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[I型多发性内分泌综合征中的多发性神经病]

[Polyneuropathy in type I multiple endocrine syndrome].

作者信息

Conri C, Ducloux G, Lagueny A, Ferrer M, Vital C

机构信息

Service de Médecine interne, Centre François Magendie, Hôpital du Haut-Levêque, Pessac.

出版信息

Presse Med. 1990 Feb 17;19(6):247-50.

PMID:2156253
Abstract

Since 1928, approximately 30 cases of peripheral polyneuropathy consecutive to hypoglycaemia caused by insulinomas or islet cell pancreatic tumours have been published. We report a case of polyneuropathy in a young woman presenting with type I multiple endocrine neoplasia syndrome including multiple insulinomas and parathyroid adenomas. The predominantly motor polyneuropathy was symmetrical and involved both the upper and lower limbs. Biopsy showed muscular atrophy and, in particular non-specific acute axonal lesions. Twelve months after surgery the clinical situation was relatively favourable with resumption of physical activities but persistence of muscular atrophy. Wether hyperparathyroidism was responsible for the neuropathy was uncertain, the subacute post-hypoglycaemia axonal damage being a more likely suspect.

摘要

自1928年以来,已发表了约30例由胰岛素瘤或胰岛细胞胰腺肿瘤引起低血糖继发的周围性多发性神经病病例。我们报告一例年轻女性的多发性神经病,该患者患有I型多发性内分泌肿瘤综合征,包括多发性胰岛素瘤和甲状旁腺腺瘤。以运动为主的多发性神经病呈对称性,累及上下肢。活检显示肌肉萎缩,特别是非特异性急性轴索性病变。手术后12个月,临床情况相对良好,患者恢复了体力活动,但肌肉萎缩仍持续存在。甲状旁腺功能亢进是否导致了神经病尚不确定,低血糖后亚急性轴索损伤更有可能是病因。

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