Results 11 to 20 of about 3,826 (158)

Association of imaging-defined brain age with disease severity and adverse outcomes in CADASIL. [PDF]

Alzheimers Dement
Abstract INTRODUCTION Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by cysteine‐altering NOTCH3 variants. We examined whether neuroimaging‐defined brain age is altered in CADASIL and its association with disease severity and outcomes.
Hsu SL, Lee PL, Chou KH, Kuo CY, Lin CP, Liao YC, Chung CP, Lee YC.   +7 more
europepmc   +2 more sources

NOTCH3 CADASIL Variant Receptor Aggregation Requires NOTCH3 Wild-Type Receptors: Identification of Highly Selective Inhibitors That Block the Process. [PDF]

FASEB J
CADASIL is the major cause of early‐onset stroke and cognitive dysfunction, including dementia. It is caused by mutations in the NOTCH3 receptor that result in the formation of protein aggregates in the small vessel walls of the brain. We demonstrated that NOTCH3 CADASIL variant receptor aggregation is strictly dependent on interactions with NOTCH3 ...
Wang H, Liu X, Gravesteijn G, Atella A, Liu J, Rutten JW, Ten Dijke P, Gonçalves MAFV, Lesnik Oberstein SAJ, Baker DA.   +9 more
europepmc   +2 more sources

Management of Coronary Artery Disease in CADASIL Patients: Review of Current Literature

Medicina, 2023
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common heritable form of vascular dementia in adults.
Maria Servito, Isha Gill, Joshua Durbin, Nader Ghasemlou, Aron-Frederik Popov, Christopher D. Stephen, Mohammad El-Diasty   +6 more
doaj   +1 more source

Effect of corticosubcortical iron deposition on dysfunction in CADASIL is mediated by white matter microstructural damage

NeuroImage: Clinical, 2023
Iron dysregulation may attenuate cognitive performance in patients with CADASIL. However, the underlying pathophysiological mechanisms remain incompletely understood. Whether white matter microstructural changes mediate these processes is largely unclear.
Xiuqin Jia, Yingying Li, Yunqing Ying, Xuejia Jia, Weijun Tang, Yueyan Bian, Jiajia Zhang, Danny J.J. Wang, Xin Cheng, Qi Yang   +9 more
doaj   +1 more source

The first report of CADASIL in Peru: Olfactory dysfunction on initial presentation

eNeurologicalSci, 2016
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is a rare, heritable, small vessel vascular disease caused by mutations in the Notch3 gene that is characterized by migraines, subcortical vascular ...
Anastasia Vishnevetsky, Miguel Inca-Martinez, Karina Milla-Neyra, Danny Moises Barrientos-Iman, Ivan Cornejo-Herrera, Carlos Cosentino, Mario Cornejo-Olivas   +6 more
doaj   +1 more source

Microglial reactivity predicts hippocampal, but not global, atrophy in cerebral small vessel disease. [PDF]

Alzheimers Dement
Abstract INTRODUCTION Cerebral small vessel disease (CSVD) is the most prevalent pathology underlying vascular dementia. Increased neuroinflammation and blood‐brain barrier (BBB) permeability have been implicated in CSVD pathogenesis. We determined whether microglial reactivity and BBB permeability at baseline predicted whole‐brain and hippocampal ...
Zainurin A, Brown RB, Tozer DJ, Markus HS.   +3 more
europepmc   +2 more sources

A Case of CADASIL with NOTCH3 Gene Mutation Presenting with Focal Epileptic Seizure: A Case of CADASIL Presenting with Focal Epileptic Seizure

Archives of Epilepsy, 2023
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a hereditary disease of cerebral microvessels with autosomal dominant inheritance due to the NOTCH3 gene mutation. Epileptic seizures were observed in
Gülgün Uncu, Demet İlhan Algın, Oğuz Osman Erdinç, Demet Özbabalık Adapınar   +3 more
doaj   +1 more source

Is the oxidant/antioxidant status altered in CADASIL patients?

PLoS ONE, 2013
The altered aggregation of proteins in non-native conformation is associated with endoplasmic reticulum derangements, mitochondrial dysfunction and excessive production of reactive oxygen species. Cerebral autosomal dominant arteriopathy with subcortical
Jonica Campolo, Renata De Maria, Caterina Mariotti, Chiara Tomasello, Marina Parolini, Marina Frontali, Domenico Inzitari, Raffaella Valenti, Antonio Federico, Franco Taroni, Oberdan Parodi   +10 more
doaj   +1 more source

Lifelong cerebrovascular disease burden among CADASIL patients: analysis from a global health research network

Frontiers in Neurology, 2023
IntroductionData reporting on patients with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) within the United States population is limited.
Alan P. Pan, Thomas Potter, Abdulaziz Bako, Jonika Tannous, Sudha Seshadri, Louise D. McCullough, Farhaan S. Vahidy, Farhaan S. Vahidy, Farhaan S. Vahidy   +8 more
doaj   +1 more source

On the Diagnosis of CADASIL

Journal of Alzheimer's Disease, 2009
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a genetic arteriopathy related to Notch3 mutations, is difficult to diagnosis. The goal of this study was to determine the value of clinical, immunohistochemical, and molecular techniques for the diagnosis of CADASIL.
Ampuero, I, Alegre-Abarrategui, J, Rodal, I, España, A, Ros, R, Sendón, J, Galloway, E, Cervelló, A, Caminero, AB, Zabala, A, Erro, E, Jarauta, F, Morlán, L, López-Valdés, E, Aladro, Y, Seijo, M, Rivas, G, Muñoz, D, de Yébenes, J   +18 more
openaire   +2 more sources