HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Liu, H. M. Articles by Hunt, C. E. Search for Related Content PubMed PubMed Citation Articles by Liu, H. M. Articles by Hunt, C. E.

Congenital central hypoventilation syndrome

A pathologic study of the neuromuscular system

Departments of Pathology and Pediatrics (Neonatology), Children's Memorial Hospital and Northwestern University, Chicago, Illinois.

An infant with congenital central hypoventilation was managed by bilateral phrenic nerve pacing for 3 months. He died at 8 months of age, following 19 days of continuous bilateral pacing necessitated by the eventual loss of voluntary as well as autonomic ventilatory control. The phrenic nerves showed axonal dystrophy at the site of electrode implantation and more severe distal degeneration. Focal neurogenic atrophy was seen in the diaphragmatic muscle. These changes were attributed to electrical injury resulting from the period of continuous pacing. The most significant neuropathologic finding was a mild generalized decrease in the density of neurons and myelinated nerve fibers in the respiratory centers of the medulla. These morphologic abnormalities were attributed to a sublethal intrauterine lesion that would be the first example of a morphologic correlation with the functional abnormality in congenital central hypoventilation.

Dr. Liu's address is Children's Memorial Hospital, 2300 Children's Plaza, Chicago, Illinois.

Reprint requests should be addressed to Dr. Hunt, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614.

Accepted for publication October 7, 1977.

This article has been cited by other articles:

				K. E. Macey, P. M. Macey, M. A. Woo, R. K. Harper, J. R. Alger, T. G. Keens, and R. M. Harper fMRI signal changes in response to forced expiratory loading in congenital central hypoventilation syndrome J Appl Physiol, November 1, 2004; 97(5): 1897 - 1907. [Abstract] [Full Text] [PDF]

				Idiopathic Congenital Central Hypoventilation Syndrome . Diagnosis and Management Am. J. Respir. Crit. Care Med., July 1, 1999; 160(1): 368 - 373. [Full Text]

				E. C. Alvord Jr and C.-M. Shaw Congenital Difficulties With Swallowing and Breathing Associated With Maternal Polyhydramnios: Neurocristopathy or Medullary Infarction? J Child Neurol, October 1, 1989; 4(4): 299 - 306. [Abstract] [PDF]

				R. Beckerman, J. Meltzer, A. Sola, D. Dunn, and M. Wegmann Brain-Stem Auditory Response in Ondine's Syndrome Arch Neurol, July 1, 1986; 43(7): 698 - 701. [Abstract] [PDF]