giant axonal neuropathy.
Hentati F, Hentati E, Amouri R
Handb Clin Neurol 2013;115:933-8. doi: 10.1016/B978-0-444-52902-2.00052-7. PMID: 23931822
Mitochondriopathies.
Finsterer J
Eur J Neurol 2004 Mar;11(3):163-86. doi: 10.1046/j.1351-5101.2003.00728.x. PMID: 15009163

Clinical prediction guides

Upper motor neuron signs in primary lateral sclerosis and hereditary spastic paraplegia.
Santos Silva C, Correia Rodrigues C, Fortuna Baptista M, Oliveira Santos M, Gromicho M, Carvalho V, Correia Guedes L, de Carvalho M
Muscle Nerve 2024 Jul;70(1):152-156. Epub 2024 Apr 30 doi: 10.1002/mus.28100. PMID: 38687249
Degenerative Cervical Myelopathy: A Concept Review and Clinical Approach.
Gallagher DO, Taghlabi KM, Bondar K, Saifi C
Clin Spine Surg 2024 Feb 1;37(1):1-8. Epub 2023 Jun 19 doi: 10.1097/BSD.0000000000001463. PMID: 38285428
The flail-arm syndrome: the influence of phenotypic features.
Gromicho M, Santos MO, Pinto S, Swash M, Carvalho M
Amyotroph Lateral Scler Frontotemporal Degener 2023 Aug;24(5-6):383-388. Epub 2023 Jan 4 doi: 10.1080/21678421.2022.2164205. PMID: 36600571
Clinical, Genetic, and Disability Profile of Pediatric Distal Hereditary Motor Neuropathy.
Argente-Escrig H, Burns J, Donlevy G, Frasquet M, Cornett K, Sevilla T, Menezes MP
Neurology 2021 Jan 19;96(3):e423-e432. Epub 2020 Oct 16 doi: 10.1212/WNL.0000000000011054. PMID: 33067402
Frontal lobe ataxia.
Thompson PD
Handb Clin Neurol 2012;103:619-22. doi: 10.1016/B978-0-444-51892-7.00044-9. PMID: 21827922

Recent systematic reviews

Atypical parkinsonism in C9orf72 expansions: a case report and systematic review of 45 cases from the literature.
Wilke C, Pomper JK, Biskup S, Puskás C, Berg D, Synofzik M
J Neurol 2016 Mar;263(3):558-74. Epub 2016 Jan 25 doi: 10.1007/s00415-016-8021-7. PMID: 26810719
Treatment for Fisher syndrome, Bickerstaff's brainstem encephalitis and related disorders.
Overell JR, Hsieh ST, Odaka M, Yuki N, Willison HJ
Cochrane Database Syst Rev 2007 Jan 24;2007(1):CD004761. doi: 10.1002/14651858.CD004761.pub2. PMID: 17253522Free PMC Article

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