A PECULIAR FORM OF PERIPHERAL NEUROPATHY - FAMILIAR ATYPICAL GENERALIZED AMYLOIDOSIS WITH SPECIAL INVOLVEMENT OF THE PERIPHERAL NERVES
A PECULIAR FORM OF PERIPHERAL NEUROPATHY - FAMILIAR ATYPICAL GENERALIZED AMYLOIDOSIS WITH SPECIAL INVOLVEMENT OF THE PERIPHERAL NERVES
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DOI：
10.1093/brain/75.3.408
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发表时间：
1952-01-01
期刊：
影响因子：
14.5
通讯作者：
ANDRADE, C
中科院分区：
文献类型：
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作者：
ANDRADE, C
这是一种地方性疾病，具有：（1）轻瘫，特别是下肢，（2）腿的热感觉和疼痛感觉的早期损害，（3）胃肠疾病，（4）性功能和括约肌疾病。最重要的组织学发现是存在广泛性淀粉样变性，特别是高浓度。淀粉样蛋白在肾脏周围神经和胰腺中的分布提请注意该疾病的家族性及其在葡萄牙某些地区的流行情况。病因和致病因素尚不清楚。
This is an endemic disease with: (1) paresis particularly in the lower extremities, (2) early impairment of thermal and painful sensibilities of the legs, (3) gastro-intestinal disorders, (4) sexual and sphincter disorders. The most important histological finding was the presence of generalized amyloidosis with especially high concn. of the amyloid in the kidney, peripheral nerves and pancreas. Attention is drawn to the familial nature of the disease and its prevalence in certain districts of Portugal. The etiologic and pathogenic factors are unknown.