Nemaline rod myopathy is a rare disease associated with muscle weakness and the presence of rod-shaped structures in the muscle fibers. Various acquired conditions have been associated with slow progressing presentations during adulthood. Several cases have been diagnosed in adults with HIV infection. The use of intravenous immunoglobulin (IVIG) has not been previously described for the treatment of these patients. We report the cases of 2 patients with HIV-related nemaline rod myopathy who successfully responded to IVIG therapy.

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