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The Italian Journal of Neurological Sciences

, Volume 3, Issue 4, pp 341–348 | Cite as

Peripheral neuropathy in the course of progressive systemic sclerosis: Light and ultrastructural study

  • Trapani G. Di 
  • Pocchiari M. 
  • Masullo C. 
  • Albanese A. 
  • Tulli A. 
Case Reports

Abstract

We present the case of a woman with progressive systemic sclerosis (PSS) in whom the usual symptoms were preceded by a rapidly progressive peripheral neuropathy. Few cases of peripheral nerve involvement have been described. For the first time we report an ultrastructural study of an affected peripheral nerve and muscle.

In the sural nerve we found an almost complete loss of myelinated fibers. Schwann cells showed an abnormal hyperplasia of their basal membranes and structural signs of denervation. Spindle-shaped banded structures were seen in the cytoplasm of Schwann cells and in the endoneurium. On the basis of these ultrastructural data some hypotheses on the pathogenetic mechanism of this neuropathy are discussed.

Key-words

progressive systemic sclerosis peripheral nervous system electron microscopy 

Sommario

Viene presentato un caso di una paziente affetta da Sclerosi Sistemica Progressiva, caratterizzata dalla presenza di una polineuropatia ad esordio precoce accanto ai sintomi tipici della malattia. In letteratura tale associazione è stata descritta raramente. In questo lavoro viene descritto per la prima volta lo studio ultrastrutturale del nemo periferico affetto. Lo studio neuropatologico del nemo surale mostra una quasi completa perdita di fibre mieliniche. Le cellule di Schwann mostrano una anormale iperplasia della membrana basale con segni morfologici di denervazione. Di particolare interesse è la presenza di corpi di Luse (spindle-shaped banded structures) sia all'interno del citoplasma schwannico sia nell'endonevrio. Sulla base dei dati ultrastrutturali vengono discusse alcune ipotesi sul meccanismo patogenetico di questa neuropatia.

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Copyright information

© Masson Italia Editori S.p.A. 1982

Authors and Affiliations

  • Trapani G. Di 
    • 2
  • Pocchiari M. 
    • 2
  • Masullo C. 
    • 2
  • Albanese A. 
    • 2
  • Tulli A. 
    • 2
    • 1
  1. 1.Istituto di NeurologiaUniversità Cattolica del S. CuoreRoma
  2. 2.Istituto di Clinica DermatologicaUniversità Cattolica del S. CuoreRoma

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