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Ceroid-Lipofuscinosis (Batten Disease)Fluorescein Angiography, Electrophysiology, Histopathology, Ultrastructure, and a Review of Amaurotic Familial Idiocy
Helen M. Hittner, MD;
Robert S. Zeller, MD
Arch Ophthalmol. 1975;93(3):178-183.
Abstract
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.
Author Affiliations
From the departments of ophthalmology (Dr. Hittner), pediatrics (Drs. Hittner and Zeller), and neurology (Dr. Zeller), Baylor College of Medicine, Houston.
Footnotes
Submitted for publication Dec 17, 1973.
Read in part before the Ninth Annual Alumni Meeting of the Department of Ophthalmology, Baylor College of Medicine, Houston, June 23, 1972.
Reprint requests to 2415 Gramercy, Houston, TX 77025 (Dr. Hittner).
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