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Hypothalamic disorder

MedGen UID:: 6988
•Concept ID:: C0020655
•: Disease or Syndrome

Synonym:	Hypothalamic disease
SNOMED CT:	Disorder of hypothalamus (399100005); Hypothalamus disorder (399100005); Hypothalamic disease (399100005)

Monarch Initiative:	MONDO:0002150

Definition

Neoplastic, inflammatory, infectious, and other diseases of the hypothalamus. Clinical manifestations include appetite disorders; autonomic nervous system diseases; sleep disorders; behavioral symptoms related to dysfunction of the limbic system; and neuroendocrine disorders. [from MONDO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVHypothalamic disorder

Pathological process
- Disease
  - Disorder by Site
    - Disorder of nervous system
      - Disorder of the central nervous system
        Brain disorder
        Hypothalamic disorder
        Bardet-Biedl syndrome
        Bardet-Biedl syndrome 1
        Bardet-Biedl syndrome 2
        Bardet-Biedl syndrome 3
        Bardet-Biedl syndrome 4
        Bardet-Biedl syndrome 5
        Bardet-Biedl syndrome 6
        Bardet-Biedl syndrome 7
        Bardet-Biedl syndrome 8
        Bardet-Biedl syndrome 9
        Bardet-Biedl syndrome 10
        Bardet-Biedl syndrome 11
        Bardet-Biedl syndrome 12
        Bardet-Biedl syndrome 13
        Bardet-Biedl syndrome 14
        Bardet-Biedl syndrome 15
        Bardet-Biedl syndrome 16
        Hypothalamic neoplasm
        Hypothalamic Gangliocytoma
        Malignant Hypothalamic Neoplasm
        Neoplasm of the pituitary gland
        Anterior Pituitary Gland Neoplasm
        Benign neoplasm of pituitary gland
        Neoplasm of the anterior pituitary
        Neoplasm of the posterior pituitary
        Pituitary cancer
        Pituitary carcinoma
        Pituitary corticotropic cell adenoma
        Pituitary prolactin cell adenoma
        Sellar Paraganglioma
        Somatotroph Pituitary Neuroendocrine Tumor
        Pallister-Hall syndrome
        Laurence-Moon syndrome
        Pituitary gland disorder
        Diabetes insipidus
        Adipsic Diabetes Insipidus
        Central diabetes insipidus
        Dipsogenic diabetes insipidus
        Nephrogenic diabetes insipidus
        Wolfram syndrome
        Empty sella syndrome
        Hyperpituitarism
        Acromegaly
        Adrenocorticotropic hormone excess
        Gigantism
        Hyperprolactinemia
        Increased circulating prolactin concentration
        Pituitary dependent hypercortisolism
        Hypophysitis
        Primary hypophysitis
        Hypopituitarism
        Anterior hypopituitarism
        Panhypopituitarism
        Pituitary dwarfism
        Inappropriate antidiuretic hormone secretion
        Non-Neoplastic Pituitary Gland Disorder
        Ectopic posterior pituitary
        Lymphocytic Neurohypophysitis
        Necrosis of pituitary
        Pituitary apoplexy
        Pituitary gland hypoplasia
        Pituitary gland infarction

Professional guidelines

PubMed

European guideline and expert statements on the management of narcolepsy in adults and children.

Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
Eur J Neurol 2021 Sep;28(9):2815-2830. Epub 2021 Jun 25 doi: 10.1111/ene.14888. PMID: 34173695

European guideline and expert statements on the management of narcolepsy in adults and children.

Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288

Narcolepsy - clinical spectrum, aetiopathophysiology, diagnosis and treatment.

Bassetti CLA, Adamantidis A, Burdakov D, Han F, Gay S, Kallweit U, Khatami R, Koning F, Kornum BR, Lammers GJ, Liblau RS, Luppi PH, Mayer G, Pollmächer T, Sakurai T, Sallusto F, Scammell TE, Tafti M, Dauvilliers Y
Nat Rev Neurol 2019 Sep;15(9):519-539. Epub 2019 Jul 19 doi: 10.1038/s41582-019-0226-9. PMID: 31324898

See all (6)

Recent clinical studies

Etiology

Clinical characteristics of patients with unexplainable hypothalamic disorder diagnosed by the corticotropin-releasing hormone challenge test: a retrospective study.

Hataya Y, Okubo M, Hakata T, Fujimoto K, Iwakura T, Matsuoka N
BMC Endocr Disord 2022 Dec 9;22(1):312. doi: 10.1186/s12902-022-01237-7. PMID: 36494805 Free PMC Article

Bexarotene-induced central hypothyroidism assessed by TRH stimulation test in cutaneous T-cell lymphoma patients.

