Dilation of Virchow‐Robin spaces in CADASIL

Abstract

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.

Frequency and Location of Dilated Virchow-Robin Spaces in Elderly People: A Population-Based 3D MR Imaging Study

“…A special MR imaging change of cribriform cavities in the BG was the feature of this group. An "état criblé" or "status cribrosum" has been previously proved to be multiple dVRS in pathologic studies 19 and has been reported only among cases of vascular Parkinsonism, 20 subcortical ischemic vascular dementia, 21,22 or CADASIL 23 but has not been reported in the general population, to our knowledge. The present data further emphasize that the accumulation of multiple dVRS is not rare and can occur in elderly people in the absence of severe disability or dementia.…”

“…23 This may suggest 1 or several potential causes of dVRS with aging. 15 In this study, a higher grade of dVRS in the BG was observed more frequently in men than in women.…”

Subcortical Cystic Lesions within the Anterior Superior Temporal Gyrus: A Newly Recognized Characteristic Location for Dilated Perivascular Spaces

“…1 Diffusely enlarged perivascular spaces have also been reported, which have been hypothesized to occur in the context of certain pathologic conditions, including chronic microvascular ischemia, Alzheimer dementia, cerebral amyloid angiopathy, CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), and mucopolysaccharidoses. [4][5][6][7][8] Regarding the function of perivascular spaces, several theories have been proposed. They are thought to play a role in immunologic processes in the brain, to provide a drainage route for interstitial fluid, and to provide a mechanism for equalization of intracranial pressure.…”

CADASIL: pathogenesis, clinical and radiological findings and treatment

“…Gradually, diffuse myelin pallor and rarefaction of hemispheric white matter (sparing the U fibers), deep focal lesions, particularly in periventricular areas and centrum semiovale, and lacunar infarctions in the white matter, basal ganglia and pons add up 21,24 . Virchow-Robin spaces tend to become dilated, perhaps by changes related to age or the progression of degeneration of penetrating cerebral arteries 25 . Further changes were demonstrated by MR spectroscopy: metabolic reductions in the relationship between mean NAA/Cr, NAA/Cho and Cho/Cr become obvious, especially in anterior (VS. posterior) regions of the centrum semiovale 26 .…”