Hypothalamic hamartoma secreting corticotropin-releasing hormone

Voyadzis, Jean Marc; Guttman-Bauman, Ines; Santi, Mariarita; Cogen, Philip H.

doi:10.3171/ped.2004.100.2.0212

Cited by 8 publications

(9 citation statements)

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“…This effect could be emulated by the mathematical model in Table 1 as a consequence of an increased CRH output caused by LHK (Fig 3B). This novel finding strengthens the notion of an important role of CRH in the pathophysiology of mood disorders [92], and is in agreement with clinical data on hypothalamic hamartoma–patients with this non-neoplastic tumor present with mood symptoms [93] and also elevation in CRH concentration [94]. However, it might seem contradictory to other data of emerging manic symptoms in patients receiving corticosteroid treatment for medical conditions (reviewed in [95]).…”

Section: Discussionsupporting

confidence: 87%

Corticosterone oscillations during mania induction in the lateral hypothalamic kindled rat—Experimental observations and mathematical modeling

Abulseoud

Choi

et al. 2017

PLoS ONE

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Changes in the hypothalamic-pituitary-adrenal (HPA) axis activity constitute a key component of bipolar mania, but the extent and nature of these alterations are not fully understood. We use here the lateral hypothalamic-kindled (LHK) rat model to deliberately induce an acute manic-like episode and measure serum corticosterone concentrations to assess changes in HPA axis activity. A mathematical model is developed to succinctly describe the entwined biochemical transformations that underlay the HPA axis and emulate by numerical simulations the considerable increase in serum corticosterone concentration induced by LHK. Synergistic combination of the LHK rat model and dynamical systems theory allows us to quantitatively characterize changes in HPA axis activity under controlled induction of acute manic-like states and provides a framework to study in silico how the dynamic integration of neurochemical transformations underlying the HPA axis is disrupted in these states.

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Section: Discussionsupporting

confidence: 87%

Corticosterone oscillations during mania induction in the lateral hypothalamic kindled rat—Experimental observations and mathematical modeling

Abulseoud

Choi

et al. 2017

PLoS ONE

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“…Voyadzis et al . report a single case with excessive corticotropin‐releasing hormone (CRH) secretion in a 2‐year‐old boy with HH characterized clinically by excessive behavioral lability and paroxysms of rage.…”

Section: Other Endocrine Manifestations Of Hhmentioning

confidence: 88%

“…Voyadzis et al 80 report a single case with excessive corticotropin-releasing hormone (CRH) secretion in a 2-yearold boy with HH characterized clinically by excessive behavioral lability and paroxysms of rage. There were no physical signs of endocrinopathy, but CRH and adrenocorticotropic hormone (ACTH) levels were elevated and did not suppress with dexamethasone administration.…”

Section: Other Endocrine Manifestations Of Hhmentioning

confidence: 95%

Hypothalamic hamartoma with epilepsy: Review of endocrine comorbidity

2017

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Summary The most common, and usually the only, endocrine disturbance in patients with hypothalamic hamartoma (HH) and epilepsy is central precocious puberty (CPP). The mechanism for CPP associated with HH may relate to ectopic generation and pulsatile release of gonadotropin-releasing hormone (GnRH) from the HH, but this remains an unproven hypothesis. Possible regulators of GnRH release that are intrinsic to HH tissue include the following: (1) glial factors (such as transforming growth factor α[TGFα) and (2) γ-aminobutyric acid (GABA)–mediated excitation. Both are known to be present in surgically-resected HH tissue, but are present in patients with and without a history of CPP, suggesting the possibility that symptoms related to HH are directly associated with the region of anatomic attachment of the HH to the hypothalamus, which determines functional network connections, rather than to differences in HH tissue expression or pathophysiology. CPP associated with HH presents with isosexual development prior to the age of 8 years in girls and 9 years in boys. It is not uncommon for CPP with HH to present in children at an earlier age in comparison to other causes of CPP, including in infancy. Surgical resection of the HH can be effective for treating CPP, but is reserved for patients with intractable epilepsy, since GnRH agonists are widely available and effective treatment. Other endocrine disturbances with HH are rare, but can include growth hormone deficiency, hypothyroidism, and adrenal insufficiency. Diabetes insipidus is commonly encountered postoperatively, but is not observed with HH prior to surgical intervention.

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“…To date, only Voyadzis Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection et al have demonstrated corticotropin-releasing hormone (CRH) immunostaining in an HH. 56 We report the first case of a symptomatic HH with a solid CRH-positive component and associated tumor cyst in an 18 month-old girl presenting with central precocious puberty. We discuss the pathological features of this hamartoma along with approaches to its resection and management.…”

confidence: 99%

“…Our case has shown CRH immunoreactivity in the solid part of the lesion; however, there are conflicting opinions on the role of CRH in the causation of precocious puberty in these patients. 16,22,56 …”

Section: Treatment Of Hhmentioning

confidence: 99%

Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection

Manjila

Vogel

Chen

et al. 2014

PED

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Hypothalamic hamartomas (HHs) are rare developmental lesions arising from the inferior hypothalamus that may cause gelastic seizures and central precocious puberty. Cystic changes in HHs are rare, usually occurring in giant lesions. The authors describe an unusual case of cystic HH masquerading as a suprasellar arachnoid cyst in an 18-month-old girl presenting with precocious puberty. Microsurgical removal of the lesion led to complete resolution of the precocious puberty on long-term follow-up. This case is the first reported HH with pathological demonstration of corticotropin-releasing hormone immunostaining in the solid tumor and glial cells in the cyst wall of the lesion. The clinical and radiological characteristics of HHs are reviewed, along with the unique surgical strategies used to manage cystic lesions in the suprasellar region.

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Hypothalamic hamartoma secreting corticotropin-releasing hormone

Cited by 8 publications

References 0 publications

Corticosterone oscillations during mania induction in the lateral hypothalamic kindled rat—Experimental observations and mathematical modeling

Corticosterone oscillations during mania induction in the lateral hypothalamic kindled rat—Experimental observations and mathematical modeling

Hypothalamic hamartoma with epilepsy: Review of endocrine comorbidity

Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection

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