PEDIATRICS Vol. 38 No. 3 September 1966, pp. 473-482
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Bain, H. W.
Right arrow Articles by Kruyff, E.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Bain, H. W.
Right arrow Articles by Kruyff, E.

THE DIENCEPHALIC SYNDROME OF EARLY INFANCY DUE TO SILENT BRAIN TUMOR: WITH SPECIAL REFERENCE TO TREATMENT

Harry W. Bain M.D.1, John M. M. Darte M.D.2, William S. Keith M.D.3, and Evert Kruyff M.D.4

1 The Department of Paediatrics, The Hospital for Sick Children, and The University of Toronto, Toronto 2, Ontario
2 The Department of Pathology, The Hospital for Sick Children, and The University of Toronto, Toronto 2, Ontario
3 The Department of Neurosurgery, The Hospital for Sick Children, and The University of Toronto, Toronto 2, Ontario
4 The Department of Radiology, The Hospital for Sick Children, and The University of Toronto, Toronto 2, Ontario

Three patients with typical "diencephalic syndrome" have been described. The clinical observation of an emaciated and pale, but remarkably bright, cheerful, and active infant with a normal or increased appetite and food intake should suggest the diagnosis. Physical findings are conspicuous by their absence, although mild nystagmus, tremor, or ataxia may occasionally be present. An elevated total eosinophil count and an elevated cerebrospinal fluid protein are important, though not invariable, features. Metopirone test coupled with ACTH-adrenal stimulation test indicate markedly reduced pituitary reserve. Diagnosis is confirmed by pneumoencephalography and biopsy.

It is likely that with increasing knowledge, the clinical spectrum of this syndrome will be broadened, and one might expect to find, on occasions, anorexia rather than increased appetite, lassitude rather than hyperactivity, irritability rather than euphoria, obesity rather than emaciation, and different combinations of all of these features.

The results of therapy with beam-directed cobalt irradiation are encouraging.

Submitted on February 24, 1966
Accepted on April 16, 1966




This article has been cited by other articles:


Home page
 PediatricsHome page
A. Fleischman, C. Brue, T. Y. Poussaint, M. Kieran, S. L. Pomeroy, L. Goumnerova, R. M. Scott, and L. E. Cohen
Diencephalic Syndrome: A Cause of Failure to Thrive and a Model of Partial Growth Hormone Resistance
Pediatrics, June 1, 2005; 115(6): e742 - e748.
[Abstract] [Full Text] [PDF]

Home page
 CLIN PEDIATRHome page
R. A. W. Lehman, S. Krishnamurthy, and C. M. Berlin
Weight and Height Deficits in Children with Brain Stem Tumors
Clinical Pediatrics, June 1, 2002; 41(5): 315 - 321.
[Abstract] [PDF]

Home page
 Physiol. Rev.Home page
E. E. Muller, V. Locatelli, and D. Cocchi
Neuroendocrine Control of Growth Hormone Secretion
Physiol Rev, April 1, 1999; 79(2): 511 - 607.
[Abstract] [Full Text] [PDF]