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alterations in several hypothalamic functions, including appetite, temperature control, and menstruation In all likelihood, these alterations are not the result of a primary dysfunction of hypothalamic nuclei but are secondary to the extreme weight loss, which is the primary feature of the disease However, a causal link between these idiopathic diseases and hypothalamic dysfunction has been suggested by the rare patients with anorexia nervosa who were later found to have hypothalamic tumors (Bhanji and Mattingly, Berek et al, and Lewin et al) Anorexia nervosa and bulimia are probably best regarded as disorders of behavior, in this case an obsession with thinness; they are therefore discussed with the psychiatric disorders (see page 1304) But the developmental nature of the disease (arising in early adolescence), its virtual absence in men, and the hypothalamic alterations mentioned above do not allow the dismissal of a primary disorder of the brain s appetite centers Abnormalities of Growth Presumably, in most instances of growth retardation, there is a de ciency of GHRH or of GH per se In the Prader-Willi syndrome (obesity, hypogonadism, hypotonia, mental retardation, and short stature), Bray and Gallagher found the de ciency to be one of GHRH In certain congenital and developmental diseases, the hypothalamus appears to be incapable of releasing GH This appears to be the case, in the de Morsier septooptic defect of the brain (median facial cleft, cavum septum pellucidum, optic defect), Stewart and colleagues found an isolated de ciency of GH In children with idiopathic hypopituitarism in whom stunting of growth is associated with other endocrine abnormalities, the de ciency is probably in the synthesis or release of GHRH In some dwarfs (Laron dwarf, Seckel bird-headed dwarf), there are extremely high levels of circulating GH, suggesting either a defect in the GH molecule or an unresponsiveness of target organs Many patients with the more severe forms of mental retardation are subnormal in height and weight, but the explanation for this has not been ascertained It has not been reducible to changes in the level of GHRH or GH Of course, the vast majority of unusually short children who are otherwise healthy do not have a recognizable defect in GH or GHRH Often their parents are short The therapeutic use of GH in such children is a controversial matter The hormone effects a spurt in growth during the rst year of its administration, but whether it signi cantly in uences growth in the long term is still under investigation There is concern about the risk of transmitting prion or viral diseases through administration of the biologically derived hormone; this problem is obviated if genetically produced hormone is used In gigantism, most of the reported cases have been due to pituitary adenomas that secrete an excess of GH This must occur prior to closure of the epiphyses Hypersecretion of GH after closure of the epiphyses results in acromegaly The notion of a purely hypothalamic form of gigantism or acromegaly (hypothalamic acromegaly) has been af rmed by Asa and associates, who described six patients with hypothalamic gangliocytomas that produced GHRH The possibility of an ectopic source of GH must also be considered The mentally retarded individuals with gigantism described by Sotos and coworkers were found to have no abnormalities of GHRH, GH, or somatomedin Disturbances of Temperature Regulation Bilateral lesions in the anterior parts of the hypothalamus, speci cally of temperaturesensitive neurons in the preoptic area, may result in hyperthermia. open source barcode generator excel TBarCode Office: Barcode Add-In for Microsoft Word/Excel - Tec-It
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