Severity and patterns of blood-nerve barrier breakdown in patients with chronic inflammatory demyelinating polyradiculoneuropathy: correlations with clinical subtypes.
Severity and patterns of blood-nerve barrier breakdown in patients with chronic inflammatory demyelinating polyradiculoneuropathy: correlations with clinical subtypes.
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DOI：
10.1371/journal.pone.0104205
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发表时间：
2014
期刊：
影响因子：
3.7
通讯作者：
Kanda T
中科院分区：
文献类型：
--
作者：
Shimizu F;Sawai S;Sano Y;Beppu M;Misawa S;Nishihara H;Koga M;Kuwabara S;Kanda T
慢性炎性脱髓鞘性多发性神经根神经病（CIDP）目前分为临床亚型，包括典型和非典型形式（多灶性获得性脱髓鞘性感觉和运动神经病（MADSAM）和远端获得性脱髓鞘性对称性神经病（DADS））。本研究的目的是阐明的模式和严重程度的破坏的血神经屏障（BNB）在每一个CIDP亚型。我们评价了从典型CIDP、MADSAM和DADS患者和对照受试者中获得的血清对人外周神经微血管内皮细胞（PnMEC）的紧密连接蛋白表达水平和跨内皮电阻（TEER）值的影响。从具有CIDP的三种临床表型的患者获得的血清降低了PnMEC中紧密连接蛋白-5蛋白水平的量和TEER值。此外，从典型CIDP患者获得的血清比从MADSAM和DADS患者获得的血清更显著地降低PnMEC中的claudin-5蛋白水平和TEER值。此外，暴露于血清后BNB中断的严重程度与较高的Hughes分级、较低的MRC评分、正中神经运动神经传导的更明显减慢和异常时间离散度的更高频率相关。来自典型CIDP患者的血清比来自MADSAM或DADS患者的血清更严重地破坏BNB。在CIDP的情况下，BNB破坏的程度与临床残疾和神经干脱髓鞘有关。这些观察结果可以解释CIDP亚型之间的表型差异。
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is currently classified into clinical subtypes, including typical and atypical forms (multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) and distal acquired demyelinating symmetric neuropathy (DADS)). The aim of this study was to elucidate the patterns and severity of breakdown of the blood-nerve barrier (BNB) in each CIDP subtype. We evaluated the effects of sera obtained from patients with typical CIDP, MADSAM and DADS and control subjects on the expression levels of tight junction proteins and transendothelial electrical resistance (TEER) value in human peripheral nerve microvascular endothelial cells (PnMECs). The sera obtained from the patients with the three clinical phenotypes of CIDP decreased the amount of claudin-5 protein levels and TEER values in the PnMECs. In addition, the sera obtained from typical CIDP patients more prominently reduced claudin-5 protein levels and TEER values in the PnMECs than did that obtained from the MADSAM and DADS patients. Furthermore, the severity of BNB disruption after exposure to the sera was associated with higher Hughes grade, lower MRC score, more pronounced slowing of motor nerve conduction in the median nerve and higher frequency of abnormal temporal dispersion. Sera derived from typical CIDP patients destroy the BNB more severely than those from MADSAM or DADS patients. The extent of BNB disruption in the setting of CIDP is associated with clinical disability and demyelination in the nerve trunk. These observations may explain the phenotypical differences between CIDP subtypes.
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影响因子：
11
作者：
Kuwabara, S;Ogawara, K;Hattori, T
通讯作者：
Hattori, T
影响因子：
11
作者：
Kanda, T;Numata, Y;Mizusawa, H
通讯作者：
Mizusawa, H
影响因子：
11
作者：
Kuwabara, S;Misawa, S;Hattori, T
通讯作者：
Hattori, T
影响因子：
9.9
作者：
Katz, JS;Saperstein, DS;Barohn, RJ
通讯作者：
Barohn, RJ
影响因子：
2
作者：
Katz, Jonathan S.;Saperstein, David S.
通讯作者：
Saperstein, David S.