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Sporadic Inclusion-Body Myositis and Hereditary Inclusion-Body Myopathies
Diseases of Oxidative Stress and Aging?
Valerie Askanas, MD, PhD;
W. King Engel, MD
Arch Neurol. 1998;55:915-920.
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INTRODUCTION
Sporadic inclusion-body myositis and hereditary inclusion-body myopathies are progressive and highly debilitating muscle diseases. The most characteristic morphologic feature of sporadic inclusion-body myositis and hereditary inclusion-body myopathies is vacuolar degeneration of muscle fibers, accompanied by intrafiber clusters ("tangles") of paired-helical filaments and by accumulation of several proteins that are characteristic of a brain of patients with Alzheimer disease. In neither the hereditary inclusion-body myopathies nor sporadic inclusion-body myositis are the sequential steps of the pathogenic cascade understood. The several forms of hereditary inclusion-body myopathies have different genetic transmissions and probably different genetic defects. Because the sporadic inclusion-body myositis and hereditary inclusion-body myopathies have several characteristic pathologic features in common, we postulate that their different causes trigger the same upstream aberration leading to a similar downstream cascade of pathologic events, which are ultimately responsible for the characteristic muscle-fiber degeneration. We propose that important . . . [Full Text of this Article]
CHARACTERISTIC FEATURES OF IBM MUSCLE BIOPSY SPECIMENS
HEREDITARY IBM
PUTATIVE PATHOGENIC MECHANISM(S) OF SPORADIC AND HEREDITARY IBM
OXIDATIVE STRESS AS A POSSIBLE FACTOR IN THE PATHOGENIC CASCADE OF SPORADIC AND HEREDITARY IBM
MUSCLE AGING AS A POSSIBLE CONTRIBUTORY FACTOR IN THE DEVELOPMENT OF SPORADIC AND HEREDITARY IBM
POSSIBLE RELEVANCE TO AD PATHOGENESIS
PUTATIVE "JUNCTIONALIZATION" OF NONJUNCTIONAL REGIONS OF MUSCLE FIBERS IN IBM
CONCLUSIONS
From the Neuromuscular Center, Department of Neurology, University of Southern California School of Medicine, Good Samaritan Hospital, Los Angeles.
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