Dilation of Virchow-Robin spaces in CADASIL

Dilation of Virchow-Robin spaces in CADASIL
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DOI:
10.1111/j.1468-1331.2006.01113.x
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发表时间:
2006-02-01
影响因子:
5.1
通讯作者:
Chabriat, H
Chabriat, H
中科院分区:
医学3区
文献类型:
--
作者:
Cumurciuc, R;Guichard, JP;Chabriat, H

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精确CADASIL患者中Virchow-Robin间隙(VRS)扩张的严重程度,并确定其与临床表现和脑磁共振成像(MRI)其他异常的相关性。扩张的VRS先前与衰老、高血压、痴呆、癫痫或偏头痛相关。我们已经报道了CADASIL患者与没有受累单倍型的家族成员相比,VRS扩大的频率增加。我们分析了在我们中心前瞻性收集的50例CADASIL患者的临床和MRI数据。在颞叶皮质下白色物质、半卵圆中心和基底节中评估扩张VRS的存在。根据Heier提出的量表对各地区的严重程度进行了评价。我们比较了有和没有扩张VRS患者的临床资料、白色异常的严重程度和微出血的存在。78%的患者VRS扩张,主要位于豆状核(94%)和颞叶皮质下白色物质(66%)。这些病变的严重程度是可变的,但既不相关的白色物质异常的程度,也没有我们的患者的临床表现。只有年龄与VRS扩张程度有关。CADASIL中扩张的VRS常见,主要位于颞部白色物质和基底节。血管周围间隙的扩张似乎与CADASIL中缺血性或出血性病变的发生没有直接关系。相反,与年龄的关系表明,在疾病过程中,年龄、血管壁变化的进展或这两个过程都有利于CADASIL中VRS的扩展。
To precise the severity of dilated Virchow-Robin spaces (VRS) in CADASIL patients and to determine their correlation with clinical presentation and other abnormalities on cerebral Magnetic Resonance Imaging (MRI). Dilated VRS were previously associated with aging, hypertension, dementia, epilepsy or migraine. We already reported increased frequency of enlarged VRS in CADASIL patients when compared with family members without the affected haplotype. We analysed clinical and MRI data from 50 CADASIL patients collected prospectively in our center. The presence of dilated VRS was assessed in the subcortical white matter of temporal lobes, the centrum semi-ovale and the basal ganglia. Their severity in each region was evaluated according to the scale proposed by Heier. We compared the clinical data, the severity of white matter abnormalities and the presence of microbleeds in patients with and without dilated VRS. Seventy-eight percent of patients in our series had dilated VRS, mostly located in the lentiform nuclei (94%) and subcortical white matter of the temporal lobes (66%). The severity of these lesions was variable but not correlated neither to the extent of white matter abnormalities nor to the clinical presentation in our patients. Only the age was found to be related to the extent of dilated VRS. Dilated VRS are frequent in CADASIL and mostly located in the temporal white matter and basal ganglia. The dilation of perivascular spaces does not seem to be directly related to the occurrence of ischemic or hemorrhagic lesions in CADASIL. In contrast, the relation with age suggests that either aging, progression of vascular wall alterations during the course of the disease, or both of these processes can favour the extension of VRS in CADASIL.