Vascular risk control
Aggressive blood pressure, lipid, glucose, and smoking management — the highest-yield intervention in CADASIL care.
Care that changes outcomes
Treatment & management
There is no disease-modifying therapy for CADASIL yet. But the gap between great care and average care in this disease is large — and it is built from clear, well-defined choices.
Five domains of CADASIL care.
Excellent CADASIL care is multidisciplinary by definition. These five domains form the backbone of every individual care plan.
Migraine management
Targeted prevention and acute treatment, with appropriate caution around vasoactive options.
Cognition & mood
Routine screening, treatment of depression and apathy, cognitive rehabilitation, and selective use of pharmacotherapy.
Lifestyle & rehabilitation
Exercise, sleep, nutrition, social engagement, physical and occupational therapy where indicated.
Monitoring & surveillance
Periodic clinical and imaging follow-up — calibrated to the individual's age, severity, and goals.
Family & psychosocial
Genetic counselling, support for partners and caregivers, family planning, and peer connection.
Vascular risk factor management
If we could pick a single lever, this would be it. Aggressive control of vascular risk factors is the most evidence-informed, highest-impact intervention in CADASIL care.
Hypertension
Tight blood pressure control is the single most impactful target. Targets generally align with prevailing stroke-prevention guidelines, individualized by age and tolerance. Avoid permissive hypertension when possible.
Smoking
Smoking is consistently associated with worse imaging and clinical outcomes in CADASIL cohorts. Cessation is non-negotiable. Support with counselling, NRT, and pharmacotherapy as appropriate.
Lipids
Statin therapy is reasonable in line with general stroke-prevention guidelines, particularly in patients with established cerebrovascular disease.
Diabetes & weight
Glycemic control and metabolic health are central. Encourage Mediterranean-style or DASH dietary patterns where feasible.
Migraine management in CADASIL
Migraine — particularly migraine with aura — is often the earliest and most disruptive symptom. Effective management improves quality of life and may reduce ER visits for confused stroke-mimic presentations.
Acute treatment
- Standard non-vasoactive analgesics (acetaminophen, NSAIDs) where tolerated
- Triptans are not absolutely contraindicated, but caution is warranted given their vasoconstrictive profile and the underlying small-vessel arteriopathy. Decisions should be individualized in consultation with a CADASIL-aware clinician.
- Ergot alkaloids are generally avoided.
- Newer CGRP-targeted acute therapies (gepants) may be a reasonable consideration in selected patients.
Preventive treatment
- Standard preventive options — including beta-blockers, candesartan, topiramate, and selected antidepressants — are typically appropriate.
- CGRP-targeted preventive therapies (monoclonal antibodies, gepants) may have a role; small-cohort data are reassuring but more evidence is needed.
- Lifestyle and trigger management are foundational.
Stroke vs. complex aura
Patients with CADASIL frequently present to the ER with prolonged or atypical aura that is difficult to distinguish from acute ischemia. Imaging, careful examination, and CADASIL-aware decision-making are essential. Communicate the diagnosis early to ER teams.
Antithrombotic decisions
Decisions around antiplatelet and anticoagulant therapy in CADASIL must balance ischemic stroke risk against the recognized risk of intracerebral hemorrhage from microbleeds and cerebral amyloid-like vasculopathy.
- Antiplatelet therapy (typically aspirin) is commonly used after an ischemic event in CADASIL, individualized to bleeding risk and microbleed burden.
- Dual antiplatelet therapy is generally avoided long-term given hemorrhage concerns.
- Anticoagulation is reserved for clear indications (e.g., atrial fibrillation), with careful weighing of bleeding risk.
- IV thrombolysis in the setting of acute stroke in a patient with known CADASIL is a nuanced decision — local stroke teams should coordinate with neurology and consider published case series.
Important nuance
Antithrombotic decisions in CADASIL should always be made by experienced clinicians, with imaging review and shared decision-making with the patient. Recommendations evolve as evidence accumulates.
Cognition, mood, and rehabilitation
Cognitive symptoms
Depression and apathy are highly responsive to treatment in many patients. SSRIs are commonly used; behavioural strategies are critical for apathy specifically.
Cholinesterase inhibitors (e.g., donepezil) have been studied in CADASIL with mixed results — they did not demonstrate benefit on the primary global cognitive endpoint in the major randomized trial but may offer modest improvement on executive function in selected patients. Use is individualized.
Cognitive rehabilitation, structured cognitive activity, and management of sleep disorders all contribute meaningfully to function.
Mood, apathy, anxiety
These are often under-recognized. Routine screening (e.g., PHQ-9 + apathy scale) at follow-up visits is encouraged. Treat aggressively when identified.
Rehabilitation
Stroke rehabilitation principles apply — physical therapy, occupational therapy, speech-language pathology, and gait training as needed. Falls prevention becomes increasingly important with disease progression.
What patients can do — every day.
Move
Regular aerobic activity, ideally 150 minutes/week, supports vascular and cognitive health.
Eat for the brain
Mediterranean / DASH-style eating patterns are well-aligned with cerebrovascular health.
Sleep
Sleep apnea screening matters. Treat sleep disorders aggressively — they amplify vascular and cognitive risk.
Drink less
Limit alcohol; it is a recognized vascular risk factor and migraine trigger.
Stay engaged
Social, cognitive, and emotional engagement protect function. Isolation accelerates decline.
Plan ahead
Advance directives, financial planning, and legal arrangements are easier when made early — and bring peace of mind.
Drugs and procedures that warrant special caution.
- Cerebral angiography — historically associated with CADASIL-specific complications; modern non-invasive imaging (MRA, CTA) is generally preferred when feasible.
- IV thrombolytics in acute stroke — coordinate with stroke specialists and consider published case-series data.
- Triptans and ergots — discussed above.
- Hypotensive episodes — perioperative or intraoperative — should be avoided where possible.
- Hormonal contraception — discuss thrombotic and migraine risk individually with a CADASIL-aware clinician.