Severe myopathy and psychosis in a patient with Cushing's disease macroadenoma.
Cushing's disease is most commonly caused by a corticotrope adenoma of the pituitary. Between 50 and 70% of patients with spontaneous hypercortisolism have ACTH-producing pituitary adenomas. The tumors are usually microadenomas with approximately 20% of patients with the disease showing no evidence of tumor on CT-scans or MR imaging of their pituitary glands. In contrast to patients with ectopic ACTH production, plasma ACTH concentrations in patients with spontaneous disease are generally within the normal range. We describe here a patient with a pituitary macroadenoma that showed evidence of necrosis on MRI. The patient had an atypical clinical presentation with plasma ACTH levels considerably higher than that seen in patients with non-ectopic ACTH-secretory syndrome, markedly elevated urine free cortisol, lack of phenotypical signs of hypercortisolism such as wide purplish striae, and whose most prominent and distressing symptom was severe myopathy that resulted in the patient becoming bed-ridden. Psychosis was another striking feature in this patient who during his hospital course developed multiple opportunistic infections that contributed to his demise.
Tran
Minou
M
Department of Medicine, Division of Endocrinology, Diabetes and Metabolism, UC, Irvine Medical Center, 101 City Drive South, Orange, CA 92868, USA.
Elias
Alan N
AN
D000182:ACTH Syndrome, Ectopic / Q000097:blood / Q000175:diagnosis; D000236:Adenoma / Q000097:blood / Q000175:diagnosis* / Q000601:surgery; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D003480:Cushing Syndrome / Q000097:blood / Q000175:diagnosis* / Q000601:surgery; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D009133:Muscular Atrophy / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D009336:Necrosis; D018365:Neoplasm, Residual / Q000097:blood / Q000175:diagnosis; D019965:Neurocognitive Disorders / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D009894:Opportunistic Infections / Q000175:diagnosis / Q000209:etiology; D010902:Pituitary Gland / Q000473:pathology / Q000601:surgery; D010911:Pituitary Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000601:surgery; D011618:Psychotic Disorders / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone