Hyperphagia, Rage, and Dementia Accompanying a Ventromedial Hypothalamic Neoplasm

Reeves, Alexander G.; Plum, Fred

doi:10.1001/archneur.1969.00480120062005

Cited by 234 publications

(45 citation statements)

References 31 publications

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“…In vivo recordings in freely moving male mice reveal an increase in VMHvl activity during male-typical aggression, and optogenetic stimulation of VMHvl neurons elicits indiscriminate aggressive attacks towards both males and females. The VMH is molecularly heterogeneous and regulates both female sexual receptivity and male-typical aggression behaviours as well as defensive reactions to predators and energy balance in diverse animals, including humans [89,[93][94][95][96][97]130,[132][133][134][135][136][137][138][139][140][141][142][143][144][145][146]. Lesions and other manipulations that do not target molecularly defined neurons within the VMH can yield conflicting behavioural phenotypes [96][97]135,137], perhaps owing to non-targeted manipulation of these heterogeneous neurons or destruction of fibres of passage within this region.…”

Section: (B) Sex Differences In Gene Expressionmentioning

confidence: 99%

Genetic dissection of neural circuits underlying sexually dimorphic social behaviours

Bayless

Shah²

2016

Phil. Trans. R. Soc. B

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The unique hormonal, genetic and epigenetic environments of males and females during development and adulthood shape the neural circuitry of the brain. These differences in neural circuitry result in sex-typical displays of social behaviours such as mating and aggression. Like other neural circuits, those underlying sex-typical social behaviours weave through complex brain regions that control a variety of diverse behaviours. For this reason, the functional dissection of neural circuits underlying sex-typical social behaviours has proved to be difficult. However, molecularly discrete neuronal subpopulations can be identified in the heterogeneous brain regions that control sex-typical social behaviours. In addition, the actions of oestrogens and androgens produce sex differences in gene expression within these brain regions, thereby highlighting the neuronal subpopulations most likely to control sexually dimorphic social behaviours. These conditions permit the implementation of innovative genetic approaches that, in mammals, are most highly advanced in the laboratory mouse. Such approaches have greatly advanced our understanding of the functional significance of sexually dimorphic neural circuits in the brain. In this review, we discuss the neural circuitry of sex-typical social behaviours in mice while highlighting the genetic technical innovations that have advanced the field.

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Section: (B) Sex Differences In Gene Expressionmentioning

confidence: 99%

Genetic dissection of neural circuits underlying sexually dimorphic social behaviours

Bayless

Shah²

2016

Phil. Trans. R. Soc. B

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“…A rare case of HH was associated with hypogonadotropic hypogonadism and growth hormone deficiency. 45) HH manifesting as acromegaly due to excessive growth hormone-releasing hormone secretion, 65) diencephalic syndrome, 61) or progressive visual impairment is exceptional. 72) There are very rare coexisting intracranial abnormalities including supra-or infratentorial arachnoid cysts, callosal agenesis, hemispheric dysgenesis, and cerebellar hypoplasia.…”

Section: Cppmentioning

confidence: 99%

Hypothalamic Hamartoma

Arita

Kurisu

Kiura

et al. 2005

Neurol. Med. Chir.(Tokyo)

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The incidence of hypothalamic hamartomas (HHs) has increased since the introduction of magnetic resonance (MR) imaging. The etiology of this anomaly and the pathogenesis of its peculiar symptoms remain unclear, but recent electrophysiological, neuroimaging, and clinical studies have yielded important data. Categorizing HHs by the degree of hypothalamic involvement has contributed to the accurate prediction of their prognosis and to improved treatment strategies. Rather than undergoing corticectomy, HH patients with medically intractable seizures are now treated with surgery that targets the HH per se, e.g. HH removal, disconnection from the hypothalamus, stereotactic irradiation, and radiofrequency lesioning. Although surgical intervention carries risks, total eradication or disconnection of the lesion leads to cessation or reduction of seizures and improves the cognitive and behavioral status of these patients. Precocious puberty in HH patients is safely controlled by long-acting gonadotropin-releasing hormone agonists. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities may lead to earlier intervention in young HH patients and prevent the decline in their cognitive abilities and quality of life. This review of hypothalamic hamartomas presents current classifications, pathophysiologies, and treatment modalities.

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“…A case of hyperphagia, rage, and dementia in association with HH has been previously described in the pre-CT era: An exploratory craniotomy was performed and an unresectable tumor at the base of the ventricle was biopsied and found to be an HH. 24 The report does not mention if the patient's hyperphagia was alleviated by the biopsy. In the case of our patient, resection was considered to control hyperphagia as a last-ditch effort after medical therapy and counseling failed.…”

mentioning

confidence: 98%

Successful treatment of hyperphagia by resection of a hypothalamic hamartoma

Esquenazi

Sandberg

Rekate³

2013

PED

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Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a 7-year-old girl who was diagnosed with an HH due to precocious puberty and was treated medically with a gonadotropin-releasing hormone analog for 3 years. Despite normalization of her plasma levels of luteinizing hormone, follicle-stimulating hormone, and estradiol and arrest of her precocious puberty, the patient developed progressive weight gain associated with extreme hyperphagia and morbid obesity by the age of 10 years. Her compulsive eating patterns were refractory to counseling and other interventions attempted by her parents and physicians. After resection of the HH, her hyperphagia resolved and her weight stabilized. To the authors' knowledge, this is the first report describing resection of an HH for the purpose of treating hyperphagia and obesity.

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Hyperphagia, Rage, and Dementia Accompanying a Ventromedial Hypothalamic Neoplasm

Cited by 234 publications

References 31 publications

Genetic dissection of neural circuits underlying sexually dimorphic social behaviours

Genetic dissection of neural circuits underlying sexually dimorphic social behaviours

Hypothalamic Hamartoma

Successful treatment of hyperphagia by resection of a hypothalamic hamartoma

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