Pavlovic, Aleksandra M. (7003808508)Aleksandra M. (7003808508)PavlovicDobricic, V. (22952783800)V. (22952783800)DobricicSemnic, R. (6701842753)R. (6701842753)SemnicLackovic, V. (35754725400)V. (35754725400)LackovicNovakovic, I. (6603235567)I. (6603235567)NovakovicBajcetic, M. (24830364600)M. (24830364600)BajceticSternic, N. (6603691178)N. (6603691178)Sternic2025-06-122025-06-122013https://doi.org/10.1007/s13760-012-0174-2https://www.scopus.com/inward/record.uri?eid=2-s2.0-84883393935&doi=10.1007%2fs13760-012-0174-2&partnerID=40&md5=3c2688e68142668e6d2525fe7829d55chttps://remedy.med.bg.ac.rs/handle/123456789/9262Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common heritable cause of stroke and vascular dementia in adults. We present a family from Serbia presenting with stroke and depression in the lack of vascular risk factors, with brain MRI indicating CADASIL. A novel NOTCH3 Gly89Cys mutation was located in exon 3. This report illustrates that in the setting of a positive family history with typical clinical and MRI features, even with an atypical form of pedigree, a high suspicion of CADASIL should lead to genetic testing. © 2013 Belgian Neurological Society.CADASILExon 3Notch3StrokeWhite matter lesionsA novel Notch3 Gly89Cys mutation in a Serbian CADASIL family