Hypothalamic syndrome

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 h...

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Bibliographic Details
Published in:Nature reviews. Disease primers 2022-04, Vol.8 (1), p.24-24, Article 24
Main Authors: Müller, Hermann L, Tauber, Maithé, Lawson, Elizabeth A, Özyurt, Jale, Bison, Brigitte, Martinez-Barbera, Juan-Pedro, Puget, Stephanie, Merchant, Thomas E, van Santen, Hanneke M
Format: Article
Language:English
Subjects:
Brain cancer
Circadian rhythm
Craniopharyngioma
Cysts
Disease
Endocrine System Diseases - complications
Epidemiology
Females
Human health and pathology
Humans
Hypothalamus
Life Sciences
Medical prognosis
Metabolism
Mortality
Obesity
Pediatrics
Pituitary Neoplasms - complications
Population
Prader-Willi Syndrome - complications
Prader-Willi Syndrome - diagnosis
Prader-Willi Syndrome - therapy
Traumatic brain injury
Tumors
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Summary: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.
ISSN:2056-676X
2056-676X
DOI:10.1038/s41572-022-00351-z