Abstract
Nelson’s syndrome is a specific form of Cushing’s disease treated by bilateral adrenalectomy, presenting with a deep hyperpigmentation caused by a pituitary adenoma (corticotropinoma). These ACTH-secreting tumors are frequently aggressive, so early diagnosis is of prime importance. We have studied 33 patients with Nelson’s syndrome, 28 women and 5 men, aged 14–56 yr at the time of adrenalectomy and 16–58 yr at the time of Nelson’s syndrome diagnosis (observed for 5–32 yr). Methods of examination included simultaneous adrenocorticotropic hormone (ACTH) and cortisol measurements during routine hydrocortisone replacement therapy, computed tomography (CT), pituitary magnetic resonance imaging (MRI), and visual field examination. The results obtained in a group of six patients diagnosed in the last 3 yr were compared with those obtained in a group of 27 patients examined before 1992. High plasma ACTH levels accompanied by normal serum cortisol concentration were characteristic for a late stage of the disease. Absolute temporal scotomas were an early finding. MRI, especially with the gadolinium enhancement, was superior to CT in demonstrating pituitary microadenomas in Nelson’s syndrome. Thus, MRI diagnosis allowed for an early neurosurgical treatment of the patients with Nelson’s tumors.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Bebie H. (1990) Computer-assisted evaluation of visual fields.Graefe’s Arch. Clin. Exp. Ophthalmol. 228, 243–254.
Egge A. C. and Feldon S. E. (1983) Quantitative correlation between Octopus perimetry and pituitary volume.Invest. Ophthalmol. Visual Sci. 24, 133–136.
Grua J. R. and Nelson D. H. (1991) ACTH-producing pituitary tumors.Endocrinol. Metab. Clin. N. Am. 20, 319–362.
Hodgkinson S., Allolio B., Landon J., and Lowry P. (1984) Development of a non-extracted two-site immunoradiometric assay for corticotropin utilizing amino- and carboxy-terminally directed antibodies.Biochem. J. 218, 703–706.
Jenkins P. J., Trainer P. J., Plowman P. N., Shand W. S., Grossman A. B., Wass J. A. H., and Besser G. M. (1994) The long term outcome after adrenalectomy and prophylactic pituitary radiotherapy in adrenocorticotropin-dependent Cushing’s syndrome.J. Clin. Endocr. Metab. 80, 165–171.
Kasperlik-Zaluska A., Hartwig W., Wislawski J., and Guiot G. (1976) Nelson’s syndrome following bilateral total adrenalectomy.Endokrynol. Pol. 27, 401–408.
Kasperlik-Zaluska A. and Nielubowicz J. (1981) Cushing’s disease.Lancet i, 1428.
Kasperlik-Zaluska A. A., Nielubowicz J., Wislawski J., Hartwig W., Zaluska J., Jeske W., and Migdalska B. (1983) Nelson’s syndrome: incidence and prognosis.Clin. Endocrinol. 19, 693–698.
Kemink L., Pieters G., Hermus A., Smals A., and Kloppenborg P. (1994) Patient’s age is a simple predictive factor for the development of Nelson’s syndrome after total adrenalectomy for Cushing’s disease.J. Clin. Endocrinol. Metab. 79, 887–889.
McCance D. R., Russell C. F. J., Kennedy T. L., Hadden D. R., Kennedy L., and Atkinson A. B. (1993) Bilateral adrenalectomy: low mortality and morbidity in Cushing’s disease.Clin. Endocrinol. 39, 315–321.
Moore T. J., Dluhy R. G., Williams G. H., and Cain J. P. (1976) Nelsons syndrome: frequency, prognosis and effect of prior pituitary irradiation.Ann. Intern. Med. 85, 731–734.
Nelson D. H., Meakin J. W., Dealy J. B., Matson D. D., Emerson K., and Thorn G. W. (1958) ACTH-producing tumor of the pituitary gland.N. Engl. J. Med. 259, 161–164.
Nelson D. H., Meakin J. W., and Thorn G. W. (1960) ACTH-producing pituitary tumors following adrenalectomy for Cushing’s syndrome.Ann. Intern. Med. 52, 560–569.
Nelson D. H., Sprunt J. G., and Mims R. B. (1966) Plasma ACTH determinations in 58 patients before or after adrenalectomy for Cushing’s syndrome.J. Clin. Endocrinol. 26, 722–728.
Webb S. M., Ruscalleda J., Schwarzstein D., Calaf-Alsina J., Rovira A., Matos G., Puig-Domingo M., and de Leiva A. (1992) Computerized tomography versus magnetic resonance imaging: a comparative study in hypothalamic-pituitary and parasellar pathology.Clin. Endocr. 36, 459–465.
Wislawski J., Kasperlik-Zaluska A. A., Jeske W., Migdalska B., Janik J., Zaluska J., and Bonicki W. (1985) Results of neurosurgical treatment by a transsphenoidal approach in 10 patients with Nelson’s syndrome.J. Neurosurg. 62, 68–71.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kasperlik-Zaluska, A.A., Walecki, J., Jeske, W. et al. Early diagnosis of Nelson’s syndrome. J Mol Neurosci 7, 87–90 (1996). https://doi.org/10.1007/BF02736788
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02736788