Groeschel Samuel, Brockmann Knut, Hanefeld Folker
Department of Pediatrics and Neuropediatrics, Georg-August-University, Goettingen, Germany.
Eur J Paediatr Neurol. 2007 May;11(3):142-5. doi: 10.1016/j.ejpn.2006.11.014. Epub 2007 Jan 24.
Perivascular spaces of the brain, also known as Virchow-Robin spaces (VRS), are of immunological and neuropathological relevance and can be observed in magnetic resonance images (MRI). Their histopathological significance in X-linked adrenoleukodystrophy (ALD) has been reported. Aim of this study was to elucidate the prognostic or diagnostic value of VRS on MRI of the brain in the evaluation of onset or severity of the clinical course in ALD.
Clinical data and MRI from 35 patients with the cerebral form of X-ALD, 29 with the asymptomatic form (including those with adrenal insufficiency), and 36 control patients were studied retrospectively.
VRS could be visualised by MRI in 87% of patients with asymptomatic ALD, in 80% of control patients, and in 47% of patients with cerebral ALD. None of them were found to be dilated. The number of visible VRS correlated negatively with the degree of demyelination both in patients with the cerebral and the asymptomatic form. Furthermore, in the group of patients with cerebral ALD the number of visible VRS correlated positively with a milder course of the disease.
VRS on MRI of patients with ALD seem to reflect the perivascular inflammatory component of this disease. It is possible to speculate that the appearance or a higher number of visible VRS in ALD is associated with an earlier stage of the disease, or even a more benign clinical course.
脑周血管间隙,也称为魏尔啸-罗罗宾间隙(VRS),具有免疫和神经病理学相关性,可在磁共振成像(MRI)中观察到。其在X连锁肾上腺脑白质营养不良(ALD)中的组织病理学意义已有报道。本研究的目的是阐明MRI上脑VRS在评估ALD临床病程的发作或严重程度方面的预后或诊断价值。
回顾性研究了35例脑型X-ALD患者、29例无症状型(包括肾上腺功能不全患者)和36例对照患者的临床资料和MRI。
MRI可在87%的无症状ALD患者、80%的对照患者和47%的脑型ALD患者中显示VRS。未发现它们有扩张。在脑型和无症状型患者中,可见VRS的数量与脱髓鞘程度呈负相关。此外,在脑型ALD患者组中,可见VRS的数量与疾病较轻的病程呈正相关。
ALD患者MRI上的VRS似乎反映了该疾病的血管周围炎症成分。可以推测,ALD中可见VRS的出现或数量增加与疾病的早期阶段甚至更良性的临床病程有关。