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  • Title: Adult-onset rod disease with abundant intranuclear rods.
    Author: Paulus W, Peiffer J, Becker I, Roggendorf W, Schumm F.
    Journal: J Neurol; 1988 Jul; 235(6):343-7. PubMed ID: 2845006.
    Abstract:
    The third case of adult-onset rod disease (nemaline myopathy) with abundant myofibrillar as well as intranuclear rods is described. The 61-year-old woman suffered from progressive weakness of proximal extremities and of the neck, mimicking polymyositis. Muscle biopsy revealed a striking myopathic pattern, with intranuclear rods occurring in 31% of the fibres. On light and electron microscopy and by immunohistochemical study, the rods differed from myofibrillar rods. The absence of alpha-actinin in intranuclear rods suggests an enhanced readiness of actin filaments to bind to diverse proteins, instead of overproduction of alpha-actinin as the pathogenetic basis of the rod formation.
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