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Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features.
Muscle Nerve. 2009 Feb; 39(2):206-20.MN

Abstract

Lewis-Sumner syndrome (L-SS) represents the asymmetric variant of chronic inflammatory demyelinating polyneuropathy (CIDP). The characteristics and specificities of L-SS of pure upper-limb onset, as initially described by Lewis et al. [Multifocal demyelinating neuropathy with persistent conduction block. Neurology 32:958-964, 1982], have not been studied. We describe 8 such patients and review 82 previously reported cases. Distal involvement predominates and is mixed, sensory and motor from onset in only 50% of patients. Pain is a feature in about 20%. Subsequent lower-limb involvement occurs in <40% of cases. Electrophysiologically, upper-limb-onset L-SS is characterized by the presence of motor conduction blocks in arm nerves in about 90% of cases, and other demyelinating motor abnormalities are significantly less frequent. Cerebrospinal fluid (CSF) protein levels are raised in about 40% of cases and are moderate in most. Mildly raised anti-GM1 antibody titers are rare (<5%), but very high titers (> or =1:6400) have not been reported. Over 80% of treated patients respond, and intravenous immunoglobulins may be more effective than steroids. The prognosis is favorable in 40% of patients who eventually stabilize without treatment. We also reviewed 36 cases of other forms of L-SS, and present a further 2 cases. The upper-limb-onset variant is significantly less likely to spread to other limbs and may be less likely to have raised CSF protein levels. This could reflect a more localized disease process in upper-limb-onset L-SS. This variant may represent a separate entity, to be distinguished from other asymmetric forms of CIDP.

Authors+Show Affiliations

Neuromuscular Clinic, Department of Neurology, University Hospitals of Leicester, Leicester LE5 4PW, UK. yusuf.rajabally@uhl-tr.nhs.ukNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

19145651

Citation

Rajabally, Yusuf A., and Govindsinh Chavada. "Lewis-sumner Syndrome of Pure Upper-limb Onset: Diagnostic, Prognostic, and Therapeutic Features." Muscle & Nerve, vol. 39, no. 2, 2009, pp. 206-20.
Rajabally YA, Chavada G. Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features. Muscle Nerve. 2009;39(2):206-20.
Rajabally, Y. A., & Chavada, G. (2009). Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features. Muscle & Nerve, 39(2), 206-20. https://doi.org/10.1002/mus.21199
Rajabally YA, Chavada G. Lewis-sumner Syndrome of Pure Upper-limb Onset: Diagnostic, Prognostic, and Therapeutic Features. Muscle Nerve. 2009;39(2):206-20. PubMed PMID: 19145651.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features. AU - Rajabally,Yusuf A, AU - Chavada,Govindsinh, PY - 2009/1/16/entrez PY - 2009/1/16/pubmed PY - 2009/4/10/medline SP - 206 EP - 20 JF - Muscle & nerve JO - Muscle Nerve VL - 39 IS - 2 N2 - Lewis-Sumner syndrome (L-SS) represents the asymmetric variant of chronic inflammatory demyelinating polyneuropathy (CIDP). The characteristics and specificities of L-SS of pure upper-limb onset, as initially described by Lewis et al. [Multifocal demyelinating neuropathy with persistent conduction block. Neurology 32:958-964, 1982], have not been studied. We describe 8 such patients and review 82 previously reported cases. Distal involvement predominates and is mixed, sensory and motor from onset in only 50% of patients. Pain is a feature in about 20%. Subsequent lower-limb involvement occurs in <40% of cases. Electrophysiologically, upper-limb-onset L-SS is characterized by the presence of motor conduction blocks in arm nerves in about 90% of cases, and other demyelinating motor abnormalities are significantly less frequent. Cerebrospinal fluid (CSF) protein levels are raised in about 40% of cases and are moderate in most. Mildly raised anti-GM1 antibody titers are rare (<5%), but very high titers (> or =1:6400) have not been reported. Over 80% of treated patients respond, and intravenous immunoglobulins may be more effective than steroids. The prognosis is favorable in 40% of patients who eventually stabilize without treatment. We also reviewed 36 cases of other forms of L-SS, and present a further 2 cases. The upper-limb-onset variant is significantly less likely to spread to other limbs and may be less likely to have raised CSF protein levels. This could reflect a more localized disease process in upper-limb-onset L-SS. This variant may represent a separate entity, to be distinguished from other asymmetric forms of CIDP. SN - 0148-639X UR - https://www.unboundmedicine.com/medline/citation/19145651/Lewis_sumner_syndrome_of_pure_upper_limb_onset:_diagnostic_prognostic_and_therapeutic_features_ L2 - https://doi.org/10.1002/mus.21199 DB - PRIME DP - Unbound Medicine ER -