Völker W, Ringelstein E B, Dittrich R, Maintz D, Nassenstein I, Heindel W, Grewe S, Kuhlenbäumer G
Institute of Experimental Medicine, University of Kiel, Neurozentrum, Schittenhelmstr. 10, D-24105 Kiel, Germany.
J Neurol Neurosurg Psychiatry. 2008 Sep;79(9):1007-12. doi: 10.1136/jnnp.2007.115634. Epub 2008 Jan 25.
The aetiopathogenesis of spontaneous cervical artery dissection (sCAD) is largely unknown. Electron microscopic (EM) examination of skin biopsies of patients with sCAD revealed very subtle pathological changes of dermal connective tissue in about half of these patients leading to the hypothesis of an underlying connective tissue disorder. However, connective tissue abnormalities did not allow clear discrimination between patients and controls in our hands. Therefore, we sought to establish an objective basis for the assessment of connective tissue abnormalities in patients with sCAD using standardised morphometric assessment of collagen fibrils.
In this study a blinded examination was performed of collagen in skin biopsies and it sought parameters which are able to discriminate between patients with sCAD and controls. Various morphometric parameters were compared between patients with sCAD (n = 20) and control subjects (n = 18).
Previously described "flower-like" collagen fibrils in skin biopsies were extremely rare in patients and controls and did not discriminate between the groups. However, they were abundant in the skin biopsy of a patient with Ehlers-Danlos syndrome type III (EDSIII) used as a reference. Morphometric parameters such as collagen fibril diameter, fibril density and relative fibril area did not discriminate between patients and controls on an individual basis, but the mean diameter of collagen fibrils in the skin was lower in patients with sCAD compared with controls while fibril density was higher resulting in nearly equal fibril areas per unit of area (relative fibril area) comparing both groups as well as individuals.
Blinded pathological and morphometric analysis of collagen fibres in skin biopsies was, in our hands, unable to discriminate reliably between patients with sCAD and controls on an individual basis but did show differences in collagen fibril morphometry on a group basis. Furthermore, systematic and blinded pathological studies of skin biopsies in patients with sCAD and controls are needed.
自发性颈内动脉夹层(sCAD)的发病机制尚不清楚。对sCAD患者的皮肤活检进行电子显微镜(EM)检查发现,约半数患者的真皮结缔组织存在非常细微的病理变化,这引发了关于潜在结缔组织疾病的假说。然而,在我们的研究中,结缔组织异常并不能明确区分患者和对照组。因此,我们试图通过对胶原纤维进行标准化形态计量评估,为评估sCAD患者的结缔组织异常建立一个客观依据。
在本研究中,对皮肤活检中的胶原进行了盲法检查,并寻找能够区分sCAD患者和对照组的参数。比较了20例sCAD患者和18例对照受试者之间的各种形态计量参数。
先前描述的皮肤活检中的“花状”胶原纤维在患者和对照组中极为罕见,无法区分两组。然而,在用作参考的III型埃勒斯-当洛综合征(EDSIII)患者的皮肤活检中,它们大量存在。胶原纤维直径、纤维密度和相对纤维面积等形态计量参数在个体水平上无法区分患者和对照组,但与对照组相比,sCAD患者皮肤中胶原纤维的平均直径较低,而纤维密度较高,导致两组以及个体的每单位面积纤维面积(相对纤维面积)几乎相等。
在我们的研究中,对皮肤活检中的胶原纤维进行盲法病理和形态计量分析,无法在个体水平上可靠地区分sCAD患者和对照组,但在组水平上确实显示出胶原纤维形态计量学的差异。此外,需要对sCAD患者和对照组的皮肤活检进行系统的盲法病理研究。