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Case Report

A Case of Late Infantile Batten's Disease

Kyung-Tae Whang, Jong-Wan Kim, Young-Hoon Kim, Seung-Yun Chung, In-Goo Lee, Je-Geun Chi

Clin Exp Pediatr. 1994;37(12):1745-1751. Published online December 15, 1994

We experienced a case of late infantile Batten's disease in a 4-year-7-month-old boy who was admitted to child neurology service of Kangnam St. Mary's hospital for evaluation of progressive psychomotor deterioration. He was in quit normal state of development until 3 years of age when his mother first became concerned because he showed such emotional change as crying and fear. Since...

A Case of Neuronal Ceroid-Lipofuscinosis with characterstic photic evoked spikes in EEG

Joo Hyun Han, Jae Won Oh, Jae Hoon Shin, In Joon Seol, Young Hyae Koh, Moon Hyang Park

Clin Exp Pediatr. 1992;35(2):252-256. Published online February 15, 1992

We experienced a case of neuronal ceroid lipofuscinosis in a 1 year old female child. Her chief complaints on admission were retarded growth and development, recurrent seizures and hypotonia. Electroencephalographic examination showed very characteristic photic responses, so called, photic evoked spikes which are synchronous with the flashes at 5 flashes per second. Final diagnosis was made with electron microscopic examination of...

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