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NEUROLOGY 1982;32:441
© 1982 American Academy of Neurology

Infantile hypoventilation syndrome, neurenteric cyst, and syringobulbia

Hyung Doo Chung, M.D., Daphne E. DeMello, M.D., Noreen D'Souza, M.D. and Jaime Estrada, M.D.

Departments of Pathology (Drs. Chung, DeMello, and D'Souza) and Pediatrics (Dr. Estrada). Veterans Administration Medical Center, Cardinal Glennon Memorial Hospital for Children, and St. Louis University School of Medicine, St. Louis, MO.

Hypoventilation syndrome was observed in an infant with a neurenteric cyst. She died at 9 months of age following episodic bradycardia and the loss of voluntary as well as autonomic respiratory control.

There was a mediastinal neurenteric cyst with anterior spina bifida and extension into the upper thoracic spinal cord. Secondary syringomyelobulbia was present at necropsy. The syringobulbia involved the hypoglossal nuclei, the dorsal motor nuclei of the vagus in the lower medulla, the right nucleus ambiguus, the right fasciculus and nucleus solitarius, the right mediolateral reticular formation, and the right dorsal motor nucleus of the vagus at the level of the obex. This morphologic distribution is attributed to the intraspinal rupture and upward extension of the mucinous contents of the cyst, and appears to be responsible for the hypoventilation syndrome.

Address correspondence and reprint requests to Dr. Chung, Laboratory Services, 113 JC. Veterans Administration Medical Center, 915 N. Grand Boulevard, St. Louis, MO 63125.

Accepted for publication August 26, 1981.






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