Neuropsychiatric Manifestations in CADASIL

Overview

Journal Dialogues Clin Neurosci

Specialty Neurology

Date 2007 Aug 31

PMID 17726918

Citations 17

Authors

Hugues Chabriat

Marie-Germaine Bousser

Affiliations

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Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small-artery disease of mid-adulthood caused by mutations of the NOTCH3 gene. The disease is responsible for widespread white-matter lesions associated with lacunar infarctions in various subcortical areas. The disease is responsible for migraine with aura and ischemic strokes, and is associated with various degrees of cognitive impairment and with mood disturbances. CADASIL is considered as a unique model to investigate what is known as "subcortical ischemic vascular dementia." Recent data suggest that the number of lacunar infarctions and severity of cerebral atrophy are the main magnetic resonance imaging markers associated with cognitive and motor disabilities in this disorder. Mood disturbances are reported in 10% to 20% of patients, most often in association with cognitive alterations. Their exact origin remains unknown; the presence of ischemic lesions within the basal ganglia or the frontal white matter may promote the occurrence of these symptoms. Further studies are needed to better understand the relationships between cerebral lesions and both cognitive and psychiatric symptoms in this small-vessel disease of the brain.

Citing Articles

The prevalence of neuropsychiatric symptoms and correlation with MRI findings in CADASIL patients.

Bai L, Yan H, Guo Y, Shan Y, Peng Q, Jin H Ann Clin Transl Neurol. 2024; 11(11):3010-3018.

PMID: 39344629 PMC: 11572744. DOI: 10.1002/acn3.52214.

CADASIL: A NOTCH3-associated cerebral small vessel disease.

Yuan L, Chen X, Jankovic J, Deng H J Adv Res. 2024; 66:223-235.

PMID: 38176524 PMC: 11674792. DOI: 10.1016/j.jare.2024.01.001.

Cognition, mood and behavior in CADASIL.

Chabriat H, Lesnik Oberstein S Cereb Circ Cogn Behav. 2022; 3:100043.

PMID: 36324403 PMC: 9616390. DOI: 10.1016/j.cccb.2022.100043.

Investigating the Migraine Cycle over 21 Consecutive Days Using Proton Magnetic Resonance Spectroscopy and Resting-State fMRI: A Pilot Study.

Filippi V, Steiger R, Beliveau V, Frank F, Kaltseis K, Gizewski E Brain Sci. 2022; 12(5).

PMID: 35625032 PMC: 9139142. DOI: 10.3390/brainsci12050646.

CADASIL presenting as late-onset mania with anosognosia.

Uppal M, Kanellopoulos D, Kotbi N Clin Case Rep. 2020; 8(1):47-50.

PMID: 31998484 PMC: 6982508. DOI: 10.1002/ccr3.2594.

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