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Daniel E. Hale, MD
Children's Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104

Robert P. Cruse, DO
Cleveland Clinic Foundation 9500 Euclid Ave Cleveland, OH 44106

Andrew Engel, MD
Mayo Clinic 200 First St SW Rochester, MN 55905

Arch Neurol. 1985;42(12):1133.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

To the Editor

—In 1984, Cruse et al¹ described two siblings with systemic carnitine deficiency. One of these children had a recurrent metabolic encephalopathy that was diagnosed as Reye syndrome. The first episode of this toxic syndrome occurred at 11 months of age and was preceded by a four-day history of low-grade fever, decreased appetite, and diarrhea. Her initial blood glucose concentration was 2 mg/dL. She had a hepatomegaly of 3 cm, and her liver enzyme levels were moderately elevated. Intravenous glucose was begun and the child was alert by 24 hours. At age 36 months the child again became obtunded during the course of an intercurrent upper respiratory tract infection. Again the child was found to have hypoglycemia, mild hyperammonemia, and hepatomegaly. Subsequent evaluation showed that she also had decreased serum and tissue levels of carnitine. Her older sibling, who was asymptomatic, was also found to be carnitine . . . [Full Text PDF of this Article]

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