0
Article |

Cerebral Gigantism FREE

John E. Ott, MD; Arthur Robinson, MD
[+] Author Affiliations

Received for publication May 9, 1968.

Reprint requests to Department of Biophysics, University of Colorado Medical Center, 4200 E Ninth Ave, Denver 80220 (Dr. Robinson).


Am J Dis Child. 1969;117(3):357-368. doi:10.1001/archpedi.1969.02100030359023.
Text Size: A A A
Published online

SINCE Sotos et al1 described the syndrome of cerebral gigantism characterized by large stature, typical facies, nonprogressive neurological disorder, and mental retardation, there have been 33 cases reported.2-10 The purpose of this paper is to present an additional case report which includes some studies not previously emphasized, and to review the literature.

Report of a Case  The patient is an 11½-year-old white girl who has been evaluated annually since her fifth year because of her large stature. Following a term pregnancy complicated by hyperemesis gravidarum, vaginal bleeding, marked edema, and a difficult six-hour labor, normal deliver ensued. There were no neonatal difficulties except moderate jaundice which persisted for one month. At birth, her measurements were as follows: length, 56 cm (22½ inches) (> 97th percentile); weight, 4,423 gm (9 lb 12 ounces) (> 97th percentile); and head circumference, 40 cm (> 97th percentile).General development was within normal

