par Borenstein, Samuel 
;Noël, Philippe ;Jacquy, Jean ;Flament Durand, Jacqueline
Référence Journal of the neurological sciences, 34, 1, page (87-99)
Publication Publié, 1977
;Noël, Philippe ;Jacquy, Jean ;Flament Durand, Jacqueline Référence Journal of the neurological sciences, 34, 1, page (87-99)
Publication Publié, 1977
Article révisé par les pairs
| Résumé : | A striking enlargement of peripheral nerves was observed in a patient with myotonic dystrophy (Steinert's disease). Clinical findings disclosed a polyneuropathy which was confirmed by electrophysiological and morphological investigations. Myopathic as well as neurogenic alterations were found on electromyography in wasted hand muscles. The prominent finding on ultrastructural study of a sural nerve biopsy was an important collagen infiltration with rather few 'onion bulb' formations. This extensive endoneural collagen formation appeared to be responsible for the nerve hypertrophy. Evidence of segmental demyelination and remyelination was also found. Glycogen deposits were present in some myelinated axons and in some Schwann cells. Peculiar crystalline inclusions were observed in fibroblast processes. The relation between myotonic dystrophy and polyneuropathy remains a matter of discussion. |
