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AJNR - American Journal of Neuroradiology

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Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy: Decrease in Regional Cerebral Blood Volume in Hyperintense Subcortical Lesions Inversely Correlates with Disability and Cognitive Performance

Roland Bruening^a, Martin Dichgans^a, Christian Berchtenbreiter^a, Tarek Yousry^a, Klaus C. Seelos^a, Ren H. Wu^a, Michael Mayer^a, Gunnar Brix^a and Maximilian Reiser^a

^a From the Institute of Clinical Radiology (R.B., C.B., M.R.), Ludwig-Maximilians University, Munich, Germany; the Department of Neurology (M.D., M.M.), University of Munich, Munich, Germany; the Department of Neuroradiology at the Institute of Clinical Radiology (T.Y., K.C.S.), Ludwig-Maximilians University, Munich, Germany; the Department of Radiology (R.H.W.), University of Virginia Health Science Center, Charlottesville, VA; and the Department of Medical Radiation Hygiene (G.B.), Institute for Radiation Hygiene, Federal Office for Radiation Protection, Munich, Germany.

View larger version (17K): [in a new window]   FIG 1. Mean rCBV of 24 patients with CADASIL, as measured in gray matter and subcortical white matter

View larger version (116K): [in a new window]   FIG 2. MR imaging findings of a 53-year-old man with CADASIL (MMSE score, not measurable; Rankin score, 4). A, T2-weighted image (2300/85/1). Abnormal increases in the signal intensity in the subcortical white matter are seen in the frontal and occipital white matter (arrows) and in the basal ganglia bilaterally. B, rCBV map of the same section. Decrease of rCBV in the frontal and occipital lobes is detectable (arrows)

View larger version (112K): [in a new window]   FIG 3. MR imaging findings of a 50-year-old man with CADASIL (MMSE score, 21; Rankin score, 1). A, T2-weighted image (2300/85/1). Lacunar defects (one arrow) and subcortical white matter abnormalities were detected (two arrows). B, rCBV map of the same section. Bilateral reduction of rCBV in the frontal lobes is visible on the rCBV map (two arrows). Lacunar defect visible on the T2-weighted image was also visible (one arrow). rCBV was calculated to be zero

View larger version (14K): [in a new window]   FIG 4. rCBV values of 24 patients with CADASIL in normal appearing white matter as a function of age (error bars denote SD; blue dot denotes mean values).FIG 5. rCBV determined in the subcortical CADASIL lesions for each age group

View larger version (16K): [in a new window]   FIG 6. Correlation between disability (Rankin score) of patients with CADASIL and rCBV in white matter that appeared abnormal on T2-weighted images. In our group, no patient had a Rankin score of 3. One patient with a Rankin score of 5 had an rCBV of 2.2 mL/100 g. (* = statistically different from Rankin = 0.)FIG 7. Correlation between cognitive performance (MMSE score, 22) and the rCBV in white matter that appeared abnormal on T2-weighted images. (*See text for definition of cognitive impairment.)