[en] Several subgroups of Cushing's disease were recently described (anterior or intermediate lobe origin, hyper-or hypo-pulsatility of cortisol, presence or absence of response after GRH or TRH, cyclical Cushing's disease). We present here a detailed case report on a patient suffering from Cushing's disease whose endocrine functions were extensively investigated. Treatment with bromocriptine, as well as subsequent transsphenoidal surgery, were followed by rapid but transient reversal of symptoms. When clinical manifestations reoccurred, daily measurements of free urinary cortisol revealed a cyclic pattern of cortisol hyperexcretion. A study of ultradian rhythm revealed hyperpulsatility of cortisol secretion. More interestingly, a treatment with sodium valproate, a drug known to inhibit CRH production, was followed by a rapid and longstanding normalization of clinical and biological data for 2 years. Based on these data, and on information from the literature, the present case of Cushing's disease exhibits characteristics suggesting a possible hypothalamic origin.
Disciplines :
Endocrinology, metabolism & nutrition
Author, co-author :
Beckers, Albert ; Université de Liège - ULiège > Département des sciences cliniques > Endocrinologie
Stevenaert, Achille ; Centre Hospitalier Universitaire de Liège - CHU > Neurochirurgie
Pirens, G.
Flandroy, P.
Sulon, Joseph
Hennen, Georges ; Université de Liège - ULiège > Endocrinologie clinique
Language :
English
Title :
Cyclical Cushing's disease and its successful control under sodium valproate.
Lamberts S.W.J., De Lange S.A., Stefanko S.Z. (1982) Adenocorticotropin-secreting pituitary adenomas originate from the anterior or the intermediate lobe in Cushing’s disease: differences in the regulation of hormone secretion. J. Clin. Endocrinol. Metab. 54:286.
Van Cauter E., Refetoff S. (1985) Evidence for two subtypes of Cushing’s disease on the analysis of episodic Cortisol secretion. N. Engl. J. Med. 312:1343.
Brown R.D., Van Loon G.R., Orth D.N., Liddle G.W. (1973) Cushing’s disease with periodic hormonogenesis: one explanation for paradoxical response to dexa-methasone. J. Clin. Endocrinol. Metab. 36:445.
Atkinson A.B., Chestnutt A., Crothers E., Woods R., Weaver J.A., Kennedy L., Sheridan B. (1985) Cyclical Cushing’s disease: two distinct rhythms in a patient with a basophil adenoma. J. Clin. Endocrinol. Metab. 60:328.
Atkinson A.B., Kennedy A.L., Carson D.J., Hadden D.R., Weaver J.A., Sheridan B. (1985) Five cases of cyclical Cushing’s syndrome. Br. Med. J. 291:1453.
Merriam G., Wachter K. (1982) Algorythms for the study of episodic hormone secretion. Am. J. Physiol. 43:E310.
Knott G., Reece D. (1972) MLAB: A civilized curve fitting system. Proceedings of the online 72 International Conference, Vol. 1 , Brunei University, London; 497.
Stevenaert A., Beckers A., Vandalem J.L., Hennen G. (1986) Early normalization of luteinizing hormone pulsatility after successful transsphenoidal surgery in women with microprolactinomas. J. Clin. Endocrinol. Metab. 62:1044.
Sternberger L.A., Hardy P.H., Cuculis J., Meyer H.G. (1970) The unlabeled antibody enzyme method of immuno-chemistry. Preparation and properties of soluble antigen-antibody complex (Horseradish peroxidase) and its use in the identification of spirochetes. J. Histochem. Cytochem. 18:315.
Pieters G.F.F.M., Smals A.G.H., Goverde H.J.M., Pesman G.J., Meyer E. (1982) Kloppenborg P.W.C. Adrenocorticotropin and Cortisol responsiveness to thyrotropin-releasing hormone and luteinizing hormone-releasing hormone discloses two subsets of patients with Cushing’s disease. J. Clin. Endocrinol. Metab. 55:1188.
