Quantitative sensation and autonomic test abnormalities in transthyretin amyloidosis polyneuropathy.
Quantitative sensation and autonomic test abnormalities in transthyretin amyloidosis polyneuropathy.
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DOI：
10.1002/mus.21332
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发表时间：
2009-09
期刊：
影响因子：
3.4
通讯作者：
Dyck, Peter J.
中科院分区：
文献类型：
--
作者：
Kim, Dong Hwee;Zeldenrust, Steven R.;Low, Phillip A.;Dyck, Peter J.
关键词：
本研究评估标准定量自主神经(QAT)和感觉(QST)测试在检测、表征和量化甲状腺素淀粉样多发性神经病(TTR-A-PN)严重程度方面的价值。这些信息是前瞻性治疗试验、流行病学调查和医疗实践所需要的。我们回顾了1997至2007年间接受评估的36例TTR-A-PN患者。他们进行了神经学、遗传学、电诊断和自主神经反射筛查评估，并允许出于研究目的评估他们的医疗记录和测试结果。其中，22名患者还接受了定量感觉测试(QST)。中位症状持续时间为4年(1~30年)。在所评价的定量神经检查中，神经传导综合评分(Σ5 NC NDS)、QST综合评分(Σ3QST NDS)和定量自主神经测试(QSART、HRDB和CASS)的异常发生率较高。这些结果表明，我们研究的大多数患者的外周自主神经和小纤维感觉功能障碍是突出的和特征的。然而，对于小直径的感觉神经纤维和自主神经纤维，这种受累并不是有选择性的；大的运动和感觉神经纤维也显示出功能障碍。大纤维功能障碍与小纤维功能障碍的发生率大致相同。这项研究为在流行病学调查、治疗试验和医疗实践中使用QAT、QST和Σ5 NC NDS作为量化TTRA-PN严重程度的标准、客观和定量测量方法提供了理论依据。
This study assesses the value of standard quantitative autonomic (QAT) and sensation (QST) tests in detecting, characterizing, and quantitating the severity of transthyretin amyloid polyneuropathy (TTR-A-PN). This information is needed for prospective therapeutic trials, epidemiologic surveys and medical practice. We reviewed thirty six patients with TTR-A-PN who were evaluated between 1997 and 2007. They had neurologic, genetic, electrodiagnostic, and autonomic reflex screen evaluations and allowed their medical records and test results to be evaluated for research purposes. Of these, 22 patients had also been tested by quantitative sensation tests (QSTs). The median symptom duration was 4 years (range 1 to 30 years). Among quantitative nerve tests evaluated, composite scores of nerve conduction (Σ 5 NC nds), a composite score of QSTs (Σ3QST nds), and quantitative autonomic tests (QSART, HRdb, and CASS) gave high frequencies of abnormality. These results show that peripheral autonomic and small fiber sensory dysfunction is prominent and characteristic of most of the patients we studied. However this involvement was not selective for small-diameter sensory and autonomic nerve fibers; large motor and sensory fibers were also shown to be dysfunctional. Dysfunction of large fibers was approximately as frequent as that of small fibers. This study provides a rationale for use of QAT, QST, and Σ 5 NC nds as standard, objective and quantitative measures for quantitating the severity of TTR-A-PN in epidemiologic surveys, therapeutic trials and medical practice.
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影响因子：
9.9
作者：
DYCK, PJ;LITCHY, WJ;OBRIEN, PC
通讯作者：
OBRIEN, PC
影响因子：
11.2
作者：
LOW, PA;CASKEY, PE;DYCK, PJ
通讯作者：
DYCK, PJ
DOI：
10.1073/pnas.75.9.4499
发表时间：
1978-01-01
影响因子：
11.1
作者：
COSTA, PP;FIGUEIRA, AS;BRAVO, FR
通讯作者：
BRAVO, FR
DOI：
10.1016/s0006-291x(02)00465-5
发表时间：
2002-06-07
影响因子：
3.1
作者：
Andersson, K;Olofsson, A;Lundgren, E
通讯作者：
Lundgren, E
影响因子：
9.9
作者：
Koike, H;Misu, K;Sobue, G
通讯作者：
Sobue, G