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CADASIL and Cerebral Small-Vessel Disease

Hereditary cerebral small-vessel disease caused by NOTCH3 mutations.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is CADASIL and Cerebral Small-Vessel Disease?

CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy) is a hereditary small-vessel disease caused by NOTCH3 mutations and characterized by deposition of granular osmiophilic material in the walls of small arterioles.

Beginning with migraine attacks in early adulthood, it can progress to lacunar strokes, cognitive decline, and subcortical dementia.

MRI shows characteristic white-matter changes, especially hyperintensity in the anterior temporal lobes and external capsule.

Symptoms

Migraine attacks with aura beginning in early adulthood
Recurrent lacunar strokes
Progressive cognitive decline
Mood disorders and depression
Gait disturbances
Executive-function and attention problems
Progressive subcortical dementia

Risk Factors

Family history of NOTCH3 mutation
Early stroke in a parent
Coexistence of migraine and stroke
Additional vascular risk factors (hypertension, smoking)
Family history of unexplained subcortical dementia

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Recurrent severe migraine with aura at a young age
  • Family history of early-onset stroke
  • Unexplained cognitive decline
  • Recurrent lacunar stroke
  • Depression and mood swings
  • Need for genetic counseling and testing

Treatment Methods

01
Diagnosis confirmed by NOTCH3 gene analysis
02
Migraine prophylaxis and cautious use of triptans during attacks
03
Aggressive control of vascular risk factors
04
Evaluation for antiplatelet therapy
05
Cognitive rehabilitation and cholinesterase inhibitors
06
SSRIs for depression
07
Genetic counseling for family members

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.