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         Cerebral Gigantism:     more detail

21. Webkatalog
3. Soto's Syndrome Also known as cerebral gigantism, an information sheet compiled by NINDS.
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

22. Sotos Syndrome
cerebral gigantism. Disorder Subdivisions. None. General Discussion. Sotos Syndromeis a rare genetic disorder characterized by excessive growth that occurs
http://my.webmd.com/hw/health_guide_atoz/nord271.asp
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Who We Are About WebMD Site Map You are in Medical Library Choose a Topic Our Content Sources Ask A Question Clinical Trials Health Guide A-Z Health Topics Symptoms Medical Tests Medications ... For a Complete Report Sotos Syndrome Important It is possible that the main title of the report Sotos Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms
  • Cerebral Gigantism
Disorder Subdivisions
  • None
General Discussion Resources March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 Tel: (914)428-7100 Fax: (914)997-4763 Tel: (888)663-4637 TDD: (914)997-4764 Email: Askus@marchofdimes.com Internet: http://www.marchofdimes.com The Arc (a national organization on mental retardation) 1010 Wayne Ave Suite 650 Silver Spring, MD 20910

23. Arch Pediatr Adolesc Med -- Abstract: Cerebral Gigantism Associated With Wilms'
cerebral gigantism associated with Wilms tumor Two children had cerebralgigantism in unique association with Wilms tumor. An increased incidence of
http://archpedi.ama-assn.org/cgi/content/abstract/138/5/486
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 138 No. 5, May 1984 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager Articles in PubMed by Maldonado V Poznanski AK Contact me when this article is cited
Cerebral gigantism associated with Wilms' tumor
V. Maldonado, P. S. Gaynon and A. K. Poznanski
Two children had cerebral gigantism in unique association with Wilms' tumor. An increased incidence of neoplasms has been reported in cerebral gigantism, as well as in Beckwith-Wiedemann syndrome. We report similarities and differences between these disorders.
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24. Arch Pediatr Adolesc Med -- Abstract: A Case Of Cerebral Gigantism And Hepatocar
A 14year-old boy, who had the physical and neurological characteristics ofcerebral gigantism (Sotos syndrome), developed hepatocarcinoma.
http://archpedi.ama-assn.org/cgi/content/abstract/131/6/631
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 131 No. 6, June 1977 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager Articles in PubMed by Sugarman GI Reed WB Contact me when this article is cited
A case of cerebral gigantism and hepatocarcinoma
G. I. Sugarman, E. T. Heuser and W. B. Reed
A 14-year-old boy, who had the physical and neurological characteristics of cerebral gigantism (Sotos syndrome), developed hepatocarcinoma. This tumor is rare in children and has never, to our knowledge, been recorded in a patient with cerebral gigantism. An autopsy was performed, the first we are aware of in a patient with cerebral gigantism without increased size in ventricles.

25. Cerebral Gigantism In Childhood; A Report Of Two Cases And A Review Of The Liter
The term cerebral gigantism was first applied by Sotos, et al. to five childrenwith a syndrome characterized by gigantism, a nonprogressive neurological
http://pediatrics.aappublications.org/cgi/content/abstract/40/3/395
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This Article P Rs: Submit a response Alert me when this article is cited Alert me when P ... Alert me if a correction is posted Services E-mail 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 PubMed PubMed Citation Articles by Milunsky, A. Articles by Donoghue, E. C. Pediatrics, Sep 1967, 395-402, Vol 40, No. 3
Cerebral gigantism in childhood; A report of two cases and a review of the literature
A Milunsky, VA Cowie and EC Donoghue
Department of Pediatrics, Tufts-New England Medical Center, Boston, Massachusetts 02111. The term cerebral gigantism was first applied by Sotos, et al. to five children with a syndrome characterized by gigantism, a non-progressive neurological disorder with mental retardation, typical facies, and a high arched palate. Pneumoencephalography in three patients revealed a dilated ventricular system. Nine further cases have been documented. In this study

