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Start free with EleplanSusac syndrome
ORPHA:838Disease
Also called Retinocochleocerebral vasculopathy
What it is
A rare systemic or rheumatologic disease characterized by the triad of central nervous system (CNS) dysfunction, branch retinal artery occlusions (BRAOs) and sensorineural hearing loss (SNHL) due to autoimmune-mediated occlusions of microvessels in the brain, retina, and inner ear.
Key facts
- Age of onset
- All ages
- Inheritance
- Unknown
- Classified as
- Disease
Signs and symptoms
Common30–79%
6- Abnormal corpus callosum morphology
- Cognitive impairment
- Confusion
- Generalized hypotonia
- Sensorineural hearing impairmentDiagnostic criterion
- Visual lossDiagnostic criterion
Sometimes5–29%
16- Abnormal emotion/affect behavior
- Abnormality of eye movement
- Apathy
- Atypical behavior
- Diplopia
- Dysarthria
- Gait ataxia
- Lethargy
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.
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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