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A Patient with Intravenous Immunoglobulin-Responsive Lower Motor Neuron Syndrome

Author(s): Takaomi Kessoku | Takashi Koide | Katsunori Akiyama | Sachiko Irie | Tadayuki Ishihara | Fumihito Yoshii

Journal: International Journal of Clinical Medicine
ISSN 2158-284X

Volume: 03;
Issue: 03;
Start page: 190;
Date: 2012;
Original page

Keywords: Lower Motor Neuron Syndrome (LMNS) | Multifocal Motor Neuropathy (MMN) | Localized Proximal Muscle Weakness | Intravenous Immunoglobulin (IVIg) | Anti-GM1 Ganglioside Antibody

We report a 50-year-old woman who developed localized proximal muscle weakness, in addition to transient elevation of antibodies to GM-1 ganglioside, without multifocal conduction block. She was treated with intravenous immunoglobulin (IVIg) and steroid pulse therapy, which were effective for over 10 years. Her clinical course and laboratory tests were consistent with lower motor neuron syndrome (LMNS) with localized proximal muscle weakness. We suggest that some patients diagnosed as LMNS may remain responsive to IVIg or steroid pulse therapy for a long time.
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