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CARDINAL MANIFESTATIONS OF NEUROLOGIC DISEASE
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disorders, should the patient be hyponatremic, it is desirable to determine the intravascular volume and the urine sodium output before instituting treatment Other Disturbances of ADH and Thirst Conditions have been described in which the osmoreceptor control of ADH and of thirst appear to be dissociated As reported by Hayes and coworkers, one of our colleagues patients repeatedly developed severe hypernatremia (levels as high as 180 to 190 meq/L), at which time he became confused and stuporous Although the patient was able to initiate a release of ADH, his thirst mechanism was nonfunctional Only when the patient was compelled to drink water at regular intervals did his serum sodium fall Robertson and others have described similar cases with abnormalities of thirst These have been reported under the title of central or essential hypernatremia
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A hamartoma of the hypothalamus (part of von Recklinghausen disease or of polyostotic brous dysplasia) is a leading cause of precocious puberty in both boys and girls; in a number of such cases, so-called gelastic seizures have been conjoined (Breningstall, see page 278) Neurologic study entails computed tomography (CT) and MRI imaging of the hypothalamus, ovaries, and adrenals Adiposogenital Dystrophy (Froehlich Syndrome) Under this title, in 1901, Froehlich rst described the association of obesity and gonadal underdevelopment He related the disorder to a pituitary tumor But a few years later, Erdheim recognized that the same syndrome could be a manifestation of a lesion (a suprasellar cyst in his case) involving or restricted to the hypothalamus Later it was determined that obesity and hypogonadism could occur together or separately and were often combined with a loss of vision and unprovoked rage, aggression, or antisocial behavior Diabetes insipidus may be added In some patients, the clinical state is characterized by abulia, apathy, and reduced verbal output The usual causes of the Froehlich syndrome are craniopharyngioma, adamantinoma, and glioma, but many other tumors have been reported (pituitary adenoma, cholesteatoma, lipoma, meningioma, glioma, angiosarcoma, and chordoma) The condition bears clinical similarities to the Prader-Willi syndrome, in which hypothalamic abnormalities are not found (page 864) Hypothalamic Disorders Associated with Alterations in Weight Precise neuroanatomic studies have localized a satiety center in the ventromedial nucleus of the hypothalamus and an appetite center in the ventrolateral nucleus Lesions in the lateral hypothalamus may result in a failure to eat and, in the neonate, failure to thrive; lesions in the medial hypothalamus, in overeating and obesity Bray and Gallagher, who analyzed eight cases of the latter type, concluded that the critical lesion was a bilateral destruction of the ventromedial regions of the hypothalamus Most of the reported cases of this type have been due to tumors, particularly craniopharyngioma, and some to trauma, in ammatory disease, and hydrocephalus (Suzuki et al) In a case that was subject to clinicopathologic correlation, Reeves and Plum found that a hamartoma had destroyed the medial eminence and the ventromedial nuclei bilaterally but spared the lateral hypothalamus Hyperphagia and rage reactions were the main clinical features; the associated polydipsia and polyuria were due to extension of the tumor to the anterior hypothalamus It is evident that in only a tiny fraction of persons can obesity be traced to a hypothalamic lesion Of overriding importance are genetic factors, such as the number of lipocytes that one inherits and their ability to store fat A rare disorder of infants has been described under the title of diencephalic syndrome Progressive and ultimately fatal emaciation (failure to thrive), despite normal or near-normal food intake, in an otherwise alert and cheerful infant is the main clinical feature The lesion has usually proved to be a low-grade astrocytoma of the anterior hypothalamus or optic nerve (Burr et al) Extrahypothalamic parts of the brain, if diseased, may also be associated with increased food-seeking behavior, food ingestion, and weight gain Examples are involvement of limbic structures, as in the Kluver-Bucy syndrome (page 448) and basal frontal lobe lesions leading to gluttony Anorexia Nervosa and Bulimia The special syndromes called anorexia nervosa and bulimia have been dif cult to classify and are mentioned in this chapter only because they are associated with
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