University of Iowa Health Care

Ophthalmology and Visual Sciences

Photo 6

Q1: What clinical findings are present?

  • mild optic nerve pallor, central macular mottling, attenuated arterioles, and peripheral retinal granularity

Q2: A 6 year old female with previously normal vision presents with this fundus appearance and worsening vision over the past 6 months to 20/200. What diagnosis must be on your differential, and a deletion in what gene is responsible?

  • Batten disease (Juvenile Ceroid Lipofuscinosis); CNL3

Q3: What would an electroretinogram (ERG) likely show?

  • The ERG would likely be non-recordable

see the Photo Atlas Entry← this link will open a new window, close it to continue the quiz

Right Eye

Right eye

Left Eye

Left Eye

last updated: 9/18/2013
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