J. Endocrinol. Invest. 30: 776-779, 2007
776
Key-words: Cushing’s syndrome, hypercortisolism, psychotic disorders,
etomidate, mifepristone.
Correspondence: Y.M. Bilgin, MD, Department of Internal Medicine, IJssel-
land hospital, PO Box 690, 2900 AR Capelle aan den Ijssel, The Netherlands.
E-mail: bilginyavuz@hotmail.com
Accepted November 2, 2006.
CASE REPORT
Treatment of severe psychosis due to ectopic Cushing’s
syndrome
Y.M. Bilgin
1
, H.E. van der Wiel
1
, H.R.A. Fischer
1
, and W.W. De Herder
2
1
Department of Internal Medicine, IJsselland Hospital, Capelle aan den IJssel;
2
Department of Internal
Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
ABSTRACT. Severe psychosis in patients with Cush-
ing’s syndrome is rare and generally difficult to
treat. We report a 46-yr-old woman suffering from
Cushing’s syndrome caused by an inoperable ACTH-
producing lung carcinoma. She was initially treated
with chemotherapy and radiotherapy. Six months
later she presented with severe psychosis. Labora-
tory findings revealed a severe hypokaliemia and
metabolic alkalosis, which was caused by extremely
high serum ACTH (788 ng/l) and cortisol (4.2 mu-
mol/l). She was unresponsive to treatment with con-
ventional antipsychotic drugs; she was therefore
sedated and intubated. Treatment was started iv
with etomidate, which blocks the cortisol synthesis,
and orally by nasogastric tube with mifepristone,
which competes with cortisol for binding to their
receptors. To counteract adrenal insufficiency, she
received corticosteroids. After 5 days there was a
normalization of the ACTH, cortisol levels, and the
metabolic disorders. After discontinuing etomidate
she was extubated; there were no signs of psycho-
sis observed. Computed tomography (CT) scan of
the brain showed no metastasis, however CT scan
of the abdomen showed liver metastasis and bilat-
eral adrenal enlargement. Unfortunately, the clini-
cal situation worsened and the patient died due
to progression of the metastasis. This case report
demonstrates the efficacy of a treatment of mife-
pristone with etomidate in a patient with an ectopic
ACTH-producing Cushing’s syndrome.
(J. Endocrinol. Invest. 30: 776-779, 2007)
©
2007, Editrice Kurtis
INTRODUCTION
Psychiatric disturbances in patients with Cushing’s
syndrome have frequently been observed. Most of
the patients suffer from depression; severe psychosis
in patients with Cushing’s syndrome is very rare (1).
Psychosis in Cushing’s syndrome is generally diffi-
cult to treat and in most cases there is little or no
response to antipsychotic drugs.
Mifepristone (RU 486) is an antiprogestin, which
at higher dosages competes with glucocorticoids
for binding to their receptor. Oral mifepristone
has been successfully used for the treatment of
acute psychosis in patients with Cushing’s syn-
drome (2-4).
Etomidate, a substituted imidazole, is a hypnotic
drug, which blocks the enzyme 11- -hydroxylase,
that catalyses the production of cortisol from 11-
deoxycortisol. It lowers serum cortisol levels within
10-12 h. It has been administered in low doses iv to
hospitalized patients with Cushing’s syndrome who
cannot be treated with oral medication (5, 6).
Here we report the combined use of mifepristone
and etomidate in a patient with severe psychosis
caused by an ectopic ACTH-producing lung carci-
noma and Cushing’s syndrome.
CASE REPORT
A 46-yr-old woman was presented to our hospital with
severe psychosis. One year before admission she had
been diagnosed with Cushing’s syndrome caused by
an inoperable ectopic ACTH-producing small cell
carcinoma of the lung. She had been treated with 3
cycles of chemotherapy (carboplatin and paclitaxel)
and external radiotherapy on the lung tumor (15 x
3 Gy). After this therapy, there was no further tumor
regression and there were no signs of metastasis.