Acute Psychosis As the Initial Manifestation of ACTH-Dependent Cushing's Syndrome

Presentation Number: SUN-0775
Date of Presentation: June 22nd, 2014

Pooja Raghavan*1, Andrea Kassai2, Susmeeta T. Sharma3, Maya Peltsverger4 and Lynnette K. Nieman2
1National Institutes of Health, North Bethesda, MD, 2National Institutes of Health, Bethesda, MD, 3Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, 4New Hanover Regional Medical Center, Wilmington, NC


Introduction: Cushing’s syndrome (CS) is associated with a variety of psychiatric disturbances; depression is the commonest, mania and psychosis are rare. We report 3 patients with ACTH-dependent CS who presented with acute psychosis. None of them had a prior history of psychiatric dysfunction. All had resolution of symptoms once eucortisolemia was achieved.

Clinical cases:

Case-1: 35 y.o. female presented with a 2-week history of mania and agitation. Over the next 5 years, she developed hypertension (HTN), prediabetes, amenorrhea, 75lb weight gain, and severe proximal muscle weakness and was admitted 5 times with visual and auditory hallucinations controlled partially on antipsychotics. Endocrine evaluation revealed ACTH-dependent CS (UFC=1,009 mcg/d, normal: 3.5-45). CRH stimulation test and a 9mm lesion on MRI Pituitary suggested a pituitary source. Transsphenoidal resection (TSR) of the ACTH-staining adenoma led to remission of CS and regression of psychosis.

Case-2: 40 y.o. female presented with frequent panic attacks unresponsive to sertraline treatment. She was later diagnosed with depression and bipolar disorder and required 18day hospitalization for paranoid delusions and agitation. Development of HTN, type 2 diabetes mellitus, amenorrhea, 60lb weight gain, hirsutism, and proximal muscle weakness led to the diagnosis of ACTH-dependent CS (UFC=137 mcg/d). MRI pituitary along with CRH stimulation and dexamethasone suppression tests indicated a pituitary source. TSR of an ACTH-staining adenoma led to remission of CS with resolution of psychosis and improvement in depression.

Case-3: 39 y.o. male presented with mania, agitation, combative behavior, 35lb weight gain and HTN (200/100 mmHg) requiring hospitalization for 10 days. Endocrine evaluation revealed cyclic ACTH-dependent CS (UFC=2,365 mcg/d). Diagnostic tests including inferior petrosal sinus sampling, performed in the setting of hypercortisolemia, suggested an ectopic source. Structural and functional imaging studies failed to identify the source of ACTH. Eucortisolemia was achieved on ketoconazole therapy (800mg/d) with resolution of psychosis.

Conclusion: Acute psychosis can occasionally be the initial manifestation in CS, regardless of the degree of hypercortisolemia. Recognition of this association can lead to early diagnosis of CS. Control of hypercortisolemia, via surgical or medical therapy, along with antipsychotic medications can help ameliorate psychiatric symptoms.


Nothing to Disclose: PR, AK, STS, MP, LKN