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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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Males with prolactin-secreting tumors rarely have galactorrhea and usually present with a larger tumor and complaints such as headache, impotence, and visual abnormalities In normal persons, the serum prolactin rises markedly in response to the administration of chlorpromazine or thyrotropin-releasing hormone (TRH); patients with a prolactin-secreting tumor fail to show such a response With large tumors that compress normal pituitary tissue, thyroid and adrenal function will also be impaired It should be noted that large, nonfunctioning pituitary adenomas also cause modest hyperprolactinemia by distorting the pituitary stalk and reducing dopamine delivery to prolactin-producing cells Acromegaly This disorder consists of acral growth and prognathism in combination with visceromegaly, headache, and several endocrine disorders (hypermetabolism, diabetes mellitus) The highly characteristic facial and bodily appearance, well known to all physicians, is due to an overproduction of growth hormone (GH) after puberty; prior to puberty, an oversecretion of GH leads to gigantism In a small number of acromegalic patients, there is an excess secretion of both GH and prolactin, derived apparently from two distinct populations of tumor cells The diagnosis of this disorder, which is often long delayed, is made on the basis of the characteristic clinical changes, the nding of elevated serum GH values (010 ng/mL), and the failure of the serum GH concentration to rise in response to the administration of glucose or TRH The new growth hormone receptor antagonist pegvisomant has been introduced to reduce many of the manifestations of acromegaly (see the editorial by Ho) Cushing Disease Described in 1932 by Cushing, this condition is only about one-fourth as frequent as acromegaly A distinction is made between Cushing disease and Cushing syndrome, as indicated in Chap 27 The former term is reserved for cases that are caused by the excessive secretion of pituitary ACTH, which in turn causes adrenal hyperplasia; the usual basis is a pituitary adenoma Cushing syndrome refers to the effects of cortisol excess from any one of several sources excessive administration of steroids (the most common cause), adenoma of the adrenal cortex, ACTH-producing bronchial carcinoma, and very rarely other carcinomas that produce ACTH The clinical effects are the same in all of these disorders and include truncal obesity, hypertension, proximal muscle weakness, amenorrhea, hirsutism, abdominal striae, glycosuria, osteoporosis, and in some cases a characteristic mental disorder (page 978) Although Cushing originally referred to the disease as pituitary basophilism and attributed it to a basophil adenoma, the pathologic change may consist only of hyperplasia of basophilic cells or of a nonbasophilic microadenoma Seldom is the sella turcica enlarged: visual symptoms or signs due to involvement of the optic chiasm or nerves and extension to the cavernous sinus are therefore rare The diagnosis of Cushing disease is made by demonstrating increased concentration of plasma and urinary cortisol; these levels are not suppressed by the administration of relatively small doses of dexamethasone (05 mg four times daily), but they are suppressed by high doses (8 mg daily) A low level of ACTH and a high level of cortisol in the blood, increased free cortisol in the urine, and nonsuppression of adrenal function after administration of high doses of dexamethasone are evidence of an adrenal source of the Cushing syndrome usually a tumor and less often a micronodular hyperplasia of the adrenal gland Diagnosis of Pituitary Adenoma This is virtually certain when a chiasmal syndrome is combined with an endocrine syndrome of either hypopituitary or hyperpituitary type Laboratory data that are
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con rmatory of an endocrine disorder, as described above, and sometimes a ballooned sella turcica in plain lms of the skull are occasionally found Patients who are suspected of harboring a pituitary adenoma but in whom the plain lms are normal should be examined by MRI This procedure will visualize pituitary adenomas as small as 3 mm in diameter and show the relationship of the tumor to the optic chiasm This also provides the means of following the tumor s response to therapy (Fig 31-18) It should be kept in mind that pituitary tissue normally enhances on CT and MRI, and small tumors are therefore revealed as nonenhancing nodules Tumors other than pituitary adenomas may sometimes expand the sella Enlargement may be due to an intrasellar craniopharyngioma, carotid aneurysm, or cyst of the pituitary gland Intrasellar epithelium-lined cysts are rare lesions They originate from the apical extremity of Rathke s pouch, which may persist as a cleft between the anterior and posterior lobes of the hypophysis Rarer still are intrasellar cysts that have no epithelial lining and contain thick, dark brown uid, the product of intermittent hemorrhages Both types of intrasellar cyst may compress the pituitary gland and mimic the endocrine-suppressive effects of pituitary adenomas Neoplasms originating in the nasopharynx or sinuses may invade the sella and pituitary gland, and sarcoid lesions at the base of the brain may do the same Also, the pituitary gland (and the chiasm) may be the site of metastases, most of them from the lung and breast (Morita et al); they give rise to diabetes insipidus, pituitary
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Figure 31-18 Pituitary tumors Upper gure: T1-weighted, enhanced coronal MRI A microadenoma is seen as a nonenhancing nodule (arrow) within the normally enhancing pituitary gland The optic chiasm is seen just above the gland Lower gure: Nonenhanced MRI Macroadenoma (arrow) compressing the optic chiasm
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