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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 22:1268-1274 (8 2001)
© 2001 American Society of Neuroradiology

ARTICLE

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.

BACKGROUND AND PURPOSE: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an arteriopathic syndrome related to a genetic defect on chromosome 19. Characteristic changes in CADASIL can be observed onT2-weighted MR images in the subcortical white matter. The purpose of this study was to measure changes of regional cerebral blood volume (rCBV) with dynamic contrast-enhanced MR imaging and to correlate the changes to disability and cognitive performance.

METHODS: We obtained rCBV measurements of 24 individuals with proven CADASIL on a 1.5-T MR imaging unit. A susceptibility-weighted MR imaging sequence was used for bolus tracking. Principles of the indicator dilution theory were applied to estimate values of absolute rCBV (mL/100 g). Disability was determined by using the Rankin scale, and overall cognitive performance was assessed by using the Mini-Mental State Examination.

RESULTS: The mean rCBV in the subcortical white matter that was hyperintense on the T2-weighted images (2.7 ± 0.8 mL/100 g) was significantly lower than the rCBV in the white matter that appeared normal on the T2-weighted images (4.4 ± 1.3 mL/100 g) (P < .05). The mean rCBV in the gray matter was within the normal range (8.3 ± 1.7 mL/100 g). Both cognitive impairment and disability negatively correlated with rCBV in the subcortical white matter that was hyperintense (P < .05) but not with rCBV in the normal appearing white matter. rCBV did not correlate with age.

CONCLUSION: rCBV measured in the hyperintense subcortical white matter in individuals with CADASIL was decreased and inversely correlated with disability and cognitive impairment.

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				K. Amberla, M. Waljas, S. Tuominen, O. Almkvist, M. Poyhonen, S. Tuisku, H. Kalimo, and M. Viitanen Insidious Cognitive Decline in CADASIL Stroke, July 1, 2004; 35(7): 1598 - 1602. [Abstract] [Full Text] [PDF]

				S. Tuominen, Q. Miao, T. Kurki, S. Tuisku, M. Poyhonen, H. Kalimo, M. Viitanen, H. T. Sipila, J. Bergman, and J. O. Rinne Positron Emission Tomography Examination of Cerebral Blood Flow and Glucose Metabolism in Young CADASIL Patients Stroke, May 1, 2004; 35(5): 1063 - 1067. [Abstract] [Full Text] [PDF]