Fulminant multifocal motor neuropathy: a report of two cases

The International Journal of Neuroscience
Giuliana GalassiPatrizia Sola

Abstract

Multifocal motor neuropathy (MMN) shows stepwise progression over decades. The multifocal weakness usually remains asymmetric, confined to distal limb muscles, while sparing cranial, phrenic, and sensory nerves. One electrophysiological hallmark is partial motor conduction block (CB) at sites not exposed to compression; whether CB is an essential feature remains debatable. High titer of anti-GM1 antibodies is found with figures usually between 40% and 50% of patients. Intravenous immuneglobulin (IVIg) is effective in almost 80%, but plasmapheresis and steroids are not. The condition is reported as lethal exceptionally, mimicking motor neuron diseases (MND). We have studied two patients who failed to respond to treatment and who died with respiratory failure; one of the two had high titer of IgM antibody to the ganglioside GM1. Our cases confirm that great attention should be paid in order to define the borderland between MMN and MND and the entity of their clinical and electrophysiological overlaps.

References

Dec 12, 1997·Journal of the Neurological Sciences·G Pelliccioni, O Scarpino
Sep 20, 2001·Muscle & Nerve·S R BeydounD Commins
Aug 2, 2006·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·UNKNOWN European Federation of Neurological SocietiesP A van Doorn
Oct 5, 2007·Journal of the Neurological Sciences·Eduardo Nobile-OrazioMarinella Carpo

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Citations

Feb 15, 2017·Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion·Anil TombakEyup Naci Tiftik

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