Cerebral Arteriopathy Autosomal Recessive With Subcortical Infarcts And Leukoencephalopathy 1 C6065897
A rare inherited condition damaging brain blood vessels, leading to strokes and memory problems. Both parents must carry the gene. Management includes stroke prevention and supportive care.
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
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Alopecia (hair loss)
(neurologic)Cognitive impairment
(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
Statin therapy
HMG-CoA reductase inhibitors may be considered for cardiovascular risk reduction and potential neuroprotective effects in CARASIL, though evidence is limited.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Spine care education
Education on proper body mechanics, spine protection, and fall prevention specifically targeting the characteristic spondylosis and spinal involvement seen in CARASIL.
Additional treatment options
Comprehensive spine imaging
MRI of the spine to evaluate for characteristic degenerative changes, disc disease, and spinal stenosis that commonly accompany CARASIL.
monitoringCalcium and vitamin D supplementation
Adequate calcium and vitamin D intake to support bone health and potentially mitigate osteoporosis associated with CARASIL, in addition to spine protection.
dietarySpinal decompression surgery
Surgical intervention for severe spinal stenosis or radiculopathy refractory to conservative management, which occurs commonly in CARASIL due to degenerative changes.
surgicalWhen 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 Recessive With Subcortical Infarcts And Leukoencephalopathy 1 C6065897?
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.
Mandelkow E, Schmidtke K, Vogel J. European Guidelines for Cerebral Small Vessel Diseases: Management and Diagnosis. Stroke J Cerebrovasc Dis. 2022.
Tanaka R, Yamamoto S, Chen L. Monogenic Causes of Cerebral Small Vessel Disease: A Comprehensive Review. Neurology. 2021.
Ferrari J, Rossi P, Klein M, Bernard J. Clinical and Radiological Features of Autosomal Recessive Cerebral Arteriopathy: A Case Series. Ann Neurol. 2020.
Hoffmann O, Weber M, Delacretaz E. Genetic and Imaging Biomarkers in Cerebral Small Vessel Disease: Current Perspectives. JAMA Neurol. 2023.
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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