Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (
CADASIL) is a genetically transmitted
cerebrovascular disease. Typically, the first clinical manifestation is
migraine and the full clinical spectrum of the disease with recurrent
strokes of the subcortical type, cognitive, and
mood disorders is seen during the fourth and fifth decades of life. Vascular risk factors are usually absent in
CADASIL patients and the diagnosis of the disease is particularly suspected in young adults with cerebrovascular events of unknown cause, diffuse
leukoencephalopathy on computed tomography or magnetic resonance imaging, and a history of
cerebrovascular diseases or
dementia in many family members. We describe three Italian
CADASIL patients who presented to medical attention for cerebrovascular events occurred after the age of 55 and had, in addition to
hypertension and
hyperlipidemia, thrombophilic risk factors such as
hyperhomocysteinemia, elevated levels of
lipoprotein(a), and
antiphospholipid antibodies. Symptoms possibly related to cortical involvement, such as
dysphasia and visual field deficits, were reported by two of these patients. We conclude that a diagnosis of
CADASIL should not be disregarded in patients with vascular risk factors and presenting with symptoms not immediately referable to subcortical damage at ages more advanced than commonly reported.