An Ectopic Cushing’s Syndrome with Severe Psychiatric Presentation

an-ectopic-cushingrsquos-syndrome-with-severe-psychiatric-presentation-9744

We report a case of severe EAS in a young Tunisian man resulting from a well differentiated Neuroendocrine Tumor (NET) of the lung. Besides catabolic signs and profound hypokalemia orienting towards Cushing’s Syndrome (CS), psychiatric symptoms were particularly severe, dominant and atypical including persecutory delusions, depression and anxiety.

Etomidate in the Treatment Of Cushing Syndrome

Cushing syndrome is a metabolic disease caused by chronic exposure to high levels of glucocorticoids. It can present as an endocrine emergency due to a rapid increase in circulating cortisol leading to increased risk of cardiovascular disease and infection. Etomidate rapidly reduces plasma cortisol levels by inhibiting the action of 11β-hidroxilase. We report the case of a patient with severe hypercortisolaemia accompanied by metabolic and psychiatric disorders in whom administration of etomidate reduced preoperative levels of cortisol.

Introduction

Cushing’s syndrome is a metabolic disease caused by chronic exposure to high levels of glucocorticoids. The main causes are ectopic ACTH secretion, adrenal tumours (adenomas or carcinomas), adrenal hyperplasia, and administration of exognous glucocorticoids—the latter being the most common aetiology.1

In most cases, Cushing’s syndrome presents an indolent course for years before diagnosis is made, although it can sometime present as an endocrine emergency due to a rapid increase in circulating cortisol levels.2 In these cases, treatment to control hypercortisolaemia must be started quickly due to the high morbidity and mortality associated with the potentially life-threatening metabolic, infectious, and neuropsychiatric alterations that occur in this syndrome.1, 2, 3, 4

The options for treating Cushing’s syndrome include surgery, radiotherapy, and pharmacological treatment. The most commonly used drugs are adrenal steroidogenesis inhibitors (ketoconazole, metyrapone),3 but this treatment is not always well tolerated and its efficacy is limited.2 Etomidate is a drug from the imidazole family that inhibits the enzyme 11β-hydroxylase, and can reduce cortisol secretion within 48−72 h.2

Section snippets

Case report

Our patient was a 27-year-old woman with no known drug allergies or personal history of interest. She was studied in April 2021 for anxious-depressive symptoms with rapidly evolving paranoid ideation and hirsutism. A Nugent test was performed, which was positive (46.1 mcg/dl), and cortisol in urine was measured (2715 mcg/24 h), leading to a diagnosis of Cushing’s syndrome.

A CT scan showed a large mass on the right adrenal gland, compatible with a primary adrenal gland tumour (Fig. 1).

Discussion

Endogenous Cushing’s syndrome is characterized by over-production of cortisol. In patients such as ours, the syndrome presents in its most serious form, with very high hypercortisolaemia and metabolic, cardiovascular, and neuropsychiatric disorders. Cushing’s syndrome is a medical emergency due to its association with several comorbidities and its high rate of mortality.5 The first therapeutic option is surgical resection of the underlying tumour; however, the accompanying hypercortisolaemia

Conclusion

In its severe form, Cushing’s syndrome is a medical emergency that must be rapidly controlled.

Etomidate is both safe and effective, and has shown promising results in the treatment of severe hypercortisolaemia.

We believe that these patients should be admitted to the Anaesthesia Intensive Care Unit during etomidate therapy in order to monitor their level of consciousness, lung function, and haemodynamics, and to closely monitor cortisol and electrolyte levels.

Ethical considerations

Informed consent was obtained for the use of patient information for teaching and research purposes in accordance with our hospital protocol.

Conflict of interests

None.

Funding

The authors have not received any funding for this manuscript.

References (8)

  • A. Ferriere et al.

    Cushing’s syndrome: Treatment and new therapeutic approaches

    Best Pract Res Clin Endocrinol Metab

    (2020)
  • Juszczak A, Morris D, Grossman A. Cushing’s Syndrome [Internet]. South Dartmouth (MA): MDText.com, Inc; 2000 [revised…
  • T.B. Carroll et al.

    Continuous Etomidate Infusion for the Management of Severe Cushing Syndrome: Validation of a Standard Protocol

    J Endocr Soc

    (2018)
  • V.A. Preda et al.

    Etomidate in the management of hypercortisolaemia in Cushing’s syndrome: a review

    Eur J Endocrinol

    (2012)
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© 2023 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España, S.L.U. All rights reserved.

Psychotropic drugs in patients with Cushing’s disease before diagnosis and at long-term follow-up

The Journal of Clinical Endocrinology & Metabolism, dgab079, https://doi.org/10.1210/clinem/dgab079

Abstract

Context

Psychiatric symptoms are common in Cushing’s disease (CD) and seem only partly reversible following treatment.

