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—Dr Dyken's description of four major clinical subtypes of neuronal ceroid-lipofuscinosis is very informative and indicates the vast experience that he has in this field. His comments are much appreciated.I would like to add that a major advance in research in this field has just been reported by Wolfgang Zeman, MD, of the Department of Pathology at Indiana State University. Dr Zeman has discovered the enzyme defect (peroxidase deficiency) in neuronal ceroid-lipofuscinosis, which is of great importance in the diagnosis of affected persons, the detection of carriers, and the prevention of the disease by amniocentesis and abortion.
Hittner HM. Retinal Changes in Neuronal Ceroid-Lipofuscinosis-Reply. Arch Ophthalmol. 1976;94(4):688. doi:10.1001/archopht.1976.03910030343025