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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 20:91-100 (1 1999)
© 1999 American Society of Neuroradiology

ARTICLE

Characteristic MR Lesion Pattern and Correlation of T1 and T2 Lesion Volume with Neurologic and Neuropsychological Findings in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)^,

Tarek A. Yousry^,a, Klaus Seelos^a, Michael Mayer^a, Roland Brüning^a, Ingo Uttner^a, Martin Dichgans^a, Sylvia Mammi^a, Andreas Straube^a, Norbert Mai^a and Massimo Filippi^a

^a From the Departments of Neuroradiology (T.A.Y., K.S.), Neurology (M.M., I.U., M.D., A.S., N.M.), and Diagnostic Radiology (R.B.), Klinikum Grosshadern, Ludwig-Maximilian University, Munich, Germany; and the Department of Neurology, Scientific Institute Ospedale San Raffaele, University of Milan, Italy (S.M., M.F.).

BACKGROUND AND PURPOSE: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an arteriopathy related to a genetic defect of the notch 3 gene on chromosome 19. The purpose of this study was to evaluate lesion distribution and volume using MR imaging and to correlate the lesion volume with the neurologic and neuropsychological findings.

METHODS: Twenty members of two families (14 with CADASIL as determined by linkage analysis, six healthy) were studied with MR imaging. Two observers evaluated the MR findings semiquantitatively and quantitatively. MR results were then correlated with neurologic and neuropsychological findings.

RESULTS: A typical pattern of lesion distribution in patients with CADASIL was found: the frontal lobe was the site with the highest lesion load, followed by the temporal lobe and the insula. The total lesion volume on T1-weighted MR images correlated significantly with the degree of disability and the degree of impairment in neuropsychological functions (including attention, memory, and conceptual and visuospatial functions).

CONCLUSION: In CADASIL patients, a common pattern of cerebral lesion distribution is found. The total T1 lesion volume is an important parameter to correlate with disability, as it may prove to be helpful in predicting the natural history of the disease.

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