Hypothalamic syndrome.
Hermann L MüllerMaïthé TauberElizabeth A LawsonJale ÖzyurtBrigitte BisonJuan Pedro Martínez-BarberaStephanie PugetThomas E MerchantHanneke M van SantenPublished in: Nature reviews. Disease primers (2022)
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.
Keyphrases
- weight gain
- weight loss
- metabolic syndrome
- case report
- insulin resistance
- body mass index
- type diabetes
- birth weight
- germ cell
- minimally invasive
- emergency department
- high grade
- bariatric surgery
- stem cells
- early onset
- coronary artery bypass
- single cell
- magnetic resonance imaging
- skeletal muscle
- bone marrow
- coronary artery disease
- genome wide
- atrial fibrillation
- optical coherence tomography
- contrast enhanced
- replacement therapy
- surgical site infection