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BENIGN CONCENTRIC ANNULAR MACULAR DYSTROPHY [1 record]

Record 1 2025-08-27

English

Subject field(s)
  • Visual Disorders
CONT

Benign concentric annular macular dystrophy (BCAMD) is a progressive autosomal dominant macular dystrophy characterized by parafoveal hypopigmentation followed by a retinitis pigmentosa-like phenotype (nyctalopia and peripheral vision loss) with a bull's eye configuration.

OBS

Although the macular dystrophy was initially termed "benign," a follow-up examination of the original cases revealed evidence of progression of the condition. A progressive decrease in visual acuity, nyctalopia and dyschromatopsia were found in some of the original family members with the macular dystrophy 10 years after the original description of the condition. An increase in pigmentary maculopathy and peripheral bone corpuscular changes were also observed.

French

Domaine(s)
  • Troubles de la vision
OBS

[La dystrophie maculaire annulaire concentrique bénigne] était au départ considérée comme bénigne, cependant la reprise des cas princeps a montré que la maculopathie évoluait de façon défavorable [...] et qu'il apparaissait en périphérie des pigmentations ostéoblastiques.

OBS

La dystrophie maculaire annulaire concentrique est transmise de façon autosomique dominante.

Spanish

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