26. Cerebral Gigantism -- Stephenson Et Al. 41 (1): 130 -- Pediatrics
cerebral gigantism. JN Stephenson, RC Mellinger and G Manson The clinicalsyndrome termed cerebral gigantism by Sotos et al. has three principal
http://pediatrics.aappublications.org/cgi/content/abstract/41/1/130
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This Article P Rs: Submit a response Alert me when this article is cited Alert me when P ... Alert me if a correction is posted Services E-mail 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 PubMed PubMed Citation Articles by Stephenson, J. N. Articles by Manson, G. Pediatrics, Jan 1968, 130-138, Vol 41, No. 1
Cerebral gigantism
JN Stephenson, RC Mellinger and G Manson
Department of Pediatrics and Division of Endocrinology Henry Ford Hospital, Detroit ABSTRACT. 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

27. EMedicine - Gigantism And Acromegaly : Article By Melanie Shim, MD
Exogenous obesity cerebral gigantism (Sotos syndrome) Weaver syndrome Ray M,Malhi P, Bhalla AK, Singhi PD cerebral gigantism with West syndrome.
http://www.emedicine.com/ped/topic2634.htm
(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Pediatrics Endocrinology
Gigantism and Acromegaly
Last Updated: July 29, 2004 Rate this Article Email to a Colleague Synonyms and related keywords: growth hormone excess, GH excess, giantism, gigantosoma, giant, hypersomia, somatomegaly, acromegalia, endocrine system, pituitary gland, abnormal growth AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Clinical Differentials ... Bibliography
Author: Melanie Shim, MD , Clinical Instructor, Department of Pediatrics, Division of Pediatric Endocrinology, Mattel Children's Hospital and University of California at Los Angeles Coauthor(s): Pinchas Cohen, MD , Director of Research and Training, Professor, Department of Pediatrics, Division of Endocrinology, Mattel Children's Hospital, University of California at Los Angeles Melanie Shim, MD, is a member of the following medical societies:

28. EMedicine - Hyperpituitarism : Article By Melanie Shim, MD
cerebral gigantism (Sotos syndrome) BeckwithWiedemann syndrome Marfan syndromeWeaver syndrome Fragile X syndrome The differential diagnosis of GH excess
http://www.emedicine.com/ped/topic1092.htm
(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Pediatrics Endocrinology
Hyperpituitarism
Last Updated: March 18, 2004 Rate this Article Email to a Colleague Synonyms and related keywords: pediatric pituitary adenomas, primary hypersecretion of pituitary hormones, prolactinoma, corticotropinoma, somatotropinoma, thyrotropinoma, Cushing disease, Cushing's disease, Cushing syndrome, Cushing's syndrome, pituitary disease AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Clinical Differentials ... Bibliography
Author: Melanie Shim, MD , Clinical Instructor, Department of Pediatrics, Division of Pediatric Endocrinology, Mattel Children's Hospital and University of California at Los Angeles Coauthor(s): Pinchas Cohen, MD , Director of Research and Training, Professor, Department of Pediatrics, Division of Endocrinology, Mattel Children's Hospital, University of California at Los Angeles Melanie Shim, MD, is a member of the following medical societies:

29. Database Search Results
by the US Department of Health and Human Services. Searched keywords for CerebralGigantism. Sotos Syndrome Support Association
http://www.health.gov/nhic/NHICScripts/Hitlist.cfm?Keyword=Cerebral Gigantism

30. Health Information Resource Database: Sotos Syndrome Support Association
Sotos syndrome, also known as cerebral gigantism, is a neurological brainbaseddisorder resulting in physical and mental developmental delays in children.
http://www.health.gov/nhic/NHICScripts/Entry.cfm?HRCode=HR2419

