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急性全自主神经功能不全

Acute panautonomic neuropathy.

作者信息

Low P A, Dyck P J, Lambert E H, Brimijoin W S, Trautmann J C, Malagelada J R, Fealey R D, Barrett D M

出版信息

Ann Neurol. 1983 Apr;13(4):412-7. doi: 10.1002/ana.410130407.

Abstract

Two patients with acute panautonomic neuropathy had severe impairment of sympathetic and parasympathetic function of acute onset. Autonomic tests suggesting a postganglionic lesion and direct evidence of loss of unmyelinated and small myelinated fibers on nerve biopsy were found in Patient 1. There was a selective loss of small myelinated and unmyelinated nerve fibers; C potential was absent in the in vitro compound action potential; dopamine-beta-hydroxylase activity was unmeasurable. No definite abnormalities were found on the nerve biopsy of Patient 2. The acute panautonomic neuropathies appear to comprise a spectrum of somatic and autonomic involvement.

摘要

两名急性全自主神经病变患者出现急性起病的交感和副交感神经功能严重受损。患者1的自主神经测试提示节后病变,神经活检发现无髓鞘和小髓鞘纤维丢失的直接证据。存在小髓鞘和无髓鞘神经纤维的选择性丢失;体外复合动作电位中C波消失;多巴胺-β-羟化酶活性无法测量。患者2的神经活检未发现明确异常。急性全自主神经病变似乎包括一系列躯体和自主神经受累情况。

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