Journal
MUSCLE & NERVE
Volume 41, Issue 2, Pages 272-276Publisher
WILEY
DOI: 10.1002/mus.21504
Keywords
nemaline myopathy; IVIg; monoclonal gammopathy
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Sporadic late onset nemaline myopathy (SLONM) is a progressive myopathy of indeterminate etiology and poor outcome. If associated with a monoclonal gammopathy, SLONM carries a more unfavorable prognosis. Immunotherapy was unsuccessful. We report two HIV-negative SLONM/monoclonal gammopathy patients who improved following intravenous immunoglobulin (IVIg) treatment alone or in combination with immunosuppressant agents. This favorable response to treatment suggests that a dysimmune mechanism is operative in some SLONM individuals. We suggest that IVIg deserves initial consideration for SLONM therapy. Muscle Nerve 41: 272-276, 2010
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