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Best vitelliform macular dystrophy
1 OMIM reference -
1 associated gene
5 connected diseases
6 signs/symptoms
Disease Type of connection
Adult-onset foveomacular vitelliform dystrophy
Autosomal dominant vitreoretinochoroidopathy
MRCS syndrome
Retinitis pigmentosa
Retinopathy, Burgess-Black type
Synonym(s):
- BMD
- BVMD
- Best disease
- Best macular dystrophy
- Early-onset vitelliform macular dystrophy
- Juvenile-onset vitelliform macular dystrophy
- Polymorphic vitelline macular degeneration
- Vitelliform macular dystrophy type 2
Classification (Orphanet):
- Rare eye disease
- Rare genetic disease
Classification (ICD10):
- Diseases of the eye and adnexa -
Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: childhood
Average age of death: normal
Type of inheritance: autosomal dominant
External references:
1 OMIM reference -
No MeSH references
Gene symbol UniProt reference OMIM reference
BEST1 O76090607854
Very frequent
- Autosomal dominant inheritance
- Macular dystrophy / absence / hypoplasia of the macula
- Mild visual loss / impaired visual acuity

Frequent
- Achromatopsia / dyschromatopsia / daltonism / impaired colour vision

Occasional
- Abnormal visual field / hemianopsia / hemianopia / scotoma / visual peripheral rim
- Choroidal anomalies / atrophy / choroideremia