Toi N, Kurajoh M, Miyaoka D, Nagata Y, Yamada S, Imanishi Y, Hayashi D, Tateishi C, Inaba M, Tsuruta D, Morita A, Emoto M
Endocr J 2022 Jan 28;69(1):101-105. Epub 2021 Aug 25 doi: 10.1507/endocrj.EJ21-0313. PMID: 34433736

European guideline and expert statements on the management of narcolepsy in adults and children.

Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288

Clinical perspectives on the genetics of schizophrenia: a bottom-up orientation.

Verhoeven WM, Tuinier S
Neurotox Res 2008 Oct;14(2-3):141-50. doi: 10.1007/BF03033806. PMID: 19073422

See all (21)

Diagnosis

Nakamura-Utsunomiya A
Int J Mol Sci 2022 Jun 21;23(13) doi: 10.3390/ijms23136899. PMID: 35805903 Free PMC Article

European guideline and expert statements on the management of narcolepsy in adults and children.

Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288

Narcolepsy - clinical spectrum, aetiopathophysiology, diagnosis and treatment.

Brain tumors and anorexia nervosa syndrome.

Chipkevitch E
Brain Dev 1994 May-Jun;16(3):175-9, discussion 180-2. doi: 10.1016/0387-7604(94)90064-7. PMID: 7943600

See all (23)

Therapy

European guideline and expert statements on the management of narcolepsy in adults and children.

Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288

Use of somatostatin analogues in obesity.

Tzotzas T, Papazisis K, Perros P, Krassas GE
Drugs 2008;68(14):1963-73. doi: 10.2165/00003495-200868140-00003. PMID: 18778119

Neurologic manifestations of hypothalamic disease.

Martin JB, Riskind PN
Prog Brain Res 1992;93:31-40; discussion 40-2. doi: 10.1016/s0079-6123(08)64561-6. PMID: 1480755

In vitro testing of thyroid function: a review.

Pain RW
Pathology 1975 Jan;7(1):1-12. doi: 10.3109/00313027509073765. PMID: 806877

See all (19)

Prognosis

Variable hormonogenesis in Cushing's syndrome.

Shapiro MS, Shenkman L
Q J Med 1991 Apr;79(288):351-63. PMID: 1649480

Late recurrence of surgically removed prolactinomas.

Parl FF, Cruz VE, Cobb CA, Bradley CA, Aleshire SL
Cancer 1986 Jun 15;57(12):2422-6. doi: 10.1002/1097-0142(19860615)57:12<2422::aid-cncr2820571229>3.0.co;2-b. PMID: 3697940

The effects of clomiphene citrate on the hypothalamic-pituitary-gonadal axis in anorexia nervosa.

Wakeling A, Marshall JC, Beardwood CJ, Souza VF, Russell GF
Psychol Med 1976 Aug;6(3):371-80. doi: 10.1017/s0033291700015804. PMID: 996198

See all (3)

Clinical prediction guides

Clinical characteristics of patients with unexplainable hypothalamic disorder diagnosed by the corticotropin-releasing hormone challenge test: a retrospective study.

Hataya Y, Okubo M, Hakata T, Fujimoto K, Iwakura T, Matsuoka N
BMC Endocr Disord 2022 Dec 9;22(1):312. doi: 10.1186/s12902-022-01237-7. PMID: 36494805 Free PMC Article

Bexarotene-induced central hypothyroidism assessed by TRH stimulation test in cutaneous T-cell lymphoma patients.

Development and validation of a new questionnaire assessing quality of life in adults with hypopituitarism: Adult Hypopituitarism Questionnaire (AHQ).

Ishii H, Shimatsu A, Okimura Y, Tanaka T, Hizuka N, Kaji H, Hanew K, Oki Y, Yamashiro S, Takano K, Chihara K
PLoS One 2012;7(9):e44304. Epub 2012 Sep 11 doi: 10.1371/journal.pone.0044304. PMID: 22984490 Free PMC Article

Hypercortisolism in patients with functional hypothalamic-amenorrhea.

Suh BY, Liu JH, Berga SL, Quigley ME, Laughlin GA, Yen SS
J Clin Endocrinol Metab 1988 Apr;66(4):733-9. doi: 10.1210/jcem-66-4-733. PMID: 3346352

Sexual and hypothalamic dysfunction in the postconcussional syndrome.

Kosteljanetz M, Jensen TS, Nørgård B, Lunde I, Jensen PB, Johnsen SG
Acta Neurol Scand 1981 Mar;63(3):169-80. doi: 10.1111/j.1600-0404.1981.tb00769.x. PMID: 6782821

See all (14)

Recent systematic reviews

European guideline and expert statements on the management of narcolepsy in adults and children.

Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288

See all (2)

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Hypothalamic disorder

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

Related information

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