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Start free with EleplanCerebral autosomal recessive arteriopathy-subcortical infarcts-leukoencephalopathy
ORPHA:199354Disease
Also called CARASIL · Maeda syndrome
What it is
CARASIL is a hereditary cerebral small vessel disease characterized by early-onset gait disturbances, premature scalp alopecia, ischemic stroke, acute mid to lower back pain and progressive cognitive disturbances leading to severe dementia.
Key facts
- Age of onset
- Adolescent, Adult
- Inheritance
- Autosomal recessive
- Classified as
- Disease
Signs and symptoms
Common30–79%
13Sometimes5–29%
16- Abnormal pyramidal sign
- Abulia
- Aggressive behavior
- Akinetic mutism
- Alopecia of scalp
- Apathy
- Dementia
- Diffuse cerebral atrophy
and 8 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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