Variability of morphological features in early infantile polyneuropathy with defective myelination |
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| Authors: | A Vital C Vital J P Riviere C Brechenmacher J Marot |
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| Institution: | (1) Centre de Microscopie Electronique, Université de Bordeaux II, F-33076 Bordeaux Cedex, France;(2) Centre Hospitalier de Saint Denis, La Réunion, France;(3) Centre Hospitalier, Angoulême, France |
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| Abstract: | Summary Four cases of early infantile polyneuropathy with defective myelination are reported. The peripheral nerve was studied by light and electron microscopy; different morphological characteristics have been noticed in these patients. Case 1 presented aspects of defective myelination with atypical  onion bulb formation composed of multiple layers of basement membrane. In case 2, defective myelination and atypical onion bulb formation were associated with aberrant hypermyelination. Cases 3 and 4 were brothers, who presented axonal damage and atypical onion bulb formation. |
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| Keywords: | Axon Myelin Infantile polyneuropathy Peripheral nerve Schwann cell |
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