Diagnostic criteria for the hypothalamic syndrome in childhood
Open accessAbstract
Objective: Hypothalamic syndrome (HS) in childhood is a rare condition. Its epidemiology is not well known because incidence and prevalence are related to very rare underlying diseases. In addition, different criteria for the syndrome are used across studies. Recognizing the HS may be difficult, due to its rareness and variety of symptoms. Having diagnostic criteria for signs and symptoms of hypothalamic dysfunction may aid in early recognition and diagnosis, in the reporting and understanding of its etiology, in predicting its course and its management. We aimed to define diagnostic criteria for hypothalamic dysfunction and a score for the presence of HS in childhood. Methods: Diagnostic criteria for hypothalamic dysfunction were developed and subdivided into hyperphagia, hypophagia, body mass index, behavioral problems, sleep disorders, temperature regulation disorders, pituitary dysfunction, radiological hypothalamic assessment, and presence/suspicion of a hypothalamic genetic syndrome. Subsequently, the scoring system was tested in a retrospective cohort of 120 patients at risk for hypothalamic dysfunction. Results: A score for presence of HS was developed. Using this new hypothalamic score, in total 52.5% were scored as having HS. Of these patients, 76.7% were diagnosed with pituitary dysfunction, 32.5% with hyperphagia, 40% with sleep disorders, and 14.2% with temperature dysregulation. For several criteria, clinical data was missing in more than 50% of cases. Conclusions: The here proposed diagnostic criteria for hypothalamic dysfunction and score for presence of HS may be used for care purposes and to aid in early recognition. Also it will be useful for research or registration purposes.
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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 [1]
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 [1]
The diagnostic criteria include assessment of background risk from radiologic assessment of the hypothalamus and/or risk for a genetic disorder associated with hypothalamic syndrome, and then assessment of multiple clinical domains: status of pituitary hormone function, including AVP-D with or without thirst; appetite and weight trajectory; sleep and circadian function; temperature regulation; and
“These criteria include assessment of background risk from radiologic assessment of the hypothalamus and/or risk for a genetic disorder associated with hypothalamic syndrome, and then assessment of multiple clinical domains: status of pituitary hormone function, including AVP-D with or without thirst; appetite and weight trajectory; sleep and circadian function; temperature regulation; and behavior”
From Approach to the patient with acquired hypothalamic syndrome (2026) · cites this paper as [1]
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 [1]
Formal diagnostic criteria, including common symptoms and a scoring system, were proposed to encompass both acquired and congenital forms of hypothalamic syndrome.
“Formal diagnostic criteria, including the common symptoms and a scoring system (1), were proposed to encompass both acquired and congenital forms of hypothalamic syndrome.”
From Approach to the patient with acquired hypothalamic syndrome (2026) · cites this paper as [1]
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van Roessel, I. M. A. A., Hulsmann, S. C., Schouten-van Meeteren, A. Y. N., Hoving, E. W., Janssens, G. O., Raphael, M. F., Zijtregtop-Blom, E. A. M., de Vos-Kerkhof, E., Bakker, B., Tissing, W. J. E., et al. (2025) · eClinicalMedicine · doi:10.1016/j.eclinm.2025.103313 · Cited by 10 · see citing works
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
- van Santen, H. M., van Schaik, J., van Roessel, I. M. A. A., Beckhaus, J., Boekhoff, S., & Müller, H. L.
- Affiliations
- Department of Pediatric Endocrinology, Wilhelmina Children's Hospital, University Medical Center , Utrecht 3584 EA , The Netherlands
- Carl von Ossietzky Universität Oldenburg (DE)
- Department of Pediatrics and Pediatric Hematology/Oncology, University Children's Hospital, Klinikum Oldenburg AöR, Carl von Ossietzky University , Oldenburg 26133 , Germany
- ORCID
- Venue
- European Journal of Endocrinology
- Publisher
- Oxford University Press (OUP)
- Published
- 2023-02-03
- Volume
- 188
- Issue
- 2
- Pages
- 214-225
- ISSN
- 0804-4643 (ISSN-L)
- 1479-683X
- OpenAlex
- W4319067224
- Cited by
- 67
- References
- 5
- Citations by year
- 2026: 21 · 2025: 25 · 2024: 15 · 2023: 6
- FWCI
- 13.78
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- European Journal of Endocrinology (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Health Sciences › Medicine › Reproductive Medicine › Hypothalamic control of reproductive hormones
- Life Sciences › Neuroscience › Endocrine and Autonomic Systems › Regulation of Appetite and Obesity
- Keywords
- hypothalamic syndrome, childhood, diagnostic criteria, scoring system, hypothalamic dysfunction, hyperphagia, hypophagia, body mass index, sleep disorders, pituitary dysfunction, behavioral problems
- MeSH terms
- Humans, Hyperphagia, Hypothalamic Diseases, Hypothalamus, Retrospective Studies, Syndrome
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