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Topic 1091 Version 27.0 https://www.uptodate.com/contents/cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-cadasil/print 27/32 7/5/23, 12:35 PM Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) - UpToDate GRAPHICS Main MRI changes in CADASIL (A) Lacunar infarcts shown on T1-weighted imaging are mainly located in the brainstem (pons), thalamus, an lentiform nuclei in a 61-year-old man with a history of stroke, gait difficulties, and executive dysfunction with memory deficits. (B) Small deep infarcts are shown on fluid-attenuated inversion recovery images in association with diffuse a confluent white-matter hyperintensities involving the anterior part of the temporal lobes. (C) Microbleeds are visible on T2 or gradient-echo images as small hypointense foci in the thalamus and brainstem. CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. https://www.uptodate.com/contents/cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-cadasil/print 28/32 7/5/23, 12:35 PM Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) - UpToDate Reproduced from: Chabriat H, Joutel A, Dichgans M, et al. Cadasil. Lancet Neurol 2009; 8:643. Illustration used with the permission of Elsevier Inc. All rights reserved. Graphic 113184 Version 1.0 https://www.uptodate.com/contents/cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-cadasil/print 29/32 7/5/23, 12:35 PM Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) - UpToDate Brain MRI in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) Axial images of the brain with fluid-attenuated inversion-recovery (FLAIR) MRI in representative patients with biopsy-proved CADASIL show a typical pattern of brain lesions. Note the symmetry of the lesions and their extension into the superficial white matter (arrows). MRI: magnetic resonance imaging. Reprinted with permission from: Auer, D, Putz, B, Gossl, C, et al. Di erential Lesion Patterns in CADASIL and Sporadic Subcortical Arteriosclerotic Encephalopathy: MR Imaging Study with Statistical Parametric Group Comparison. Radiology 2001; 218:443. Copyright 2001 Radiological Society of North America. Graphic 60844 Version 3.0 https://www.uptodate.com/contents/cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-cadasil/print 30/32 7/5/23, 12:35 PM Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) - UpToDate Anterior temporal lobe involvement in CADASIL Brain MRI of a 46-year-old male with CADASIL and skin biopsy positive for granular osmiophilic material. There is hyperintensity in the periventricular and subcortical white matter on T2-weighted (A) and FLAIR (B) sequences, a nonspecific pattern that may be seen with other small vessel ischemic disease processes. In addition, there is increased signal intensity in the anterior temporal lobe white matter on T2-weighted (D) and FLAIR (E) sequences, a finding that may be more specific for CADASIL. Additional findings include a right periventricular lacunar infarction of CSF signal intensity (A, B, C) and involvement of the pons (D, E). CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy; MRI: magnetic resonance imaging; FLAIR: fluid-attenuated inversion recovery. Images courtesy of Eric Schwartz, MD. Graphic 81669 Version 3.0 https://www.uptodate.com/contents/cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-cadasil/print 31/32 7/5/23, 12:35 PM Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) - UpToDate Contributor Disclosures Martin Dichgans, MD Grant/Research/Clinical Trial Support: Bayern Innovativ, Gesellschaft f r Innovation und Wissenstransfer mbH [Stroke]; DFG/DLR [Stroke]; DZNE [Stroke]; European Union, Horizon 2020 [Stroke and dementia]; Foundation Leducq [Small vessel disease]; Intramural Funds Ludwig-Maximilians University [Stroke and dementia]; Josef-Hackl-Foundation [Clinical biomarkers for stroke]; National Institutes of Health [Early-onset stroke]; Vascular Dementia Research Foundation [Dementia]. Consultant/Advisory Boards: Ever Pharma [Stroke]. Speaker's Bureau: Bayer [Stroke]; Pfizer [Stroke]. All of the relevant financial relationships listed have been mitigated. Scott E Kasner, MD Grant/Research/Clinical Trial Support: Bayer [Stroke]; Bristol Meyers Squibb [Stroke]; Medtronic [Stroke]; WL Gore and Associates [Stroke]. Consultant/Advisory Boards: Abbvie [Stroke]; AstraZeneca [Stroke]; BMS [Stroke]; Diamedica [Stroke]; Medtronic [Stroke]. All of the relevant financial relationships listed have been mitigated. John F Dashe, MD, PhD No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-cadasil/print 32/32