Vital A, Vital C, Brechenmacher C, Fontan D, Castaing Y
Laboratoire d'Anatomie Pathologique, Hôpital Pellegrin, Bordeaux, France.
Eur J Pediatr. 1990 Jun;149(9):654-8. doi: 10.1007/BF02034757.
Peripheral nerve biopsies (PNB) from four children suffering from subacute or chronic inflammatory demyelinating polyneuropathy were studied by electron microscopy. Remyelinating features with onion bulb formations, inflammatory cell infiltrates and active demyelinating lesions were strongly suggestive of the disease. In the first case, a second PNB, performed after 7 months of severe subacute course, showed a striking evolution of the lesions. In the second case and in spite of severe neurological symptoms, the PNB was almost normal, suggesting that inflammatory lesions were mainly located in the proximal parts of the nerve. No signs of active demyelination could be seen in the third case but onion bulb formations and inflammatory cell infiltrates were present. In the fourth case, characteristic lesions of active demyelination were associated with a history of familial polyneuropathy; this association suggests an auto-immune process in certain kindreds with hereditary motor and sensory neuropathy.
对4例患有亚急性或慢性炎症性脱髓鞘性多发性神经病的儿童进行了周围神经活检（PNB），并通过电子显微镜进行研究。有洋葱球形成、炎性细胞浸润和活动性脱髓鞘病变的再髓鞘化特征强烈提示该疾病。在第一例中，在严重亚急性病程7个月后进行的第二次PNB显示病变有显著进展。在第二例中，尽管有严重的神经症状，但PNB几乎正常，提示炎性病变主要位于神经近端。在第三例中未见活动性脱髓鞘迹象，但存在洋葱球形成和炎性细胞浸润。在第四例中，活动性脱髓鞘的特征性病变与家族性多发性神经病病史相关；这种关联提示在某些遗传性运动和感觉神经病的亲属中存在自身免疫过程。
