[CADASIL with minimal symptoms after 60 years].
BACKGROUND
CADASIL is a hereditary cerebral arteriopathy leading to progressive disability and dementia usually observed at 60 years.


METHODS
We report four patients aged>60 years with typical Notch3 mutations leading to CADASIL who did not have dementia or disability. Three of them presented with only transient neurological manifestations. MRI results showed extensive hyperintense signals in the white-matter on T2-weighted images contrasting with very few lacunar infarcts.


CONCLUSIONS
These observations suggest that silent or symptomatic infarcts, which were rare in the present cases may be responsible for the clinical severity in this disorder.
Mourad
A
A
Service de Neurologie CHG, Place du Général Leclerc, Orsay.
Levasseur
M
M
Bousser
M G
MG
Chabriat
H
H
D000368:Aged; D046589:CADASIL / Q000503:physiopathology* / Q000523:psychology; D003072:Cognition Disorders / Q000209:etiology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged