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Lewis-Sumner syndrome and multifocal motor neuropathy.
Muscle Nerve. 2005 Jan; 31(1):88-94.MN

Abstract

We compared the clinical, electrophysiological, laboratory, and pathological features of 13 patients with Lewis-Sumner syndrome (LSS) with those of 20 patients with multifocal motor neuropathy (MMN). LSS and MMN patients have several common clinical features: age at onset, weakness in the distribution of individual peripheral nerves, mild wasting, cramps and fasciculations, partial areflexia, and frequent stepwise disease course. Cerebrospinal fluid protein level was normal or slightly elevated, but always less than 100 mg/dl. Conduction blocks are the electrophysiological hallmarks of these two neuropathies, and no differences in distribution and number of blocks were found. Contrary to MMN, lower-limb involvement at onset was frequent in LSS but extension to the upper limbs was a frequent later feature of the disease. Cranial nerve involvement was noted in 4 LSS patients during relapses and absent in all MMN patients. The major distinguishing features were the clinical and electrophysiological sensory involvement in LSS, and the lack of anti-GM1 antibodies in LSS, whereas IgM anti-GM1 were found in 40% of MMN patients. Some LSS patients responded to steroid therapy, whereas this was ineffective in MMN. From these features, LSS can be considered an entity distinct from MMN, with its own clinical, laboratory, and electrophysiological characteristics, and as an intermediate link between chronic inflammatory demyelinating polyneuropathy and MMN.

Authors+Show Affiliations

Department of Neurology and Neuromuscular Diseases, Timone Hospital, Boulevard Jean Moulin, 13005 Marseille, France. annie.verschueren@ap-hm.fr <annie.verschueren@ap-hm.fr>No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

15536613

Citation

Verschueren, Annie, et al. "Lewis-Sumner Syndrome and Multifocal Motor Neuropathy." Muscle & Nerve, vol. 31, no. 1, 2005, pp. 88-94.
Verschueren A, Azulay JP, Attarian S, et al. Lewis-Sumner syndrome and multifocal motor neuropathy. Muscle Nerve. 2005;31(1):88-94.
Verschueren, A., Azulay, J. P., Attarian, S., Boucraut, J., Pellissier, J. F., & Pouget, J. (2005). Lewis-Sumner syndrome and multifocal motor neuropathy. Muscle & Nerve, 31(1), 88-94.
Verschueren A, et al. Lewis-Sumner Syndrome and Multifocal Motor Neuropathy. Muscle Nerve. 2005;31(1):88-94. PubMed PMID: 15536613.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Lewis-Sumner syndrome and multifocal motor neuropathy. AU - Verschueren,Annie, AU - Azulay,Jean Philippe, AU - Attarian,Shahram, AU - Boucraut,José, AU - Pellissier,Jean François, AU - Pouget,Jean, PY - 2004/11/13/pubmed PY - 2005/2/3/medline PY - 2004/11/13/entrez SP - 88 EP - 94 JF - Muscle & nerve JO - Muscle Nerve VL - 31 IS - 1 N2 - We compared the clinical, electrophysiological, laboratory, and pathological features of 13 patients with Lewis-Sumner syndrome (LSS) with those of 20 patients with multifocal motor neuropathy (MMN). LSS and MMN patients have several common clinical features: age at onset, weakness in the distribution of individual peripheral nerves, mild wasting, cramps and fasciculations, partial areflexia, and frequent stepwise disease course. Cerebrospinal fluid protein level was normal or slightly elevated, but always less than 100 mg/dl. Conduction blocks are the electrophysiological hallmarks of these two neuropathies, and no differences in distribution and number of blocks were found. Contrary to MMN, lower-limb involvement at onset was frequent in LSS but extension to the upper limbs was a frequent later feature of the disease. Cranial nerve involvement was noted in 4 LSS patients during relapses and absent in all MMN patients. The major distinguishing features were the clinical and electrophysiological sensory involvement in LSS, and the lack of anti-GM1 antibodies in LSS, whereas IgM anti-GM1 were found in 40% of MMN patients. Some LSS patients responded to steroid therapy, whereas this was ineffective in MMN. From these features, LSS can be considered an entity distinct from MMN, with its own clinical, laboratory, and electrophysiological characteristics, and as an intermediate link between chronic inflammatory demyelinating polyneuropathy and MMN. SN - 0148-639X UR - https://www.unboundmedicine.com/medline/citation/15536613/Lewis_Sumner_syndrome_and_multifocal_motor_neuropathy_ L2 - https://doi.org/10.1002/mus.20236 DB - PRIME DP - Unbound Medicine ER -