Hypothalamic syndrome
Open accessAbstract
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.
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Other tumors, such as low-grade gliomas and germinomas, also confer increased risk of acquired hypothalamic syndrome.
“As previously summarized, craniopharyngioma is the prototypic suprasellar tumor leading to acquired hypothalamic syndrome, but other tumors (eg, low-grade gliomas, germinomas) also confer increased risk.”
From Approach to the patient with acquired hypothalamic syndrome (2026) · cites this paper as [2]
Injury to the hypothalamus, whether from tumors affecting this brain area or their treatment, leads to acquired hypothalamic syndrome in approximately 80% of individuals, depending in large part on tumor characteristics.
“Injury to the hypothalamus, whether from the tumors affecting this brain area or their treatment, leads to acquired hypothalamic syndrome in approximately 80% of individuals, depending in large part on the tumor characteristics (1, 2).”
From Approach to the patient with acquired hypothalamic syndrome (2026) · cites this paper as [2]
Other highly morbid symptoms of hypothalamic syndrome include abnormal sleep and circadian function, temperature dysregulation, and behavior changes.
“Clinicians caring for such patients have also appreciated the presence of other highly morbid symptoms, including abnormal sleep and circadian function, temperature dysregulation, and behavior changes (2).”
From Approach to the patient with acquired hypothalamic syndrome (2026) · cites this paper as [2]
Craniopharyngioma is the prototypic suprasellar tumor leading to acquired hypothalamic syndrome.
“As previously summarized, craniopharyngioma is the prototypic suprasellar tumor leading to acquired hypothalamic syndrome, but other tumors (eg, low-grade gliomas, germinomas) also confer increased risk.”
From Approach to the patient with acquired hypothalamic syndrome (2026) · cites this paper as [2]
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Approach to the patient with acquired hypothalamic syndrome
Grishman, E. K., & McCormack, S. E. (2026) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/clinem/dgag197
- Authors
- Müller, H. L., Tauber, M., Lawson, E. A., Özyurt, J., Bison, B., Martinez-Barbera, J.-P., Puget, S., Merchant, T. E., & van Santen, H. M.
- Affiliations
- Centre National de la Recherche Scientifique (FR)
- Harvard University (US)
- Carl von Ossietzky Universität Oldenburg (DE)
- University Hospital Augsburg (DE)
- St. Jude Children's Research Hospital (US)
- ORCID
- Venue
- Nature Reviews Disease Primers
- Published
- 2022-04-21
- Volume
- 8
- Issue
- 1
- Pages
- 24-24
- ISSN
- 2056-676X (ISSN-L)
- OpenAlex
- W4224262686
- Cited by
- 144
- References
- 15
- Citations by year
- 2026: 27 · 2025: 48 · 2024: 31 · 2023: 24 · 2022: 13 · 2021: 1
- FWCI
- 4.12
- Type
- journal article
- Language
- en
- Open access
- green
- dspace.library.uu.nl
- Licence
- Locations
- Nature Reviews Disease Primers (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion)
- Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
- UCL Discovery (University College London) (submittedVersion)
- OPUS (Augsburg University) (submittedVersion)
- Funders
- Cancer Research UK (27727)
- CHILDREN with CANCER UK (15/190)
- Brain Tumour Charity (GN-000382)
- Medical Research Council (MR/M000125/1)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Life Sciences › Neuroscience › Endocrine and Autonomic Systems › Regulation of Appetite and Obesity
- Health Sciences › Medicine › Reproductive Medicine › Hypothalamic control of reproductive hormones
- Keywords
- hypothalamic syndrome, hypothalamic injury, morbid obesity, craniopharyngioma, germ cell tumors, Langerhans cell histiocytosis, Prader-Willi syndrome, septo-optic dysplasia, endocrine disorders, memory impairment, obesity treatment, pathophysiology, diagnosis, prevention
- MeSH terms
- Craniopharyngioma, Endocrine System Diseases, Humans, Hypothalamus, Pituitary Neoplasms, Prader-Willi Syndrome
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