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Christian E. Keller, MD; Arthur P. Hays, MD; Lewis P. Rowland, MD; Behzad Moghadaszadeh, PhD; Alan H. Beggs, PhD; Govind Bhagat, MD

Arch Neurol. 2006;63:132-134.

A 45-year-old man with severe proximal muscle weakness had findings diagnostic of adult-onset nemaline myopathy. He also had a monoclonal gammopathy. This is the fifth report of adult-onset nemaline myopathy in a patient with monoclonal gammopathy, suggesting that the occurrence of these entities may be more than a chance association. Myocyte-bound immunoglobulin or light chains were not detected and immunotherapy was not effective in this patient. Other causes of adult-onset nemaline myopathy were ruled out, including the most common mutations of sarcomeric thin filament genes.

Author Affiliations: Department of Pathology (Dr Keller), Division of Neuropathology (Dr Hays), Division of Hematopathology (Dr Bhagat), College of Physicians and Surgeons, Columbia University, New York, NY; Department of Neurology, Neurological Institute of New York, Columbia University Medical Center (Dr Rowland); Genomics Program and Division of Genetics, Children's Hospital Boston, Harvard Medical School, Boston, Mass (Drs Moghadaszadeh and Beggs).

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