TY - JOUR
T1 - Hypothalamic syndrome
AU - Müller, Hermann L.
AU - Tauber, Maithé
AU - Lawson, Elizabeth A.
AU - Özyurt, Jale
AU - Bison, Brigitte
AU - Martinez-Barbera, Juan Pedro
AU - Puget, Stephanie
AU - Merchant, Thomas E.
AU - van Santen, Hanneke M.
N1 - © 2022. Springer Nature Limited.
PY - 2022/4/21
Y1 - 2022/4/21
N2 - 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.
AB - 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.
KW - Craniopharyngioma
KW - Endocrine System Diseases/complications
KW - Humans
KW - Hypothalamus
KW - Pituitary Neoplasms/complications
KW - Prader-Willi Syndrome/complications
UR - http://www.scopus.com/inward/record.url?scp=85128632552&partnerID=8YFLogxK
UR - https://www.mendeley.com/catalogue/ea06b60d-46ef-36dc-91e9-01a3e5093e77/
U2 - 10.1038/s41572-022-00351-z
DO - 10.1038/s41572-022-00351-z
M3 - Article
C2 - 35449162
AN - SCOPUS:85128632552
SN - 2056-676X
VL - 8
SP - 24
JO - Nature reviews. Disease primers
JF - Nature reviews. Disease primers
IS - 1
ER -