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Gazzetta Medica Italiana Archivio per le Scienze Mediche 2012 August;171(4):523-7

Copyright © 2012 EDIZIONI MINERVA MEDICA

language: English

Severe congestive heart failure caused by adrenomyeloneuropathy and hyperthyroidism

Kretzschmar D. 1, Guenther A. 2, Hunger-Battefeld W. 3, Yilmaz A. 1, Pfeifer R. 1, Mall G. 4, Jung C. 1, Drescher R. 5, Hüge S. 2, Ferrari M. 1

1 Division of Cardiology and, Intensive Care Medicine, Department of Internal Medicine I, University Hospital Jena, Jena, Germany; 2 Hans-Berger-Clinic of Neurology, University Hospital Jena, Jena, Germany; 3 Division of Endocrinology and Nephrology, Department of Internal Medicine III, University Hospital Jena, Jena, Germany: 4 Institute of Pathology, Hospital Darmstadt, Darmstadt, Germany; 5 Division of Neuroradiology, Institute of Radiology, University Hospital Jena, Jena, Germany


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Adrenomyeloneuropathy (AMN) is a peroxisomal disorder that leads to demyelination of the nervous system and adrenal insufficiency due to accumulation of long-chain fatty acids in the tissue. Congenital or acquired disorders of the thyroid gland causing hyperthyroidism leading to severe congestive heart failure have not yet been described in AMN. A 44-year-old man presented with weakness, lethargy and vomiting. He suffered from spastic paraparesis in his lower extremities which had been slowly progressive in the past 15 years. An AMN with adrenal insufficiency was diagnosed for which cortison was administered. At the same time, he developed a severe hyperthyroidism due to autoimmune thyroiditis. Due to hemodynamic deterioration he was transferred to the intensive care unit. A highly impaired left ventricular function was observed. Heart failure rapidly deteriorated. Administration of levosimendan improved the hemodynamic situation and the clinical status. The association of autoimmune thyroiditis causing severe hyperthyroidism with adrenal insufficiency in conjunction with AMN has not been described previously.

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