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Hypothalamic syndrome

Müller, Hermann L; Tauber, Maithé; Lawson, Elizabeth A; Özyurt, Jale; Bison, Brigitte; Martinez-Barbera, Juan-Pedro; Puget, Stephanie; ... van Santen, Hanneke M; + view all (2022) Hypothalamic syndrome. Nature Reviews Disease Primers , 8 (1) , Article 24. 10.1038/s41572-022-00351-z. Green open access

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Abstract

Hypothalamic syndrome (HS) is a rare disorder caused by disease-related and/or treatment-related injury to the hypothalamus, most commonly associated with rare, non-cancerous parasellar masses, such as craniopharyngiomas, germ cell tumours, gliomas, cysts of Rathke's pouch and Langerhans cell histiocytosis, as well as with genetic neurodevelopmental syndromes, such as Prader-Willi syndrome and septo-optic dysplasia. HS is characterized by intractable weight gain associated with severe morbid obesity, multiple endocrine abnormalities and memory impairment, attention deficit and reduced impulse control as well as increased risk of cardiovascular and metabolic disorders. Currently, there is no cure for this condition but treatments for general obesity are often used in patients with HS, including surgery, medication and counselling. However, these are mostly ineffective and no medications that are specifically approved for the treatment of HS are available. Specific challenges in HS are because the syndrome represents an adverse effect of different diseases, and that diagnostic criteria, aetiology, pathogenesis and management of HS are not completely defined.

Type: Article
Title: Hypothalamic syndrome
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1038/s41572-022-00351-z
Publisher version: https://doi.org/10.1038/s41572-022-00351-z
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Craniopharyngioma, Endocrine System Diseases, Humans, Hypothalamus, Pituitary Neoplasms, Prader-Willi Syndrome
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10161539
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