REFERENCES

Sotos, J.F., et al:  Cerebral Gigantism in Childhood: A Syndrome of Excessively Rapid Growth With Acromegalic Features and a Nonprogressive Neurologic Disorder , New Eng J Med 271:109-116 ( (July 16) ) 1964;.
Marie, J., et al:  Gigantisme Avec Encéphalopathie et Dysmorphie Cranio-Faciale , Ann Pediat 12:2574-2583 ( (Nov) ) 1965;.
Kjellman, B.:  Cerebral Gigantism , Acta Paediat Scand 54:603-609 ( (Nov) ) 1965;.
Kowlessar, M.:  Cerebral Gigantism , Minn Med 48:1610-1614 ( (Dec) ) 1965;.
Cohen, M.:  Cerebral Gigantism in Childhood , New Eng J Med 271:635 ( (Sept 17) ) 1964;.
Hook, E.B., and Reynolds, J.W.:  Cerebral Gigantism: Endocrinological and Clinical Observations of Six Patients Including a Congenital Giant, Concordant Monozygotic Twins and a Child Who Achieved Adult Gigantic Size , J Pediat 70:900-914 ( (June) ) 1967;.
Turner, E.K., and Sloan, L.E.G.:  Cerebral Gigantism in Childhood: A Report of Two Further Cases , Aust Paediat J 1:243-249 ( (Dec) ) 1965;.
Feingold, cited by Milunsky, A., et al:  Cerebral Gigantism in Childhood: A Report of Two Cases and a Review of the Literature , Pediatrics 40:395-402 ( (Sept) ) 1967;.
Milunsky, A.; Cowie, V.; and Donoghue, E.C.:  Cerebral Gigantism in Childhood: A Report of Two Cases and a Review of the Literature , Pediatrics 40: 395-402, ( (Sept) ) 1967;.
Stephenson, J.N.; Meuinger, R.C.; and Manson, G.:  Cerebral Gigantism , Pediatrics 41:130-138 ( (Jan) ) 1968;.
Holt, S.B.:  Quantitative Genetics of Finger-Print Patterns , Brit Med Bull 17:247-250 ( (Sept) ) 1961;.
Hansman, C.:  Growth of Interorbital Distance and Skull Thickness as Observed in Roentgenographic Measurements , Radiology 86:87-96 ( (Jan) ) 1966;.
Heald, F.P., et al:  Plasma Levels of Free Fatty Acids in Adolescents , Amer J Clin Nut 20: 1010 ( (Sept) ) 1967;.
Poznanski, A.K., and Stephenson, J.M.:  Radiographic Findings in Hypothalamic Acceleration of Growth Associated With Cerebral Atrophy and Mental Retardation (Cerebral Gigantism) , Radiology 88:446-456 ( (March) ) 1967;.
Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence , ed 3, Springfield, Ill: Charles C Thomas, Publisher, 1965;, p 181 and pp 460-462.
Sacrez, J.M.L., and Bernardy, L.:  Gigantisme Avec Hypertrophie Musculaire Généraliśee Chez un Garçon de deux ans , Arch Franc Pediat 15:709-714 ( (Nov 5) ) 1958;.
Nelhaus, G.:  Head Circumference, Birth to Eighteen Years: Practical Composite International and Interracial Graphs , Pediatrics 41:106-114 ( (Jan) ) 1968;.
Bhatia, M.L.; Misra, S.C.; and Prakash, J.:  Laryngeal Manifestations in Acromegaly , J Laryng 80:412-417 ( (April) ) 1966;.
Brobeck, J.R.:  Mechanism of Development of Obesity in Animals With Hypothalamic Lesions , Physiol Rev 26:541-559 ( (July) ) 1946;.
Reichlin, S.:  Growth and the Hypothalamus , Endocrinology 67:760-773 ( (Dec) ) 1960;.
Franz, J.; Haselbach, C.H.; and Libert, O.:  Studies of the Effect of Hypothalamic Extracts on Somatotropic Pituitary Function , Acta Endocr 41: 336-350 ( (Nov) ) 1962;.
Najjar, S., and Blizzard, R.M.:  Current Concepts Regarding Human Growth Hormone , Pediat Clin N Amer 13:437-457 ( (May) ) 1966;.
Beck, P.; Parker, M.L.; and Daughaday, W.H.:  Paradoxical Hypersecretion of Growth Hormone in Response to Glucose , J Clin Endocrinol 26:463-469 ( (March) ) 1966;.
Cornblath, M.; Parker, M.L.; and Reisner, S.:  Secretion and Metabolism of Growth Hormone in Premature and Full-Term Infants , J Clin Endocr 25:209-218 ( (Feb) ) 1965;.
Greulich., W., and Pyle, S.I.: Radiographic Atlas of Development of the Hand and Wrist , Palo Alto, Calif: Stanford University Press, 1959;.
Glick, S.M., et al:  Immunoassay of Human Growth Hormone in Plasma , Nature 199:784-787 ( (Aug 24) ) 1963;.
Schalch, D.S., and Parker, M.L.:  A Sensitive Double Antibody Immunoassay for Human Growth Hormone in Plasma , Nature 203:1141 ( (Sept 12) ) 1964;.

