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NEUROLOGY 1978;28:710
© 1978 American Academy of Neurology

Atypical neuronal ceroidlipofuscinosis

ROBERT S. GREENWOOD, M.D. and JAMES S. NELSON, M.D.

Departments of Pediatrics, Neurology and Pathology, Division of Neuropalhology, Washington University School of Medicine, and the Division of Neurology, St. Louis Children's Hospital, St. Louis, Missouri.

We describe the clinical, pathologic, and ultrastructural findings in a case of juvenile onset neuronal ceroid-lipofuscinosis without visual symptoms or retinal abnormalities. The histochemical and ultrastructural characteristics of the neuronal lipopigment were similar to those in typical cases of neuronal ceroid-lipofuscinosis. Atypical neuronal ceroid-lipofuscinosis may be distinguished histochemically, ultrastructurally, and clinically from another disorder called juvenile dystonic lipidosis, with which it has been confused.

Reprint requests should be addressed lo Dr. Nelson, St. LOUIS Chil dren's Hospital. 500 South Kingshighway, St. Louis, MO 63110.

This study was supported in part by USPHS Grant NS11277 and by the Allen P. and Josephine 6. Green Foundation, Mexico, Missouri.

Accepted for publication July 8, 1977.




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