Clinical and neuroimaging differences of cognitive impairment in CADASIL and dyscirculatory encephalopathy
Keywords:
CADASIL, dyscirculatory encephalopathy, cognitive impairment, MRI.Abstract
Introduction. CADASIL is a hereditary cerebral microangiopathy, whereas dyscirculatory encephalopathy represents chronic cerebrovascular insufficiency leading to cognitive impairment. The aim of this study was to comparatively evaluate the clinical and neuroimaging characteristics of cognitive impairment in patients with CADASIL and dyscirculatory encephalopathy.
Materials and Methods. A cross-sectional study included 106 participants: 31 patients with CADASIL, 45 patients with dyscirculatory encephalopathy, and 30 healthy controls. All participants underwent clinical and neurological examination, neuropsychological testing, and brain magnetic resonance imaging. White matter lesions were assessed using the Fazekas scale.
Results. SDMT scores in the CADASIL group were significantly lower than in the control group (33.8 ± 7.1 vs 48.6 ± 6.3; p < 0.001). In the dyscirculatory encephalopathy group, SDMT scores were 38.9 ± 8.4. White matter lesions of Fazekas grade ≥2 were detected in 74.2% of CADASIL patients and in 51.1% of patients with dyscirculatory encephalopathy (p = 0.03). Anterior temporal pole lesions were observed in 45.2% of CADASIL patients and external capsule lesions in 41.9%, whereas in the dyscirculatory encephalopathy group these findings were 13.3% and 17.8%, respectively (p < 0.05).
Conclusion. Cognitive impairment in CADASIL patients was more pronounced compared with dyscirculatory encephalopathy, and MRI more frequently revealed lesions in the anterior temporal pole and external capsule.
References
Livingston G, Huntley J, Sommerlad A, Ames D, Ballard C, Banerjee S, et al. Dementia prevention, intervention, and care: 2020 report of the Lancet Commission. The Lancet. 2020 Aug;396(10248):413–46. doi:10.1016/S0140-6736(20)30367-6
Wardlaw JM, Smith C, Dichgans M. Small vessel disease: mechanisms and clinical implications. The Lancet Neurology. 2019 Jul;18(7):684–96. doi:10.1016/S1474-4422(19)30079-1
Pantoni L. Cerebral small vessel disease: from pathogenesis and clinical characteristics to therapeutic challenges. The Lancet Neurology. 2010 Jul;9(7):689–701. doi:10.1016/S1474-4422(10)70104-6
Chabriat H, Joutel A, Dichgans M, Tournier-Lasserve E, Bousser MG. CADASIL. The Lancet Neurology. 2009 Jul;8(7):643–53. doi:10.1016/S1474-4422(09)70127-9
O’Sullivan M, Jarosz JM, Martin RJ, Deasy N, Powell JF, Markus HS. MRI hyperintensities of the temporal lobe and external capsule in patients with CADASIL. Neurology. 2001 Mar 13;56(5):628–34. doi:10.1212/WNL.56.5.628
Kandyba DV. DISCIRCGLATORY ENCEPHALOPATHY: HETEROGENEITY OF THEDEVELOPMENT OF CHRONIC BRAIN ISCHEMIA, MODERN APPROACHES TO THERAPY. Rossiĭskiĭ semeĭnyĭ vrach. 2012 Sep 15;16(3):4. doi:10.17816/RFD201234-13
Bos D, Wolters FJ, Darweesh SKL, Vernooij MW, De Wolf F, Ikram MA, et al. Cerebral small vessel disease and the risk of dementia: A systematic review and meta‐analysis of population‐based evidence. Alzheimer’s & Dementia. 2018 Nov;14(11):1482–92. doi:10.1016/j.jalz.2018.04.007
Yakubova MM, Rakhimova ShE, Saidakhmedova SK. New aspects of mri research in the differential diagnosis of binswanger disease. Global science research journals [Internet]. 2019;483-4857(7). Available from: https://www.globalscienceresearchjournals.org/
Hong H, Tozer DJ, Markus HS. Relationship of Perivascular Space Markers With Incident Dementia in Cerebral Small Vessel Disease. Stroke. 2024 Apr;55(4):1032–40. doi:10.1161/STROKEAHA.123.045857
Jokinen H, Kalska H, Ylikoski R, Madureira S, Verdelho A, Van Der Flier WM, et al. Longitudinal Cognitive Decline in Subcortical Ischemic Vascular Disease – The LADIS Study. Cerebrovasc Dis. 2009;27(4):384–91. doi:10.1159/000207442
