ISSN:
1432-1459
Keywords:
Adult-onset rod disease
;
Nemaline myopathy
;
α-Actinin
;
Immunohistochemistry
;
Electron microscopy
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary 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 α-actinin in intranuclear rods suggests an enhanced readiness of actin filaments to bind to diverse proteins, instead of overproduction of α-actinin as the pathogenetic basis of the rod formation.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00314229
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