Short answer · Medically reviewed summary · Last updated: 2026-05-08
The primary ICD-10-CM code for CADASIL (Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy) is I67.850 (Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy). Under the older ICD-9-CM system, there was no specific code for the condition, and it was typically classified under 437.8 (Other specified cerebrovascular diseases). Why are medical codes for CADASIL important? Accurate medical coding for CADASIL is essential for ensuring that patients receive appropriate insurance coverage and that clinical data is tracked correctly in health registries.
ICD10 code of Cadasil (Cerebral Autosomal Dominant Arteriopathy With Sub-Cortical Infarcts And Leukoencephalopathy) and ICD9 code
ICD-10 and ICD-9 codes for Cadasil (Cerebral Autosomal Dominant Arteriopathy With Sub-Cortical Infarcts And Leukoencephalopathy), with classification details for clinicians, coders and patients.
The primary ICD-10-CM code for CADASIL (Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy) is I67.850 (Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy). Under the older ICD-9-CM system, there was no specific code for the condition, and it was typically classified under 437.8 (Other specified cerebrovascular diseases).
Why are medical codes for CADASIL important?
Accurate medical coding for CADASIL is essential for ensuring that patients receive appropriate insurance coverage and that clinical data is tracked correctly in health registries. Because CADASIL is a rare genetic disorder, using the specific ICD-10 code helps clinicians and researchers distinguish it from more common causes of stroke or dementia, facilitating better longitudinal care for the 57 community members currently sharing their experiences on DiseaseMaps.org.
What is the clinical nature of CADASIL?
CADASIL is a hereditary small-vessel disease caused by mutations in the NOTCH3 gene on chromosome 19. The condition typically presents in mid-adult life and is characterized by the following clinical hallmarks:
- Recurrent subcortical ischemic strokes or transient ischemic attacks (TIAs).
- Early-onset cognitive impairment or vascular dementia.
- Migraines with aura, often occurring as the earliest symptom.
- Psychiatric disturbances, including severe depression and apathy.
- Characteristic white matter hyperintensities visible on MRI scans.
How is a diagnosis of CADASIL confirmed?
While ICD-10 code I67.850 identifies the diagnosis, definitive confirmation of CADASIL requires genetic testing to identify a pathogenic variant in the NOTCH3 gene. In some cases, a skin biopsy may be performed to look for characteristic granular osmiophilic material (GOM) deposits in the small blood vessels, though genetic testing remains the gold standard for diagnosing CADASIL.
Next steps
- Consult with a neurologist specializing in neurovascular disorders or a clinical geneticist.
- Request a brain MRI to evaluate for the characteristic leukoencephalopathy associated with CADASIL.
- Connect with the 57 members of the CADASIL community at DiseaseMaps.org to share resources and experiences.
- Discuss genetic counseling for family members, as the condition follows an autosomal dominant inheritance pattern.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): CADASIL.
- Orphanet: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (ORPHA:139).
- OMIM (Online Mendelian Inheritance in Man): #125190 (CADASIL).
- ICD-10-CM Browser (CDC/NCHS): Code I67.850.
