eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Nelson Syndrome: Multimedia

Author: Thomas A Wilson, MD, Professor of Clinical Pediatrics, Department of Pediatrics; Director of Pediatric Endocrinology, Division of Pediatric Endocrinology, Department of Pediatrics, State University of New York at Stony Brook
Coauthor(s): Vardhini Desikan, MBBS, MPH, Pediatric Endocrinology Fellow, Department of Pediatrics, State University of New York at Stony Brook; George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London), Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; Antony Lafferty, MB ChB, FRACP, Senior Lecturer of Pediatric Endocrinology, Monash University Department of Pediatrics, National Institutes of Health, Bethesda, MD, and Princess Margaret Hospital for Children, Perth, Western Australia
Contributor Information and Disclosures

Updated: Mar 4, 2009

Multimedia

Physical findings in Cushing syndrome.
(Enlarge Image)
Media file 1: Physical findings in Cushing syndrome.
[ CLOSE WINDOW ]
Physical findings in Cushing syndrome.

Physical findings in Cushing syndrome.

More on Nelson Syndrome

Overview: Nelson Syndrome
Differential Diagnoses & Workup: Nelson Syndrome
Treatment & Medication: Nelson Syndrome
Follow-up: Nelson Syndrome
Multimedia: Nelson Syndrome
References
[ CLOSE WINDOW ]

References

  1. Nelson DH, Meakin JW, Dealy JB, et al. ACTH-producing tumor of the pituitary gland. N Engl J Med. Jul 24 1958;259(4):161-4. [Medline].

  2. van Aken MO, Pereira AM, van den Berg G, Romijn JA, Veldhuis JD, Roelfsema F. Profound amplification of secretory-burst mass and anomalous regularity of ACTH secretory process in patients with Nelson's syndrome compared with Cushing's disease. Clin Endocrinol (Oxf). Jun 2004;60(6):765-72. [Medline].

  3. Chapuis Y, Pitre J, Conti F, et al. Role and operative risk of bilateral adrenalectomy in hypercortisolism. World J Surg. Sep 1996;20(7):775-9; discussion 779-80. [Medline].

  4. Biller BM, Grossman AB, Stewart PM, et al. Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement. J Clin Endocrinol Metab. Jul 2008;93(7):2454-62. [Medline].

  5. Casulari LA, Naves LA, Mello PA, Pereira Neto A, Papadia C. Nelson's syndrome: complete remission with cabergoline but not with bromocriptine or cyproheptadine treatment. Horm Res. 2004;62(6):300-5. [Medline].

  6. Pivonello R, Faggiano A, Di Salle F, Filippella M, Lombardi G, Colao A. Complete remission of Nelson's syndrome after 1-year treatment with cabergoline. J Endocrinol Invest. Dec 1999;22(11):860-5. [Medline].

  7. Shraga-Slutzky I, Shimon I, Weinshtein R. Clinical and biochemical stabilization of Nelson's syndrome with long-term low-dose cabergoline treatment. Pituitary. 2006;9(2):151-4. [Medline].

  8. Petit JH, Biller BM, Yock TI, et al. Proton stereotactic radiotherapy for persistent adrenocorticotropin-producing adenomas. J Clin Endocrinol Metab. Feb 2008;93(2):393-9. [Medline].

  9. Kobayashi T, Kida Y, Mori Y. Gamma knife radiosurgery in the treatment of Cushing disease: long-term results. J Neurosurg. Dec 2002;97(5 Suppl):422-8. [Medline].

  10. Wolffenbuttel BH, Kitz K, Beuls EM. Beneficial gamma-knife radiosurgery in a patient with Nelson's syndrome. Clin Neurol Neurosurg. Mar 1998;100(1):60-3. [Medline].

  11. Mauermann WJ, Sheehan JP, Chernavvsky DR, Laws ER, Steiner L, Vance ML. Gamma Knife surgery for adrenocorticotropic hormone-producing pituitary adenomas after bilateral adrenalectomy. J Neurosurg. Jun 2007;106(6):988-93. [Medline].

