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March 1975

Ceroid-Lipofuscinosis (Batten Disease): Fluorescein Angiography, Electrophysiology, Histopathology, Ultrastructure, and a Review of Amaurotic Familial Idiocy

Arch Ophthalmol. 1975;93(3):178-183. doi:10.1001/archopht.1975.01010020186003

Three children with ceroid-lipofuscinosis and their mother were investigated fluorescein angiographically and electrophysiologically after definitive diagnosis of the oldest child had been made from a brain biopsy specimen studied biochemically, histopathologically, and ultrastructurally. The diagnostic features of the two classes of familial amaurotic idiocy (the gangliosidoses and the ceroid-lipofuscinoses) are reviewed with emphasis on the importance of the fundus picture and fluorescein angiographic study in differentiating the two classes of disease and in identifying affected siblings.