Hereditary Multi-Infarct Dementia (CADASIL)

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a NOTCH3 mutation causing a hereditary small-vessel arteriopathy. Clinical: recurrent migraine with aura, subcortical strokes/TIAs, mood disorders, and progressive vascular dementia in mid-adulthood; no hypertension requirement. MRI shows anterior-temporal/external-capsule white-matter changes. There is no cure; supportive stroke prevention, migraine/mood management, and genetic counseling.

Common Symptoms

Migraine with aura; recurrent subcortical strokes; mood depression/apathy; progressive vascular dementia; family history

Causes and Pathogenesis

NOTCH3 mutation causes granular osmiophilic material in small penetrating arteries, leading to subcortical infarcts and leukoencephalopathy.

Treatment

Supportive; avoid smoking/hypertension; migraine/mood management; antiplatelet used cautiously; genetic counseling

Preventive Measures

Family NOTCH3 screening; avoid vascular risk factors; careful antithrombotic use.

Department to Visit

It is recommended to visit the Neurology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.