Objective

To investigate drug dispenses associated to psychiatric morbidity in CD patients before treatment and during long-term follow-up.

Design

Nationwide longitudinal register-based study.

Setting

University Hospitals in Sweden.

Subjects

CD patients diagnosed between 1990 and 2018 (N=372) were identified in the Swedish Pituitary Register. Longitudinal data was collected from 5 years before, at diagnosis and during follow-up. Four matched controls per patient were included. Cross-sectional subgroup analysis of 76 patients in sustained remission was also performed.

Main outcome measures

Data from the Swedish Prescribed Drug Register and the Patient Register.

Results

In the 5-year period before, and at diagnosis, use of antidepressants (OR 2.2[95%CI 1.3-3.7] and 2.3[1.6-3.5]), anxiolytics (2.9[1.6-5.3] and 3.9[2.3-6.6]) and sleeping pills (2.1[1.2-3.7] and 3.8[2.4-5.9]) was more common in CD than controls. ORs remained elevated at 5-year follow-up for antidepressants (2.4[1.5-3.9]) and sleeping pills (3.1[1.9-5.3]). Proportions of CD patients using antidepressants (26%) and sleeping pills (22%) were unchanged at diagnosis and 5-year follow-up, whereas drugs for hypertension and diabetes decreased. Patients in sustained remission for median 9.3 years (IQR 8.1-10.4) had higher use of antidepressants (OR 2.0[1.1-3.8]) and sleeping pills (2.4[1.3-4.7]), but not of drugs for hypertension.

Conclusions

Increased use of psychotropic drugs in CD was observed before diagnosis and remained elevated regardless of remission status, suggesting persisting negative effects on mental health. The study highlights the importance of early diagnosis of CD, and the need for long-term monitoring of mental health.

Identification Of Potential Markers For Cushing’s Disease

Endocr Pract. 2016 Jan 20. [Epub ahead of print]

Abstract

OBJECTIVE:

Cushing’s disease (CD) causes a wide variety of nonspecific symptoms, which may result in delayed diagnosis. It may be possible to uncover unusual combinations of otherwise common symptoms using ICD-9-CM codes. Our aim was to identify and evaluate dyads of clinical symptoms or conditions associated with CD.

METHODS:

We conducted a matched case-control study using a commercial healthcare insurance claims database, designed to compare the relative risk (RR) of individual conditions and dyad combinations of conditions among patients with CD versus matched non-CD controls.

RESULTS:

With expert endocrinologist input, we isolated 10 key conditions (localized adiposity, hirsutism, facial plethora, polycystic ovary syndrome, abnormal weight gain, hypokalemia, deep venous thrombosis, muscle weakness, female balding, osteoporosis) with RR varying from 5.1 for osteoporosis to 27.8 for hirsutism. The RR of dyads of these conditions ranged from 4.1 for psychiatric disorders/serious infections to 128.0 for hirsutism/fatigue in patients with vs. without CD. Construction of uncommon dyads resulted in further increases in RR beyond single condition analyses, such as osteoporosis alone had RR of 5.3, which increased to 8.3 with serious infections and to 52.0 with obesity.

CONCLUSION:

This study demonstrated that RR of any one of 10 key conditions selected by expert opinion was ≥5 times greater in CD compared to non-CD, and nearly all dyads had RR≥5. An uncommon dyad of osteoporosis and obesity had an RR of 52.0. If clinicians consider the diagnosis of CD when the highest-risk conditions are seen, identification of this rare disease may improve.

KEYWORDS:

Cushing’s disease; delay in diagnosis; disease markers; insurance claims; relative risk

PMID:
26789346
[PubMed – as supplied by publisher]

From http://www.ncbi.nlm.nih.gov/pubmed/26789346

Addison’s disease may cause psychosis, say researchers

adrenal-glands

 

Research suggests that chronic adrenal insufficiency, more commonly known as Addison’s disease, may be responsible for psychiatric symptoms in those who suffer with it. Unfortunately, these symptoms are poorly understood and inadequately studied. In one case, a 41-year-old construction worker was admitted to a psychiatric clinic complaining of depression. He had trouble sleeping and concentrating and had lost 6 pounds due to a loss of appetite. He was placed on 20mg of fluoxetine but returned 2 weeks later complaining that the therapy did not work, and even reported hallucinating his ex-wife, who had recently died in a car accident. He returned again later 4 months later and was found to have a weak pulse, major hypotension, and hyponatremia and hyperkalemia. It was at this point that he was diagnosed with Addison’s disease.

The disease was first described by Thomas Addison in the mddle of the 19th century. It involves inadequate secretion from the adrenal glands, leading to lower secretion of glucocorticoids. Its usual symptom pigmentation involves fatigue, weight loss, nausea, vomiting, weakness and abdominal pain. Among its psychiatric symptoms are psychosis and delirium.