  12. Vik-Mo EO, Oksnes M, Pedersen PH, et al. Gamma knife stereotactic radiosurgery of Nelson syndrome. Eur J Endocrinol. Feb 2009;160(2):143-8. [Medline].

  13. Assie G, Bahurel H, Coste J, et al. Corticotroph tumor progression after adrenalectomy in Cushing's Disease: A reappraisal of Nelson's Syndrome. J Clin Endocrinol Metab. Jan 2007;92(1):172-9. [Medline].

  14. De Tommasi C, Vance ML, Okonkwo DO, Diallo A, Laws ER Jr. Surgical management of adrenocorticotropic hormone-secreting macroadenomas: outcome and challenges in patients with Cushing's disease or Nelson's syndrome. J Neurosurg. Nov 2005;103(5):825-30. [Medline].

  15. Forni C, Giovanelli MA. Nelson's syndrome in the paediatric age. Childs Brain. 1977;3(5):309-14. [Medline].

  16. Gil-Cardenas A, Herrera MF, Diaz-Polanco A, Rios JM, Pantoja JP. Nelson's syndrome after bilateral adrenalectomy for Cushing's disease. Surgery. Feb 2007;141(2):147-51; discussion 151-2. [Medline].

  17. Hopwood NJ, Forsman PJ, Kenny FM, Drash AL. Hypoglycemia in hypopituitary children. Am J Dis Child. Aug 1975;129(8):918-26. [Medline].

  18. Hornyak M, Weiss MH, Nelson DH, Couldwell WT. Nelson syndrome: historical perspectives and current concepts. Neurosurg Focus. 2007;23(3):E12. [Medline].

  19. Imai T, Funahashi H, Tanaka Y, et al. Adrenalectomy for treatment of Cushing syndrome: results in 122 patients and long-term follow-up studies. World J Surg. 1996;20(7):781-786. [Medline].

  20. Jane JA, Vance ML, Woodburn CJ, Laws ER Jr. Stereotactic radiosurgery for hypersecreting pituitary tumors: part of a multimodality approach. Neurosurg Focus. May 15 2003;14(5):e12. [Medline].

  21. Karl M, Von Wichert G, Kempter E, et al. Nelson's syndrome associated with a somatic frame shift mutation in the glucocorticoid receptor gene. J Clin Endocrinol Metab. Jan 1996;81(1):124-9. [Medline].

  22. Kasperlik-Zaluska AA, Bonicki W, Jeske W, Janik J, Zgliczynski W, Czernicki Z. Nelson's syndrome -- 46 years later: clinical experience with 37 patients. Zentralbl Neurochir. Feb 2006;67(1):14-20. [Medline].

  23. Kasperlik-Zaluska AA, Zgliczynski W, Jeske W, Zdunowski P. ACTH responses to somatostatin, valproic acid and dexamethasone in Nelson's syndrome. Neuro Endocrinol Lett. Dec 2005;26(6):709-12. [Medline].

  24. Kelly DF. Transsphenoidal surgery for Cushing's disease: a review of success rates, remission predictors, management of failed surgery, and Nelson's Syndrome. Neurosurg Focus. 2007;23(3):E5. [Medline].

  25. Kovacs K, Horvath E, Coire C, et al. Pituitary corticotroph hyperplasia preceding adenoma in a patient with Nelson's syndrome. Clin Neuropathol. Mar-Apr 2006;25(2):74-80. [Medline].

  26. Lafferty AR, Chrousos GP. Pituitary tumors in children and adolescents. J Clin Endocrinol Metab. Dec 1999;84(12):4317-23. [Medline].

  27. Mercado-Asis LB, Yanovski JA, Tracer HL, Chik CL, Cutler GB Jr. Acute effects of bromocriptine, cyproheptadine, and valproic acid on plasma adrenocorticotropin secretion in Nelson's syndrome. J Clin Endocrinol Metab. Feb 1997;82(2):514-7. [Medline].

