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Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome

Ultrastructural study of peripheral nerve biopsy from 22 patients

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Abstract

Mechanisms of peripheral neuropathies in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome are poorly understood. A peripheral nerve biopsy was performed in 22 patients. Of these 9 had histological features of Castleman's disease on lymph node biopsies, and 19 had a monoclonal lambda light chain in their serum. Certain nerve fragments were paraffin embedded, others were frozen and studied by direct immuno-fluorescence, and others were fixed for ultrastructural examination. Paraffin-embedded fragments did not show any amyloid deposits, and at direct immunofluorescence there was no immunoglobulin fixation. At ultrastructural examination, features of uncompacted myelin lamellae (UML) were present in 19 patients, and their frequency varied from 1% to 16% of myelinated fibres. Up to now UML have been reported only in 7 patients with POEMS syndrome in the literature. UML have also been noticed in a few cases of inflammatory demyelinating polyradiculoneuritis and inherited tendency to pressure palsy.

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Authors and Affiliations

  1. Laboratoire d'Anatomie-Pathologique, Hôpital Pellegrin, F-33076, Bordeaux Cedex, France

    C. Vital & A. Vital

  2. X, Créteil, France

    R. Gherardi

  3. Lyon, France

    N. Kopp

  4. Marseille, France

    J. F. Pellissier

  5. Clermont-Ferrand, France

    M. Soubrier & P. Clavelou

  6. Fort-de-France, France

    R. Bellance

  7. Toulouse, France

    M. B. Delisle

  8. Tours, France

    M. M. Ruchoux

  9. Salpétrière, Paris, France

    J. J. Hauw

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Vital, C., Gherardi, R., Vital, A. et al. Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome. Acta Neuropathol 87, 302–307 (1994). https://doi.org/10.1007/BF00296746

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  • DOI: https://doi.org/10.1007/BF00296746

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