31. Sotos Syndrome
Synonyms. cerebral gigantism. Disorder Subdivisions. None. General Discussion.Sotos Syndrome is a rare genetic disorder characterized by excessive growth
http://www.bchealthguide.org/kbase/nord/nord271.htm
var hwPrint=1;var hwDocHWID="nord271";var hwDocTitle="Sotos Syndrome";var hwRank="1";var hwSectionHWID="nord271-Header";var hwSource="en-caQ2_05";var hwDocType="Nord";
National Organization for Rare Disorders, Inc.
Sotos Syndrome
Important
It is possible that the main title of the report Sotos Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Cerebral Gigantism
Disorder Subdivisions
  • None
General Discussion
Resources
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
TDD: (914)997-4764
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com
The Arc (a national organization on mental retardation) 1010 Wayne Ave Suite 650 Silver Spring, MD 20910 Tel: (301)565-3842 Fax: (301)565-3843 Tel: (800)433-5255 TDD: (817)277-0553 Email: info@thearc.org Internet: http://www.thearc.org/ Sotos Syndrome Support Association P.O. Box 4626

32. Arquivos De Neuro-Psiquiatria -
ABSTRACT Sotos syndrome or cerebral gigantism is characterized by cerebral gigantism in childhood a syndrome of excessively rapid growth and
http://www.scielo.br/scielo.php?pid=S0004-282X2002000200009&script=sci_arttext&t

33. Arq. Neuro-Psiquiatr.  Vol.60 no.2A; Abstract: S0004-282X2002000200009
Translate this page Sotos syndrome (cerebral gigantism) analysis of 8 cases. Sotos syndrome orcerebral gigantism is characterized by macrocephaly, overgrowth,
http://www.scielo.br/scielo.php?pid=S0004-282X2002000200009&script=sci_abstract&

34. BDJ | Advanced Dental Development In Cerebral Gigantism
The role of the BDJ is to inform its readers of ideas, opinions, developmentsand key issues in dentistry clinical, practical and scientific - stimulating
http://www.nature.com/bdj/journal/v161/n10/abs/4805978a.html
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You are in: BDJ Archive Volume 161 : Issue 10
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British Dental Journal 1986; Volume 161, No. 10, pages 374-374. doi: 10.1038/sj.bdj.4805978
Advanced dental development in cerebral gigantism
Crosher, R
Abstract
No abstract available. Keywords
Age Determination by Skeleton, Bone and Bones, abnormalities, Case Report, Facial Bones, Female, Gigantism, Human, Infant,Newborn, Syndrome, Tooth Eruption, Tooth,Deciduous Previous abstract Table of Contents Next abstract BDJ e-Alerts ... Contact the BDJ The British Dental Journal is published by Nature Publishing Group for the British Dental Association

35. Karger Publishers
A Case of Sotos Syndrome (cerebral gigantism) and Psychosis Sotos syndrome,or cerebral gigantism, is a syndrome of accelerated growth during early
http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowAbstract&ProduktNr=

36. Spine - UserLogin
Scoliosis in cerebral gigantism, Sotos Syndrome A Case Report The authorshave seen five cases of of cerebral gigantism affected by scoliosis and
http://www.spinejournal.com/pt/re/spine/fulltext.00007632-199607150-00017.htm
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37. Spine - Abstract: Volume 21(14) July 15, 1996 P 1699-1702 Scoliosis In Cerebral
Scoliosis in cerebral gigantism, Sotos Syndrome A Case Report. Spine.21(14)16991702, July 15, 1996. Haga, Nobuhiko MD *; Nakamura, Shigeru MD *; Shimode
http://www.spinejournal.com/pt/re/spine/abstract.00007632-199607150-00017.htm
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Spine. 21(14):1699-1702, July 15, 1996.
Haga, Nobuhiko MD *; Nakamura, Shigeru MD *; Shimode, Masanori MD +; Yanagisako, Yasuo MD ++; Iwaya, Tsutomu MD [S] Abstract:
Study Design: This retrospective study reviewed five patients with scoliosis associated with cerebral gigantism (Sotos syndrome). Objective: To clarify the clinical characteristics and progression of scoliosis in patients with cerebral gigantism. Summary of Background Data: Little has been reported about spinal deformity in this syndrome. Methods: The records and radiographs of five patients were reviewed. Results: Scoliosis was first noticed at the age of 5 years in one case and at 6-16 months in the others. Their curve patterns were not consistent. Cobb's angles increased rapidly by 4 years of age and after 8 years of age despite attempts at bracing. Conclusions: Excessive growth in infancy and growth-spurt period after 8 years of age may be related to the rapid progression of scoliosis in the same period. (C) Lippincott-Raven Publishers.

