Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy
- 22 October 2009
- journal article
- case report
- Published by Wiley in Muscle & Nerve
- Vol. 41 (2), 272-276
- https://doi.org/10.1002/mus.21504
Abstract
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, 2010Keywords
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