Department of Neurology, Wolfson Medical Center, Holon and Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Case Report
Late-Onset Myopathy Responsive to Immunomodulatory Treatment
Author(s): Menachem Sadeh*, Yakov Fellig and Ron Dabby
Late-Onset Sporadic Nemaline Myopathy (SLONM) is a rare, treatable or potentially life-threatening, muscle disorder that typically manifests late in life and is characterized by the presence of nemaline rods within muscle fibers, serving as the hallmark of the disease and the key to diagnosis. We report a case of an elderly patient with sub-acute onset of severe weakness affecting the upper and lower limbs, neck extensors and abdominal muscles. Muscle biopsies showed nonspecific myopathic changes without inflammation, and electron microscopy did not reveal rods or aggregates. The laboratory workup was unremarkable except for the detection of Monoclonal Gammopathy of Undetermined Significance (MGUS). Steroid treatment was ineffective; however, there was a notable positive response to intravenous immunoglobulins. The neurological findings, subacute course, normal CK levels, presence of .. Read More»
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