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Patterns of MRI lesions in CADASIL
H Chabriat1, C Levy, H Taillia
1Service de Neurologie, Hôpital Lariboisière, Paris, France.
Neurology
|August 26, 1998
Summary
MRI abnormalities in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) are common, particularly in white matter and basal ganglia. Lesion severity increases with age and is higher in symptomatic patients.
Area of Science:
- Neurology
- Radiology
- Genetics
Background:
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited condition characterized by MRI signal abnormalities.
- These abnormalities are linked to Notch 3 gene mutations and are present in both symptomatic and asymptomatic individuals.
Purpose of the Study:
- To determine the location and severity of MRI signal abnormalities in patients with CADASIL.
- To correlate MRI findings with clinical symptoms and age.
Main Methods:
- Review of MRIs from 75 patients (43 symptomatic) by a masked neuroradiologist.
- Assessment of T1- and T2-weighted image lesions in subcortical regions (white matter, basal ganglia, infratentorial areas).
- Severity scoring of hyperintensities using global and regional scales.
Main Results:
- 90% of patients showed white matter hyperintensities, predominantly in periventricular (96%) and deep white matter (85%).
- Basal ganglia (60%) and brainstem (45%) also showed abnormalities; 62% had T1 hypointensities.
- Lesion frequency and severity increased with age and were higher in symptomatic patients; diffuse white matter abnormalities correlated with dementia or high Rankin scores.
Conclusions:
- Subcortical areas exhibit varying vulnerability to ischemia in CADASIL.
- Age is associated with lesion accumulation, suggesting disease progression.
- Further prospective studies are needed to assess the prognostic value of MRI lesion ratings in CADASIL.