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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment.
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
Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database.
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
Treatment of chronic immune-mediated neuropathies: chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and the Lewis-Sumner syndrome.
Sederholm BH
Semin Neurol 2010 Sep;30(4):443-56. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267289. PMID: 20941678

Recent clinical studies

Etiology

Comparison of Lewis-Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre.
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
Motor conduction block and conduction velocity in Lewis-Sumner syndrome and multifocal motor neuropathy.
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
Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database.
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
Lewis-Sumner syndrome in a patient with rheumatoid arthritis: Link between rheumatoid arthritis and demyelinating polyradiculoneuropathies.
Kedra J, Foltz V, Viala K, Tan S, Fautrel B
Joint Bone Spine 2017 Jul;84(4):485-487. Epub 2017 Mar 28 doi: 10.1016/j.jbspin.2017.02.013. PMID: 28363822
Lewis-Sumner syndrome and multifocal motor neuropathy.
Verschueren A, Azulay JP, Attarian S, Boucraut J, Pellissier JF, Pouget J
Muscle Nerve 2005 Jan;31(1):88-94. doi: 10.1002/mus.20236. PMID: 15536613

Diagnosis

Lewis-Sumner syndrome: contribution of diffusion tensor imaging in its differential diagnosis.
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
Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment.
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
Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database.
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
Inflammatory neuropathies.
Whitesell J
Semin Neurol 2010 Sep;30(4):356-64. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267279. PMID: 20941668
Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features.
Rajabally YA, Chavada G
Muscle Nerve 2009 Feb;39(2):206-20. doi: 10.1002/mus.21199. PMID: 19145651

Therapy

Lewis-Sumner syndrome in a patient with rheumatoid arthritis: Link between rheumatoid arthritis and demyelinating polyradiculoneuropathies.
Kedra J, Foltz V, Viala K, Tan S, Fautrel B
Joint Bone Spine 2017 Jul;84(4):485-487. Epub 2017 Mar 28 doi: 10.1016/j.jbspin.2017.02.013. PMID: 28363822
Treatment of chronic immune-mediated neuropathies: chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and the Lewis-Sumner syndrome.
Sederholm BH
Semin Neurol 2010 Sep;30(4):443-56. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267289. PMID: 20941678
Treatment of immune-mediated, dysimmune neuropathies.
Finsterer J
Acta Neurol Scand 2005 Aug;112(2):115-25. doi: 10.1111/j.1600-0404.2005.00448.x. PMID: 16008538
Lewis-Sumner syndrome and multifocal motor neuropathy.
Verschueren A, Azulay JP, Attarian S, Boucraut J, Pellissier JF, Pouget J
Muscle Nerve 2005 Jan;31(1):88-94. doi: 10.1002/mus.20236. PMID: 15536613
Acquired inflammatory demyelinating neuropathies.
Ensrud ER, Krivickas LS
Phys Med Rehabil Clin N Am 2001 May;12(2):321-34, ix. PMID: 11345010

Prognosis

Comparison of Lewis-Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre.
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
Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database.
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
Inflammatory neuropathies.
Whitesell J
Semin Neurol 2010 Sep;30(4):356-64. Epub 2010 Oct 12 doi: 10.1055/s-0030-1267279. PMID: 20941668
Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features.
Rajabally YA, Chavada G
Muscle Nerve 2009 Feb;39(2):206-20. doi: 10.1002/mus.21199. PMID: 19145651
Acquired inflammatory demyelinating neuropathies.
Ensrud ER, Krivickas LS
Phys Med Rehabil Clin N Am 2001 May;12(2):321-34, ix. PMID: 11345010

Clinical prediction guides

Lewis-Sumner syndrome: contribution of diffusion tensor imaging in its differential diagnosis.
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
Ultrasound and MRI of nerves for monitoring disease activity and treatment effects in chronic dysimmune neuropathies - Current concepts and future directions.
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
HCV-related central and peripheral nervous system demyelinating disorders.
Mariotto S, Ferrari S, Monaco S
Inflamm Allergy Drug Targets 2014;13(5):299-304. doi: 10.2174/1871528113666140908113841. PMID: 25198705Free PMC Article
Response to treatment in patients with Lewis-Sumner syndrome.
Attarian S, Verschueren A, Franques J, Salort-Campana E, Jouve E, Pouget J
Muscle Nerve 2011 Aug;44(2):179-84. doi: 10.1002/mus.22024. PMID: 21755505
Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome.
Viala K, Renié L, Maisonobe T, Béhin A, Neil J, Léger JM, Bouche P
Brain 2004 Sep;127(Pt 9):2010-7. Epub 2004 Aug 2 doi: 10.1093/brain/awh222. PMID: 15289267

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