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DOI: 10.1055/s-2002-33075
© Johann Ambrosius Barth
Psychoneuroendocrine disturbances in a patient with a rare granulomatous disease
Publikationsverlauf
received 26 September 2001
first decision 20 November 2001
accepted 14 February 2002
Publikationsdatum:
30. Juli 2002 (online)
![](https://www.thieme-connect.de/media/eced/200205/lookinside/thumbnails/10.1055-s-2002-33075-1.jpg)
Summary
Erdheim-Chester disease (ECD) and Langerhans' cell histiocytosis (LCH) are rare granulomatosis-like diseases of unknown etiology which are characterized by lipoidgranulomatous infiltrates in various organs. Contrary to LCH, endocrine and cerebral lesions were infrequently observed in ECD. We report on a patient with the clinical diagnosis of ECD displaying endocrine and cerebral manifestations and skeletal, pulmonary and soft tissue involvement. Disturbance of the endocrine system was revealed by enlargement of the pituitary, partial deficiency of growth hormone (GH), hyperprolactinemia and testosterone deficiency. Cerebral involvement included sinus vein thrombosis, pathologic acoustic evoked potentials, persistence of gadolinium enhancement after magnetic resonance imaging and hypomania. These findings emphasize the importance to assess endocrine and cerebral function in patients with rare granulomatous diseases like ECD and multiorgan involvement.
Key words:
Erdheim-Chester - Langerhans' cell histiocytosis - Xanthogranulomatosis - Central nervous system - Endocrine
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