Bailey R.E. (1971) Periodic hormonogenesis: a new phenomenon. Periodicity in function of a hormone-producing tumor in man. J. Clin. Endocrinol. Metab. 32:317.
Liberman B., Wajchenberg B., Tambascia M.A., Mesquita C.H. (1976) Periodic remission in Cushing’s disease with paradoxical dexamethasone response: an expression of periodic hormono-genesis. J. Clin. Endocrinol. Metab. 43:913.
Jordan R.M., Ramos-Gabatin A., Kendall J.W., Gaudette D., Walls R.C. (1982) Dynamics of adrenocorticotropin (ACTH) secretion in cyclic Cushing’s syndrome: evidence for more than one abnormal ACTH biorhythm. J. Clin. Endocrinol. Metab. 55:531.
Birke G., Diczfalusy E., Plantin L. (1956) Fluctuations in the excretion of adrenocortical steroids in a case of Cushing’s syndrome. J. Clin. Endocrinol. Metab. 16:286.
Chajek T., Romanoff H. (1976) Cushing’s syndrome with cyclical edema and periodic secretion of corticosteroids. Arch. Intern. Med. 136:441.
Oates T.W., Mc Court J.P., Friedman W.A., Agee C.F., Rhoton A.L., Thomas W.C. (1979) Cushing’s disease with cyclical hormonogenesis and diabeted insipidus. Neurosurgery 5:598.
Halberg F. (1969) Chronobiology. Annual Review of Physiology 31:675.
Dornhorst A., Jenkins J.S., Lamberts S.W.J., Abraham R.R., Wynn V., Beckford U., Gillham B., Jones M.T. (1983) The evaluation of sodium valproate in the treatment of Nelson’s syndrome. J. Clin. Endocrinol. Metab. 56:985.
Loli P., Berselli M.E., Vignati F., De Grandi C., Tagliaferri M. (1988) Size reduction of an ACTH-secreting pituitary macroadenoma in Nelson’s syndrome by sodium valproate: effect withdrawal and reinstitution of treatment. Acta Endocrinol. (Copenh.) 119:435.
Koppeschaar H.P.F., Croughs R.J.M., Thijssen J.H.H., Schwarz F. (1983) Sodium valproate and cyproheptadine may independently induce a remission in the same patient with Cushing’s disease. Acta Endocrinol. (Copenh.) 104:160.
Koppeschaar H.P.F., Croughs R.J.M., Van’t Verlaat J.W., Hendriks M.J., Arts C.J.M., Thijssen J.H.H., Schwarz F. (1984) Successful treatment with sodium valproate of a patient with Cushing’s disease and gross enlargement of pituitary. Acta Endocrinol. (Copenh.) 107:471.
Koppeschaar H.P.F., Croughs R.J.M., Thijssen J.H.H., Schwarz F. (1986) Response to neurotransmitter modulating drugs in patients with Cushing’s disease. Clin. Endocrinol. (Oxf.) 25:661.
Cavagnini F., Invitti C., Polli E.E. (1984) Sodium valproate in Cushing’s disease. Lancet 2:162.
Jones M.T., Gillham B., Altaher A.R.H., Nicholson S.A., Campbell E.A., Watts S.M., Thody A. (1984) Clinical and experimental studies on the role of gaba in the regulation of ACTH secretion: a review. Psychoneuroendocrinology 9:107.
Lamberts S.W.J., Verleu T., Bons E.G., Uitterlinden P., Oosterom R. (1983) Effect of cyproheptadine, desmethylcyproheptadine, gamma-aminobutyric acid and sodium valproate on adrenocorticotrophin secretion by cultured pituitary tumors cells from three patients with Nelson’s syndrome. J. Endocrinol. 96:401.
Regan C.M. (1985) Therapeutic levels of sodium valproate inhibit mitotic indices in cells of neural origin. Brain Res. 347:394.
Nussey S.S., Price P., Jenkins J.S., Altaher A.R., Gillham B., Jones M.T. (1988) The combined use of sodium valproate and metyrapone in the treatment of Cushing’s syndrome. Clin. Endocrinol. (Oxf.) 28:373.