  28. Mullan KR, Leslie H, McCance DR, Sheridan B, Atkinson AB. The PPAR-gamma activator rosiglitazone fails to lower plasma ACTH levels in patients with Nelson's syndrome. Clin Endocrinol (Oxf). May 2006;64(5):519-22. [Medline].

  29. Munir A, Song F, Ince P, Walters SJ, Ross R, Newell-Price J. Ineffectiveness of rosiglitazone therapy in Nelson's syndrome. J Clin Endocrinol Metab. May 2007;92(5):1758-63. [Medline].

  30. Munir A, Song F, Ince P, Walters SJ, Ross R, Newell-Price J. Ineffectiveness of rosiglitazone therapy in Nelson's syndrome. J Clin Endocrinol Metab. May 2007;92(5):1758-63. [Medline].

  31. Oldfield EH, Schulte HM, Chrousos GP, et al. Corticotropin-releasing hormone (CRH) stimulation in Nelson's syndrome: response of adrenocorticotropin secretion to pulse injection and continuous infusion of CRH. J Clin Endocrinol Metab. May 1986;62(5):1020-6. [Medline].

  32. Pereira MA, Halpern A, Salgado LR, et al. A study of patients with Nelson's syndrome. Clin Endocrinol (Oxf). Oct 1998;49(4):533-9. [Medline].

  33. Reuss WA, Saeger W, Ludecke DK. Electron microscopical morphometry of well-differentiated and undifferentiated ACTH secreting adenomas in Cushing's disease and Nelson's syndrome. Virchows Arch A Pathol Anat Histopathol. 1991;419(5):395-401. [Medline].

  34. Thomas CG Jr, Smith AT, Benson M, Griffith J. Nelson's syndrome after Cushing's disease in childhood: a continuing problem. Surgery. Dec 1984;96(6):1067-77. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Nelson syndrome, Nelson's syndrome, bilateral adrenalectomy, Cushing disease, Cushing's disease, adrenocorticotropin, ACTH-secreting pituitary adenoma, pituitary adenoma, headaches, meningitis, hypopituitarism, hypothyroidism, galactorrhea, polyuria, polydipsia, hyperprolactinemia, diabetes insipidus, adrenal insufficiency, hypotension, vision loss, hepatomegaly, splenomegaly, hemochromatosis, optic nerve atrophy, Addison disease, autoimmune adrenal failure, adrenoleukodystrophy, hypoglycemia, thyroid disease, neuroendocrine tumor, pheochromocytoma, Fanconi anemia, neurofibromatosis type-1, optic gliomas

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Thomas A Wilson, MD, Professor of Clinical Pediatrics, Department of Pediatrics; Director of Pediatric Endocrinology, Division of Pediatric Endocrinology, Department of Pediatrics, State University of New York at Stony Brook
Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Vardhini Desikan, MBBS, MPH, Pediatric Endocrinology Fellow, Department of Pediatrics, State University of New York at Stony Brook
Vardhini Desikan, MBBS, MPH is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London), Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece
George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Antony Lafferty, MB ChB, FRACP, Senior Lecturer of Pediatric Endocrinology, Monash University Department of Pediatrics, National Institutes of Health, Bethesda, MD, and Princess Margaret Hospital for Children, Perth, Western Australia
Antony Lafferty, MB ChB, FRACP is a member of the following medical societies: Endocrine Society
Disclosure: Nothing to disclose.

Medical Editor

Angelo P Giardino, MD, PhD, Clinical Associate Professor, Department of Pediatrics, Baylor College of Medicine; Medical Director, Texas Children's Health Plan, Inc
Angelo P Giardino, MD, PhD is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, Harris County Medical Society, Helfer Society, and International Society for Prevention of Child Abuse and Neglect
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Lynne Lipton Levitsky, MD, Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor, Department of Pediatrics, Harvard University Medical School
Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds PI, also occasional consultant

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital
Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research
Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfizer, Inc. Honoraria Consulting

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.