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Lewis-Sumner syndrome

MedGen UID:
798498
Concept ID:
C1695985
Disease or Syndrome
Synonyms: MADSAM; Multifocal acquired demyelinating sensory and motor neuropathy; multifocal acquired demyelinating sensory and motor neuropathy
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018826
Orphanet: ORPHA48162

Definition

Lewis-Sumner syndrome (LSS) is a rare acquired demyelinating polyneuropathy characterized by asymmetrical distal weakness of the upper or lower extremities and motor dysfunction with adult onset. It is considered to be a variant of chronic inflammatory demyelinating polyneuropathy. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLewis-Sumner syndrome
Follow this link to review classifications for Lewis-Sumner syndrome in Orphanet.

Professional guidelines

PubMed

De Lorenzo A, Liberatore G, Doneddu PE, Manganelli F, Cocito D, Briani C, Fazio R, Mazzeo A, Schenone A, Di Stefano V, Cosentino G, Marfia GA, Benedetti L, Carpo M, Filosto M, Antonini G, Clerici AM, Luigetti M, Matà S, Rosso T, Lucchetta M, Siciliano G, Lauria Pinter G, Cavaletti G, Inghilleri M, Cantisani T, Notturno F, Ricciardi D, Habetswallner F, Spina E, Peci E, Salvalaggio A, Falzone Y, Strano C, Gentile L, Vegezzi E, Mataluni G, Cotti Piccinelli S, Leonardi L, Romano A, Nobile-Orazio E; Italian CIDP Database Study Group
Eur J Neurol 2024 Apr;31(4):e16190. Epub 2024 Jan 2 doi: 10.1111/ene.16190. PMID: 38165011
Lehmann HC, Burke D, Kuwabara S
J Neurol Neurosurg Psychiatry 2019 Sep;90(9):981-987. Epub 2019 Apr 16 doi: 10.1136/jnnp-2019-320314. PMID: 30992333
Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani C, Filosto M, Benedetti L, Mazzeo A, Marfia GA, Cortese A, Fierro B, Jann S, Beghi E, Clerici AM, Carpo M, Schenone A, Luigetti M, Lauria G, Antonini G, Rosso T, Siciliano G, Cavaletti G, Liberatore G, Santoro L, Peci E, Tronci S, Ruiz M, Cotti Piccinelli S, Toscano A, Mataluni G, Piccolo L, Cosentino G, Sabatelli M, Nobile-Orazio E; Italian CIDP Database study group
J Neurol Neurosurg Psychiatry 2019 Feb;90(2):125-132. Epub 2018 Oct 8 doi: 10.1136/jnnp-2018-318714. PMID: 30297520

Recent clinical studies

Etiology

Doneddu PE, Dentoni M, Nobile-Orazio E
Curr Opin Neurol 2021 Oct 1;34(5):613-624. doi: 10.1097/WCO.0000000000000979. PMID: 34267052Free PMC Article
Zhou XJ, Zhu Y, Zhu DS, Han L, Liu QY, Liang XN, Hao Y, Li ZZ, Guan YT
Chin Med J (Engl) 2020 Nov 5;133(21):2558-2564. doi: 10.1097/CM9.0000000000001073. PMID: 32947359Free PMC Article
Fargeot G, Maisonobe T, Psimaras D, Debs R, Lenglet T, Adams D, Vandendries C, Labeyrie C, Viala K
Eur J Neurol 2020 Mar;27(3):522-528. Epub 2019 Oct 24 doi: 10.1111/ene.14101. PMID: 31574194
Li Y, Niu J, Liu T, Guan Y, Wu S, Ding Q, Cui L, Liu M
J Clin Neurosci 2019 Sep;67:10-13. Epub 2019 Jul 1 doi: 10.1016/j.jocn.2019.06.044. PMID: 31272830
Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani C, Filosto M, Benedetti L, Mazzeo A, Marfia GA, Cortese A, Fierro B, Jann S, Beghi E, Clerici AM, Carpo M, Schenone A, Luigetti M, Lauria G, Antonini G, Rosso T, Siciliano G, Cavaletti G, Liberatore G, Santoro L, Peci E, Tronci S, Ruiz M, Cotti Piccinelli S, Toscano A, Mataluni G, Piccolo L, Cosentino G, Sabatelli M, Nobile-Orazio E; Italian CIDP Database study group
J Neurol Neurosurg Psychiatry 2019 Feb;90(2):125-132. Epub 2018 Oct 8 doi: 10.1136/jnnp-2018-318714. PMID: 30297520

