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Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy
Author(s) -
Milone Margherita,
Katz Amiram,
Amato Anthony A.,
Soderland Carl A.,
Segarceanu Miruna,
Young Nathan P.,
Jones H. Royden
Publication year - 2010
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21504
Subject(s) - nemaline myopathy , medicine , immunotherapy , myopathy , etiology , gammopathy , monoclonal gammopathy , monoclonal , immunology , pathology , antibody , monoclonal antibody , immune system
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, 2010