CEREBRAL GIGANTISM

2008 ◽  
Vol 25 (2) ◽  
pp. 248-252 ◽  
Author(s):  
Philip R. Dodge ◽  
Sandra J. Holmes ◽  
Juan F. Sotos
Keyword(s):  
Cerebral Gigantism

145 THE PATHOLOGY OF CEREBRAL GIGANTISM

1983 ◽  
Vol 42 (3) ◽  
pp. 352
Author(s):  
J. C. Goodman ◽  
W. B. Dobyns ◽  
J. B. Kirkpatrick
Keyword(s):  
Cerebral Gigantism

CEREBRAL GIGANTISM IN CHILDHOOD

PEDIATRICS ◽  
1967 ◽  
Vol 40 (3) ◽  
pp. 395-402
Author(s):  
Aubrey Milunsky ◽  
Valerie A. Cowie ◽  
Elaine C. Donoghue
Keyword(s):  
Growth Hormone ◽  
Mental Retardation ◽  
Neurological Disorder ◽  
Pathogenic Mechanism ◽  
Plasma Growth Hormone ◽  
High Arched Palate ◽  
Cerebral Gigantism ◽  
Hypothalamic Pituitary Axis ◽  
Neurological Lesion ◽  
Mechanism Operative

Two cases of cerebral gigantism in childhood are reported, and 14 earlier cases are reviewed. The major manifestations of this non-progressive neurological disorder included gigantism, macrocrania, dolichocephaly, mental retardation, characteristic facies, high arched palate, and ataxia or clumsiness. Pneumoencephalography in 10 out of 11 cases revealed a dilated ventricular system. Normal fasting plasma growth hormone levels were found in our patients, but both showed evidence of impaired function of the hypothalamic-pituitary axis in that these levels failed to rise following marked hypoglycemia. Abnormal dermatoglyphic patterns are reported and their value as an aid to diagnosis is mooted. Both the cause and the nature of the neurological lesion remain obscure. The evidence favors a pathogenic mechanism operative in utero.

CEREBRAL GIGANTISM

PEDIATRICS ◽  
1968 ◽  
Vol 41 (1) ◽  
pp. 130-138
Author(s):  
John N. Stephenson ◽  
Raymond C. Mellinger ◽  
Gordon Manson
Keyword(s):  
Mental Retardation ◽  
Bone Age ◽  
Communicating Hydrocephalus ◽  
Cerebral Gigantism ◽  
Central Nervous System Dysfunction ◽  
Anterior Pituitary Function ◽  
Adrenal Response ◽  
Steroid Excretion ◽  
Physical Findings ◽  
Dexamethasone Suppression

Studies are reported of 10 children having physical findings previously reported in cases classified as showing cerebral gigantism. These findings included mental retardation; advanced height, weight, and bone age; and a characteristic facies. Six had pneumoencephalography and all showed communicating hydrocephalus. Karyograms were normal. Urinary 17-KS steroid excretion was increased for chronologic age, but levels were compatible with physiologic age. Adrenal response to dexamethasone suppression and metyrapone administration was normal. The condition is regarded as a reflection of congenital central nervous system dysfunction with mental retardation and altered hypothalamic control of anterior pituitary function.

Ocular Findings in a Family With Sotos Syndrome (Cerebral Gigantism)

1995 ◽  
Vol 119 (5) ◽  
pp. 657-658 ◽  
Author(s):  
Robert K. Koenekoop ◽  
Kenneth N. Rosenbaum ◽  
Elias I. Traboulsi
Keyword(s):  
Sotos Syndrome ◽  
Cerebral Gigantism ◽  
Ocular Findings

Apparent Sotos syndrome (cerebral gigantism) in a child with trisomy 20p11.2-p12.1 mosaicism

2000 ◽  
Vol 91 (4) ◽  
pp. 273-276 ◽  
Author(s):  
Laurence Faivre ◽  
G�raldine Viot ◽  
Marguerite Prieur ◽  
Catherine Turleau ◽  
Philippe Gosset ◽  
...  
Keyword(s):  
Sotos Syndrome ◽  
Cerebral Gigantism

scholarly journals Evidence for autosomal recessive inheritance in cerebral gigantism

1974 ◽  
Vol 11 (2) ◽  
pp. 158-165 ◽  
Author(s):  
S. Nevo ◽  
M. Zeltzer ◽  
A. Benderly ◽  
J. Levy
Keyword(s):  
Autosomal Recessive ◽  
Autosomal Recessive Inheritance ◽  
Recessive Inheritance ◽  
Cerebral Gigantism
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