Diagnosis

Doneddu PE, Dentoni M, Nobile-Orazio E
Curr Opin Neurol 2021 Oct 1;34(5):613-624. doi: 10.1097/WCO.0000000000000979. PMID: 34267052Free PMC Article
Lehmann HC, Burke D, Kuwabara S
J Neurol Neurosurg Psychiatry 2019 Sep;90(9):981-987. Epub 2019 Apr 16 doi: 10.1136/jnnp-2019-320314. PMID: 30992333
Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani C, Filosto M, Benedetti L, Mazzeo A, Marfia GA, Cortese A, Fierro B, Jann S, Beghi E, Clerici AM, Carpo M, Schenone A, Luigetti M, Lauria G, Antonini G, Rosso T, Siciliano G, Cavaletti G, Liberatore G, Santoro L, Peci E, Tronci S, Ruiz M, Cotti Piccinelli S, Toscano A, Mataluni G, Piccolo L, Cosentino G, Sabatelli M, Nobile-Orazio E; Italian CIDP Database study group
J Neurol Neurosurg Psychiatry 2019 Feb;90(2):125-132. Epub 2018 Oct 8 doi: 10.1136/jnnp-2018-318714. PMID: 30297520
Whitesell J
Semin Neurol 2010 Sep;30(4):356-64. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267279. PMID: 20941668
Rajabally YA, Chavada G
Muscle Nerve 2009 Feb;39(2):206-20. doi: 10.1002/mus.21199. PMID: 19145651

Therapy

Ribeiro L, Monteiro A, Martins J
BMC Neurol 2020 Nov 24;20(1):427. doi: 10.1186/s12883-020-02008-4. PMID: 33234123Free PMC Article
Hughes IMW, Goodridge AE
Can J Neurol Sci 2019 Mar;46(2):243-247. Epub 2019 Feb 6 doi: 10.1017/cjn.2018.389. PMID: 30724154
Sederholm BH
Semin Neurol 2010 Sep;30(4):443-56. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267289. PMID: 20941678
Finsterer J
Acta Neurol Scand 2005 Aug;112(2):115-25. doi: 10.1111/j.1600-0404.2005.00448.x. PMID: 16008538
Ensrud ER, Krivickas LS
Phys Med Rehabil Clin N Am 2001 May;12(2):321-34, ix. PMID: 11345010

Prognosis

Ribeiro L, Monteiro A, Martins J
BMC Neurol 2020 Nov 24;20(1):427. doi: 10.1186/s12883-020-02008-4. PMID: 33234123Free PMC Article
Gilchrist JM, Dandapat S
Hand (N Y) 2020 Sep;15(5):599-607. Epub 2020 Feb 5 doi: 10.1177/1558944719898801. PMID: 32020820Free PMC Article
Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani C, Filosto M, Benedetti L, Mazzeo A, Marfia GA, Cortese A, Fierro B, Jann S, Beghi E, Clerici AM, Carpo M, Schenone A, Luigetti M, Lauria G, Antonini G, Rosso T, Siciliano G, Cavaletti G, Liberatore G, Santoro L, Peci E, Tronci S, Ruiz M, Cotti Piccinelli S, Toscano A, Mataluni G, Piccolo L, Cosentino G, Sabatelli M, Nobile-Orazio E; Italian CIDP Database study group
J Neurol Neurosurg Psychiatry 2019 Feb;90(2):125-132. Epub 2018 Oct 8 doi: 10.1136/jnnp-2018-318714. PMID: 30297520
Whitesell J
Semin Neurol 2010 Sep;30(4):356-64. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267279. PMID: 20941668
Rajabally YA, Chavada G
Muscle Nerve 2009 Feb;39(2):206-20. doi: 10.1002/mus.21199. PMID: 19145651

Clinical prediction guides

Alfaro A, Picorelli S, Jiménez J, García L, Bernabéu-Sanz A
Skeletal Radiol 2023 Jun;52(6):1237-1242. Epub 2022 Nov 16 doi: 10.1007/s00256-022-04236-w. PMID: 36380244
Feng Y, Zhang Y, Su X, Zheng C, Lu Z
Sci Rep 2020 Oct 7;10(1):16697. doi: 10.1038/s41598-020-73104-1. PMID: 33028841Free PMC Article
Zhou XJ, Zhu Y, Zhu DS, Han L, Liu QY, Liang XN, Hao Y, Li ZZ, Guan YT
Chin Med J (Engl) 2020 Nov 5;133(21):2558-2564. doi: 10.1097/CM9.0000000000001073. PMID: 32947359Free PMC Article
Décard BF, Pham M, Grimm A
Clin Neurophysiol 2018 Jan;129(1):155-167. Epub 2017 Nov 10 doi: 10.1016/j.clinph.2017.10.028. PMID: 29190522
Mariotto S, Ferrari S, Monaco S
Inflamm Allergy Drug Targets 2014;13(5):299-304. doi: 10.2174/1871528113666140908113841. PMID: 25198705Free PMC Article

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