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While surgery remains the mainstay of therapy, novel medical and radiopharmaceutical approaches are available for patients with progressive and/or unresectable tumors.Septo-optic dysplasia (SOD) or de Morsier's syndrome is a rare congenital disorder characterized by a classic triad of (a) optic nerve hypoplasia, (b) agenesis of septum pellucidum and corpus callosum, and (c) hypoplasia of the hypothalamic-pituitary axis. This chapter will outline the key information regarding the etiology and epidemiology of this syndrome with a focus on its comprehensive management. Particular attention will be paid to the diagnostic stage and the most relevant differential diagnosis, before moving to the complexities of its treatment. In fact, although SOD is not curable, many aspects of this syndrome can be improved through a tailored multidisciplinary approach consisting in hormonal replacement, corrective ophthalmological surgery, management of epileptic seizures, and active neuropsychological support.Puberty, which in humans is considered to include both gonadarche and adrenarche, is the period of becoming capable of reproducing sexually and is recognized by maturation of the gonads and development of secondary sex characteristics. Gonadarche referring to growth and maturation of the gonads is fundamental to puberty since it encompasses increased gonadal steroid secretion and initiation of gametogenesis resulting from enhanced pituitary gonadotropin secretion, triggered in turn by robust pulsatile GnRH release from the hypothalamus. This chapter reviews the development of GnRH pulsatility from before birth until the onset of puberty. In humans, GnRH pulse generation is restrained during childhood and juvenile development. This prepubertal hiatus in hypothalamic activity is considered to result from a neurobiological brake imposed upon the GnRH pulse generator resident in the infundibular nucleus. Reactivation of the GnRH pulse generator initiates pubertal development. Current understanding of the genetics and physiology of the brake will be discussed, as will hypotheses proposed to account for timing the resurgence in pulsatile GnRH and initiation of puberty. The chapter ends with a discussion of disorders associated with precocious or delayed puberty with a focus on those with etiologies attributed to aberrant GnRH neuron anatomy or function. A pediatric approach to patients with pubertal disorders is provided and contemporary treatments for both precocious and delayed puberty outlined.Klinefelter syndrome (47,XXY) is a frequent chromosomal disorder among males, often presenting with hypergonadotropic hypogonadism, small firm testicles, metabolic disorders, neurocognitive challenges, and increased height. Neurologic disorders such as epilepsy, seizures, and tremor as well as psychiatric disorders are also seen more frequently. The neurocognitive deficits seen are present in many areas of cognition, typically affecting general cognitive abilities, language, and executive functioning. Also, social dysfunction is frequent. Dyslexia is present in more than half of all males. Brain imaging studies generally show a typical pattern, with many nuclei and brain areas being smaller than among controls. However, it has not been possible to link the brain alterations found in imaging studies with the neurocognitive profile. The genetics underlying the phenotypic traits found among males with Klinefelter syndrome still remains to be elucidated; however, recent studies have described pervasive changes in the methylome and transcriptome and new and interesting candidate genes have been pinpointed, but their involvement in the phenotype of Klinefelter syndrome has not been proven.Gender identity (an individual's perception of being male or female) and sexual orientation (heterosexuality, homosexuality, or bisexuality) are programmed into our brain during early development. During the intrauterine period in the second half of pregnancy, a testosterone surge masculinizes the fetal male brain. If such a testosterone surge does not occur, this will result in a feminine brain. As sexual differentiation of the brain takes place at a much later stage in development than sexual differentiation of the genitals, these two processes can be influenced independently of each other and can result in gender dysphoria. Nature produces a great variability for all aspects of sexual differentiation of the brain. Mechanisms involved in sexual differentiation of the brain include hormones, genetics, epigenetics, endocrine disruptors, immune response, and self-organization. Furthermore, structural and functional differences in the hypothalamus relating to gender dysphoria and sexual orientation are described in this review. All the genetic, postmortem, and in vivo scanning observations support the neurobiological theory about the origin of gender dysphoria, i.e., it is the sizes of brain structures, the neuron numbers, the molecular composition, functions, and connectivity of brain structures that determine our gender identity or sexual orientation. There is no evidence that one's postnatal social environment plays a crucial role in the development of gender identity or sexual orientation.Aneurysmal subarachnoid hemorrhage (aSAH) results from the rupture of an intracranial aneurysm and represents a highly debilitating and devastating disorder. The prevalence rate of neuroendocrine impairment in the acute phase is extremely variable, ranging from 3.8% to 92.3%, depending on the time point considered, the method/test utilized, the clinical severity at admission, and probably also ethnicity. Further studies are needed to clarify such a wide range in neuroendocrine dysfunction in patients with aSAH. The overall neuroendocrine impairment rate in chronic aSAH is in the range 47%-83.3% with specific neuroendocrine impairment varying from 2.5% to 83.3%. The overall pituitary deficiency rate tends to decrease over time after SAH, with recovery of most endocrine and some de novo dysfunctions being reported. [https://www.selleckchem.com/mTOR.html mTOR inhibitor] Only one study has reported an increase of overall endocrine impairment in the chronic follow-up. Neuroendocrine dysfunction seems to have a high prevalence in aSAH patients, even though its exact impact is not precisely known and is based on contrasting findings.