01 March 2002
Case of Cushing’s syndrome due to ACTH-independent bilateral
micronodular adrenal hyperplasia presenting as a severe myopathy with diaphragm atrophy
Sylwia F. Kaźmierczak, Wojciech Zieleniewski, Krzysztof Kołomecki, Jolanta Kunert-Radek
Case Rep Clin Pract Rev 2002; 3(1):8-11 :: ID: 474864
Abstract
Background: The most typical presentation of Cushing’s syndrome includes truncal obesity, purple skin striae, moon face, emotional and sleep disturbances, plethora and proximal muscle weakness. ACTH-independent
micronodular adrenocortical hyperplasia (AIMAH) is a rare cause of Cushing’s syndrome with male predominance. We describe here a female patient with atypical Cushing’s syndrome due to AIMAH presenting as a severe
myopathy with diaphragm atrophy.
Case report: A 56-year-old female suffered from hypertension, NIDDM, central adiposity and muscle weakness for 5 years, but her appearance was not typically cushingoid. She was admitted to hospital because of respiratory
insufficiency due to diaphragm atrophy. Reconstructive surgery of the diaphragm was performed, but without any significant improvement. Six months later, she was referred to us with suspicion of Cushing’s syndrome.
Her ACTH levels in plasma samples collected throughout the day were below normal. The cortisol level was at upper limit of the normal range. The plasma circadian cortisol profile was blunted and 1 mg dexamethasone did not induce any inhibition of serum cortisol. The subsequent administration of 8 mg of dexamethasone did not cause any further suppression of cortisol. While these findings were consistent with a diagnosis of adrenocortical
adenoma, computed tomography demonstrated slightly enlarged, contrast-enhanced adrenals (both adrenal glands).
Results: ACTH-independent Cushing’s syndrome was diagnosed and the patient underwent bilateral adrenalectomy. Histological studies revealed bilateral micronodular adrenal hyperplasia. Soon after the surgery the woman
became hypoadrenal and received replacement therapy. Hypertension, NIDDM, myopathy and respiratory comfort improved.
Conclusions: Hypercortisolemia may infrequently present as atypical clinical syndrome. Patients with unclear muscle atrophy, especially accompanied by NIDDM and hypertension should by screened for Cushing’s syndrome.
Keywords: adrenal hyperplasia, Cushing’s syndrome, diaphragm atrophy
44
In Press
19 Mar 2024 : Case report
Bilateral Simultaneous Basal Ganglia Hemorrhage: A Case ReportAm J Case Rep In Press; DOI: 10.12659/AJCR.943174
19 Mar 2024 : Case report
Medial Hoffa Fracture: A Case Report and Literature Review of Approach and ManagementAm J Case Rep In Press; DOI: 10.12659/AJCR.943136
21 Mar 2024 : Case report
Pediatric Neurogenic Pulmonary Edema After Brain Tumor Removal Complicated with Severe Myocardial Injury: A...Am J Case Rep In Press; DOI: 10.12659/AJCR.943645
21 Mar 2024 : Case report
Chlamydia psittaci Pneumonia: Diagnosis, Treatment, and Challenges in the Context of COVID-19Am J Case Rep In Press; DOI: 10.12659/AJCR.942921
Most Viewed Current Articles
07 Mar 2024 : Case report
Neurocysticercosis Presenting as Migraine in the United StatesDOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
10 Jan 2022 : Case report
A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...DOI :10.12659/AJCR.935263
Am J Case Rep 2022; 23:e935263
19 Jul 2022 : Case report
Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19DOI :10.12659/AJCR.936128
Am J Case Rep 2022; 23:e936128
23 Feb 2022 : Case report
Penile Necrosis Associated with Local Intravenous Injection of CocaineDOI :10.12659/AJCR.935250
Am J Case Rep 2022; 23:e935250