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  Vol. 94 No. 4, April 1976 TABLE OF CONTENTS
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Retinal Changes in Neuronal Ceroid-Lipofuscinosis

Paul R. Dyken, MD
Augusta, Ga

Arch Ophthalmol. 1976;94(4):687-688.

Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

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 . . . [Full Text PDF of this Article]



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