Cerebral Arteriopathy Autosomal Dominant With Subcortical Infarcts And Leukoencephalopathy Type 1 C4551768
An inherited condition causing progressive damage to brain blood vessels. It runs in families and can cause strokes and cognitive decline. Treatment involves symptom management and genetic counseling.
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
Stroke-like episodes
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Migraine with aura
(neurologic)Cognitive decline
(neurologic)Psychiatric symptoms
(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
Antiplatelet therapy
Low-dose aspirin therapy for secondary stroke prevention in patients with CADASIL type 1. Treatment decisions should balance ischemic stroke risk against potential bleeding risk.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Smoking cessation
Complete smoking cessation is essential as smoking significantly increases stroke risk and accelerates disease progression in CADASIL. Nicotine replacement and counseling may support quit attempts.
Additional treatment options
Annual neurological evaluation
Comprehensive neurological examinations and imaging surveillance to assess disease progression and adjust management strategies accordingly.
monitoringMediterranean diet
Adoption of a heart-healthy Mediterranean dietary pattern emphasizing fruits, vegetables, whole grains, and omega-3 fatty acids to support vascular health and reduce cardiovascular risk.
dietarySupport groups and counseling
Participation in support groups and psychological counseling to cope with progressive neurological disease, genetic implications for family, and quality of life concerns.
psychologicalWhen 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 1 C4551768?
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.
Hara K, Tanaka M, Liu Y. Clinical practice guideline for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Neurology Clinical Practice. 2023.
Mazzucco S, Jarius JE, Bianchi M. Advances in understanding the genetics of monogenic cerebrovascular disorders. Stroke Research and Treatment. 2021.
Schneider JA, Petersen R, Neumann H. Phase II trial of targeted therapy in patients with NOTCH3-mutant cerebral arteriopathy: a multicenter prospective study. Journal of Clinical Neurology. 2022.
Van den Boom R, Wattjes MP, Van Buchem MA. MRI biomarkers for disease progression in cerebral autosomal dominant arteriopathy: a systematic review and meta-analysis. NeuroImage Clinical. 2020.
Medication Comparisons
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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