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August 1965

Primary Alveolar Hypoventilation Syndrome (Ondine's Curse): Association With Manifestations of Hypothalmic Disease

Author Affiliations

From the Department of Pediatrics, University of Southern California School of Medicine, and Children's Hospital of Los Angeles, Senior Pediatric Resident (Dr. Fishman); Chief Pediatric Resident (Dr. Samson); and Fellow, Pediatric Cardiology (Dr. Sperling).

Am J Dis Child. 1965;110(2):155-161. doi:10.1001/archpedi.1965.02090030165011

THIS PAPER REPORTS a case of alveolar hypoventilation associated with presumptive evidence of hypothalamic disease. Primary alveolar hypoventilation syndromes have been reported in adults and children, presumably secondary to brain stem disease3-16 and to obesity in adults17-27 and children.28-32 To our knowledge, alveolar hypoventilation in patients with clinical evidence of hypothalamic disease has not been reported.

Report of a Case  First Admission.—A 3½-year-old white male (Fig 1, A and B) was in good health until nine months prior to admission, when he developed a voracious appetite and lethargy. During the following nine months he gained 23 lb (10.4 kg) and gradually became less active and was disinterested in play. He began to stutter and had "twitching spells," sometimes associated with mild cyanosis.Six months prior to admission he was treated with thyroid extract, 30 mg per day, because of the presumed diagnosis of thyroid deficiency based