38. Kprones SotosID10045
cerebral gigantism in childhood a syndrome of excessively rapid growth withacromegalic features and a nonprogressive neurologic disorder.
http://www.infobiogen.fr/services/chromcancer/Kprones/SotosID10045.html
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Sotos syndrome
Identity Other names Cerebral gigantism Sos Inheritance Generally sporadic, a few inherited cases. The familial case reported in 2003 was proved to have NSD1 mutation Clinics Phenotype and clinics
  • Excessive growth, advanced bone age, typical facial gestalt,developmental delay.
  • In infancy growth is rapid, but settles down above the >97th centile in early childhood.
  • The adult height remains close to normal.
  • Large hands and feet
  • Characteristic facial gestalt: macrocephaly (>97th centile), frontal bossing, prognathism, hypertelorism, and antimongoloid slant of the palpebral fissures
  • Occasional hypotonia and delay in motor and language development
  • Cardiac, urogenital, musculoskeletal, and ophthalmologic anomalies are observed. Neoplastic risk Relatively high. Neoplasms in SoS are found with a frequency of 2.2-3.9% Cytogenetics Inborn conditions Routine chromosome analysis usually shows normal karyotype. Chromosomal abnormality concering 5q35 were reported. In addition, several chromosomal translocations other than 5q have been published. Genes involved and Proteins Gene Name Location Note Haploinsufficiency of NSD1 is a major cause of SoS.
  • 39. Geneticalliance.org
    Sotos syndrome Also known as cerebral gigantism Support Groups. 1 organization(s)found. previous Page 1 of 1 next
    http://www.geneticalliance.org/ws_display.asp?filter=support_groups_by_disease&t

    40. Sotos' Syndrome (www.whonamedit.com)
    cerebral gigantism in childhood. A syndrome of excessively rapid growth with Evidence for autosomal recessive inheritance in cerebral gigantism.
    http://www.whonamedit.com/synd.cfm/2464.html

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    Sotos' syndrome Also known as: Nevo's syndrome Sotos' sequence Synonyms: Cerebral gigantism, cerebral gigantism in childhood, constitutional gigantism, gigantismus cerebralis, gigantismus constitutionalis, gigantismus idiopathicus, hypothalamic gigantism, macrostomia-mental retardation syndrome, mental retardation-overgrowth sequence, pituitary gigantism, prenatal gigantism with macrocephaly. Associated persons: S. Nevo Juan Fernandez Sotos Description: A syndrome of excessive growth during the first 4 to 5 years of life, with cerebral gigantism and generalised large muscles in childhood, acromegalic features, and a nonprogressive cerebral disorder with nonprogressive mild mental retardation and defective coordination. After early childhood growth seems to approach normal, remaining, however, two standard deviations above means for chronological age. Birth weight and length greater than normal. Other principal features are dolicocephaly, macrocrania, hypertelorism, antimongoloid palpebral slant, high-arched palate, frontal bossing, mandibular prognathism, and precocious dentition. Occasionally, obesity, convulsions, abnormal dermatoglyphic pattern. Both sexes. Most cases are sporadic but some are transmitted as an autosomal dominant trait.

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