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Is the sensory neuropathy in ataxia-telangiectasia distinguishable from that in Friedreich's ataxia? - Acta Neuropathologica

  • ️Campanella, G.
  • ️Mon Sep 01 1986

Summary

The bioptical morphometric and ultrastructural study of sural nerve in a 17-year-old boy with ataxia-telangiectasia (AT) is reported. Our findings include a loss of fibers, particularly of large ones, axonal degenerative changes, Schwann cell inclusions of various type, and rare signs of primary demyelination. Teased-fiber study showed paranodal myelin enlargements, segmental demyelination, shortening, and/or variability of internodal length. This picture is similar to that in Friedreich's ataxia (FA), although they differ in degree and time of onset. A correct neuropathologic diagnosis of AT cannot be made on the basis of sural nerve biopsy alone.

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Authors and Affiliations

  1. Clinica Neurologica, II Facoltà di Medicina e Chirurgia, Università di Napoli, Via S. Pansini 5, I-80131, Napoli, Italy

    F. Barbieri & G. Campanella

  2. Cattedra di Neurofisiopatologia, II Facoltà di Medicina e Chirurgia, Università di Napoli, Via S. Pansini 5, I-80131, Napoli, Italy

    L. Santoro, C. Crisci & R. Massini

  3. Istituto di Strutture Biologiche ed Ultrastruttura Cellulare, II Facoltà di Medicina e Chirurgia, Università di Napoli, Via S. Pansini 5, I-80131, Napoli, Italy

    E. Russo

Authors

  1. F. Barbieri

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  2. L. Santoro

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  3. C. Crisci

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  4. R. Massini

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  5. E. Russo

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  6. G. Campanella

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Barbieri, F., Santoro, L., Crisci, C. et al. Is the sensory neuropathy in ataxia-telangiectasia distinguishable from that in Friedreich's ataxia?. Acta Neuropathol 69, 213–219 (1986). https://doi.org/10.1007/BF00688296

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  • Received: 23 April 1985

  • Accepted: 01 October 1985

  • Issue Date: September 1986

  • DOI: https://doi.org/10.1007/BF00688296

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