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

Sotos, J.F., et al:  Cerebral Gigantism in Childhood: A Syndrome of Excessively Rapid Growth With Acromegalic Features and a Nonprogressive Neurologic Disorder , New Eng J Med 271:109-116 ( (July 16) ) 1964;.
Marie, J., et al:  Gigantisme Avec Encéphalopathie et Dysmorphie Cranio-Faciale , Ann Pediat 12:2574-2583 ( (Nov) ) 1965;.
Kjellman, B.:  Cerebral Gigantism , Acta Paediat Scand 54:603-609 ( (Nov) ) 1965;.
Kowlessar, M.:  Cerebral Gigantism , Minn Med 48:1610-1614 ( (Dec) ) 1965;.
Cohen, M.:  Cerebral Gigantism in Childhood , New Eng J Med 271:635 ( (Sept 17) ) 1964;.
Hook, E.B., and Reynolds, J.W.:  Cerebral Gigantism: Endocrinological and Clinical Observations of Six Patients Including a Congenital Giant, Concordant Monozygotic Twins and a Child Who Achieved Adult Gigantic Size , J Pediat 70:900-914 ( (June) ) 1967;.
Turner, E.K., and Sloan, L.E.G.:  Cerebral Gigantism in Childhood: A Report of Two Further Cases , Aust Paediat J 1:243-249 ( (Dec) ) 1965;.
Feingold, cited by Milunsky, A., et al:  Cerebral Gigantism in Childhood: A Report of Two Cases and a Review of the Literature , Pediatrics 40:395-402 ( (Sept) ) 1967;.
Milunsky, A.; Cowie, V.; and Donoghue, E.C.:  Cerebral Gigantism in Childhood: A Report of Two Cases and a Review of the Literature , Pediatrics 40: 395-402, ( (Sept) ) 1967;.
Stephenson, J.N.; Meuinger, R.C.; and Manson, G.:  Cerebral Gigantism , Pediatrics 41:130-138 ( (Jan) ) 1968;.
Holt, S.B.:  Quantitative Genetics of Finger-Print Patterns , Brit Med Bull 17:247-250 ( (Sept) ) 1961;.
Hansman, C.:  Growth of Interorbital Distance and Skull Thickness as Observed in Roentgenographic Measurements , Radiology 86:87-96 ( (Jan) ) 1966;.
Heald, F.P., et al:  Plasma Levels of Free Fatty Acids in Adolescents , Amer J Clin Nut 20: 1010 ( (Sept) ) 1967;.
Poznanski, A.K., and Stephenson, J.M.:  Radiographic Findings in Hypothalamic Acceleration of Growth Associated With Cerebral Atrophy and Mental Retardation (Cerebral Gigantism) , Radiology 88:446-456 ( (March) ) 1967;.
Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence , ed 3, Springfield, Ill: Charles C Thomas, Publisher, 1965;, p 181 and pp 460-462.
Sacrez, J.M.L., and Bernardy, L.:  Gigantisme Avec Hypertrophie Musculaire Généraliśee Chez un Garçon de deux ans , Arch Franc Pediat 15:709-714 ( (Nov 5) ) 1958;.
Nelhaus, G.:  Head Circumference, Birth to Eighteen Years: Practical Composite International and Interracial Graphs , Pediatrics 41:106-114 ( (Jan) ) 1968;.
Bhatia, M.L.; Misra, S.C.; and Prakash, J.:  Laryngeal Manifestations in Acromegaly , J Laryng 80:412-417 ( (April) ) 1966;.
Brobeck, J.R.:  Mechanism of Development of Obesity in Animals With Hypothalamic Lesions , Physiol Rev 26:541-559 ( (July) ) 1946;.
Reichlin, S.:  Growth and the Hypothalamus , Endocrinology 67:760-773 ( (Dec) ) 1960;.
Franz, J.; Haselbach, C.H.; and Libert, O.:  Studies of the Effect of Hypothalamic Extracts on Somatotropic Pituitary Function , Acta Endocr 41: 336-350 ( (Nov) ) 1962;.
Najjar, S., and Blizzard, R.M.:  Current Concepts Regarding Human Growth Hormone , Pediat Clin N Amer 13:437-457 ( (May) ) 1966;.
Beck, P.; Parker, M.L.; and Daughaday, W.H.:  Paradoxical Hypersecretion of Growth Hormone in Response to Glucose , J Clin Endocrinol 26:463-469 ( (March) ) 1966;.
Cornblath, M.; Parker, M.L.; and Reisner, S.:  Secretion and Metabolism of Growth Hormone in Premature and Full-Term Infants , J Clin Endocr 25:209-218 ( (Feb) ) 1965;.
Greulich., W., and Pyle, S.I.: Radiographic Atlas of Development of the Hand and Wrist , Palo Alto, Calif: Stanford University Press, 1959;.
Glick, S.M., et al:  Immunoassay of Human Growth Hormone in Plasma , Nature 199:784-787 ( (Aug 24) ) 1963;.
Schalch, D.S., and Parker, M.L.:  A Sensitive Double Antibody Immunoassay for Human Growth Hormone in Plasma , Nature 203:1141 ( (Sept 12) ) 1964;.

Correspondence

CME
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.