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April 1976

Retinal Changes in Neuronal Ceroid-Lipofuscinosis

Author Affiliations

Augusta, Ga

Arch Ophthalmol. 1976;94(4):687-688. doi:10.1001/archopht.1976.03910030343024

To the Editor.  —The article entitled "Ceroid-Lipofuscinosis (Batten Disease)" by Hittner and Zeller that appeared in the March issue of the Archives (93:178, 1975) was of great interest. I would like to share with your readers some supplemental information concerning the retinal lesions of the various forms of neuronal ceroid-lipofuscinosis (NCL).We have studied a large population of patients with NCL. This disorder comprises four major clinical (and possibly pathological) subtypes. A characteristic neuropathological finding in each subtype is excessive ceroid-lipofuscin accumulation in neurons. Clinically, three of the subtypes roughly correspond to the old classification of the amaurotic familial idiocies, ie, late infantile, juvenile, and adult.1,2 We have examined a 23-year-old patient with the rapidly progressive form that is usually of late infantile onset, and, therefore, we prefer to discard the age-dependent classification and to list these types as acute, chronic, and atypical. Exact clinical separation of these

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