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Intranuclear and cytoplasmic filamentous inclusions in distal myopathy (Welander)

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Summary

Ultrastructural examination of anterior tibial muscle from four patients with late-onset autosomal dominant distal myopathy of Welander-type revealed intrasarcoplasmic filamentous inclusions in association with rimmed vacuoles. In one of the patients, identical intranuclear filamentous inclusions were also found. These filamentous inclusions are similar to those desxribed in inclusion body myositis (IBM). They have also been observed in hereditary neuromuscular disorders including autosomal recessive distal myopathy. Thus, the filamentous inclusions occur in different neuromuscular conditions with different etiologies. These findings further raise the question of the specificity of the filamentous inclusions in IBM.

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Authors and Affiliations

  1. Department of Neurology, Karolinska Hospital, S-104 01, Stockholm, Sweden

    K. Borg & L. Edström

  2. INSERM Unité 153, Paris, France

    K. Borg & F. M. S. Tomé

Authors
  1. K. Borg
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  2. F. M. S. Tomé
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  3. L. Edström
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Additional information

Supported by grants from the Swedish Medical Research Council (proj. 3875, visiting research grant K. Borg), the Swedish MS foundation, the Swedish Society of Medicine, the Swedish Society for Traffic and Polio Disabled, Erik and Edith Fernströms Foundation for Medical Research and from the Karolinska Institute

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Borg, K., Tomé, F.M.S. & Edström, L. Intranuclear and cytoplasmic filamentous inclusions in distal myopathy (Welander). Acta Neuropathol 82, 102–106 (1991). https://doi.org/10.1007/BF00293951

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