Batten Disease

Batten Disease

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7-year-old female with molecularly confirmed CLN3-associated Batten Disease.

Fundus photos demonstrate mild optic nerve pallor, central macular mottling, attenuated arterioles and peripheral retinal granularity. There was 1+ vitreous cell present. Vision was 20/800 in the right eye and hand motion in the left. Batten disease is a fatal autosomal recessive neurodegenerative disease and is the most common neuronal ceroid lipofuscinosis.

OMIM #204200

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Bullseye maculopathy in Batten Disease

Bullseye maculopathy in Batten Disease

Category(ies): Inherited Eye Diseases, Retina / Vitreous

Contributor: Ella J. Gehrke, MD; Arlene V. Drack, MD

Photographer: Douglas B. Critser, CRA, OCT-C, FOPS

Posted: 02/19/2025

Fundus photo demonstrates flat, dark pink disc with bullseye maculopathy. Vision at time of diagnosis was 20/40 OU BCVA in a 9-year-old female. Bullseye maculopathy associated with Batten disease at time of first presentation.