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[Multifocal-motor neuropathy and motor neuropathy with multifocal conduction block (Lewis-Sumner syndrome)].
Wien Klin Wochenschr. 1995; 107(10):301-8.WK

Abstract

Multifocal motor neuropathy, which mimics lower motor neuron disease, is a rare and curious demyelinating neuropathy characterised by slowly progressive, asymmetric limb weakness within the distribution of individual peripheral nerves, wasting, cramps, fasciculations and rare sensory involvement, but without upper motor neuron signs. The cardinal feature and primary pathophysiological basis for the weakness is the multifocal motor conduction block which remains stable for years at the same site and is confined to motor axons. It is defined as > 50% reduction in both the CMAP and the negative peak area on proximal stimulation, as compared with the distal stimulus response without any change in the negative peak duration. Nerves at the site of the conduction block show demyelination, endoneural edema, rudimentary onion bulbs and lymphocytic inflammation. Sensory nerves may show mild demyelination, axon loss and lymphocytic inflammation. The majority of patients shows elevated titers of anti-glycolipid antibodies, which may block the Na+ channels, produce demyelination or interfere with remyelination. However, their role in the pathogenesis of multifocal motor neuropathy remains uncertain. Multifocal motor neuropathy is regarded as the predominantly motor variant of chronic inflammatory demyelinating polyneuropathy and can be treated best with immunoglobulins and cyclophosphamide.

Authors+Show Affiliations

Neurologische Abteilung, Neurologisches Krankenhaus Rosenhügel, Wien.No affiliation info available

Pub Type(s)

English Abstract
Journal Article
Review

Language

ger

PubMed ID

7785276

Citation

Finsterer, J, and B Mamoli. "[Multifocal-motor Neuropathy and Motor Neuropathy With Multifocal Conduction Block (Lewis-Sumner Syndrome)]." Wiener Klinische Wochenschrift, vol. 107, no. 10, 1995, pp. 301-8.
Finsterer J, Mamoli B. [Multifocal-motor neuropathy and motor neuropathy with multifocal conduction block (Lewis-Sumner syndrome)]. Wien Klin Wochenschr. 1995;107(10):301-8.
Finsterer, J., & Mamoli, B. (1995). [Multifocal-motor neuropathy and motor neuropathy with multifocal conduction block (Lewis-Sumner syndrome)]. Wiener Klinische Wochenschrift, 107(10), 301-8.
Finsterer J, Mamoli B. [Multifocal-motor Neuropathy and Motor Neuropathy With Multifocal Conduction Block (Lewis-Sumner Syndrome)]. Wien Klin Wochenschr. 1995;107(10):301-8. PubMed PMID: 7785276.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Multifocal-motor neuropathy and motor neuropathy with multifocal conduction block (Lewis-Sumner syndrome)]. AU - Finsterer,J, AU - Mamoli,B, PY - 1995/1/1/pubmed PY - 1995/1/1/medline PY - 1995/1/1/entrez SP - 301 EP - 8 JF - Wiener klinische Wochenschrift JO - Wien Klin Wochenschr VL - 107 IS - 10 N2 - Multifocal motor neuropathy, which mimics lower motor neuron disease, is a rare and curious demyelinating neuropathy characterised by slowly progressive, asymmetric limb weakness within the distribution of individual peripheral nerves, wasting, cramps, fasciculations and rare sensory involvement, but without upper motor neuron signs. The cardinal feature and primary pathophysiological basis for the weakness is the multifocal motor conduction block which remains stable for years at the same site and is confined to motor axons. It is defined as > 50% reduction in both the CMAP and the negative peak area on proximal stimulation, as compared with the distal stimulus response without any change in the negative peak duration. Nerves at the site of the conduction block show demyelination, endoneural edema, rudimentary onion bulbs and lymphocytic inflammation. Sensory nerves may show mild demyelination, axon loss and lymphocytic inflammation. The majority of patients shows elevated titers of anti-glycolipid antibodies, which may block the Na+ channels, produce demyelination or interfere with remyelination. However, their role in the pathogenesis of multifocal motor neuropathy remains uncertain. Multifocal motor neuropathy is regarded as the predominantly motor variant of chronic inflammatory demyelinating polyneuropathy and can be treated best with immunoglobulins and cyclophosphamide. SN - 0043-5325 UR - https://www.unboundmedicine.com/medline/citation/7785276/[Multifocal_motor_neuropathy_and_motor_neuropathy_with_multifocal_conduction_block__Lewis_Sumner_syndrome_]_ L2 - http://www.diseaseinfosearch.org/result/4890 DB - PRIME DP - Unbound Medicine ER -