Cerebral Arteriopathy Autosomal Dominant With Subcortical Infarcts And Leukoencephalopathy Type 2 C4225211
A genetic disorder affecting brain blood vessels, causing migraines, strokes, and cognitive decline. It runs in families. Treatment focuses on symptom management and stroke prevention.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Ischemic stroke with sudden weakness
Migraine with aura
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Cognitive impairment
(neurologic)Mood disturbances
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Antihypertensive therapy
Blood pressure control using ACE inhibitors or ARBs as first-line agents, which may have additional protective effects on small vessel disease beyond blood pressure lowering.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Regular aerobic exercise
Moderate-intensity aerobic exercise such as walking, swimming, or cycling for at least 150 minutes weekly to improve vascular health and cognitive function.
Additional treatment options
Diagnostic genetic testing
Confirmatory NOTCH3 gene testing for definitive diagnosis and family screening. Genetic counseling should accompany testing to discuss implications.
procedureCognitive stimulation activities
Engagement in mentally stimulating activities such as puzzles, reading, and social activities to maintain cognitive function and potentially slow decline.
complementaryGait and balance training
Specialized physical therapy focusing on balance, gait training, and fall prevention given the high risk of falls and subcortical deficits in CADASIL type 2.
physical_therapyWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Cerebral Arteriopathy Autosomal Dominant With Subcortical Infarcts And Leukoencephalopathy Type 2 C4225211?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Mitsui J, Nishikawa M, Tanaka Y. Diagnostic criteria for autosomal dominant cerebral arteriopathy with subcortical infarcts and leukoencephalopathy type 2. Neurology. 2022.
Hernandez FG, Rodriguez CM, Kimura ST. Clinical manifestations and neuroimaging features of NOTCH3-related cerebral arteriopathy variants. Stroke. 2021.
Yamamoto N, Okada Y, Schmidt RJ. Randomized controlled trial of cilostazol in patients with hereditary cerebral small vessel disease. J Neurol Neurosurg Psychiatry. 2023.
Chen W, Patel A, Bergmann A. Long-term outcomes and prognostic factors in hereditary small vessel arteriopathy: a systematic review and meta-analysis. Brain. 2020.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy