Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary empty sella syndrome (ESS) is an anatomo-radiological picture characterized by the presence of an arachnoid herniation filled with liquor that compresses the pituitary against the sellar wall. ESS occurs particularly in obese, hypertensive, cephalgic women, it is often asymptomatic but it may be associated with ophthalmologic, neurologic and sometime non-characterizing endocrine disorders. We report here 71 cases of primary ESS observed and assessed during the last fourteen years. The following endocrinological diagnostic procedures were carried out: hormonal (RIA) basal profile: FT3, FT4, TSH, PRL, ACTH, FSH, LH, 8.00 a.m. and p.m. cortisolemia, Aldo, PRA, DHEA-S, FTe, E2, P, PTH, CT, and calcemia and phosphoremia; provocative tests: TRH, GnRH, insulin hypoglycemia, etc.; inhibition tests: "overnight" and high dose dexamethasone. Clinical, radiological (skull radiographs, CT and/or MRI) and ophthalmologic (fundus, visual fields) assessment were made. We found principally cephalgia (52/71: 73.2%), hypertension (42/71: 59.1%), obesity (47/71: 66.1%). But we found especially mental disorders (57/71: 80.2%), in our knowledge not previously reported in the literature, as anxiety or dysthymic disorders with behavioural disturbances (chiefly oral compulsion). We found endocrinopathies in 36/71 (50.7%), isolated or coexisting in some patients: hyperPRL (14%), hypopituitarism (10.4%), hypogonadism (7%), diabetes insipidus (2.8%), hyperACTH (1.4%), hypoGH (15.4%), pituitary adenomas (8.4%). Several hypothalamic illness show a clinical picture including mental disorders and obesity. The Authors hypothesize that the ESS may be a "new" hypothalamic syndrome (compression/stretching on hypophysis and/or hypophyseal stalk by arachnoidocele; disorder of some hormones and neurotransmitters as leptin, neuropeptide Y, orexins, POMC-derived peptides, etc).
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PMID:[Primary empty sella syndrome. Observations on 71 cases]. 1020 96

Mild hyperprolactinemia frequently accompanies the hypopituitarism seen in patients with pituitary macroadenomas that do not secrete PRL. Recent data suggested that the hypopituitarism and mild hyperprolactinemia in this setting are largely due to compression of pituitary stalk and portal vessels. Headaches (HAs) are frequently seen in patients with large adenomas and at times in those with microadenomas. Because the walls of the sella turcica are relatively rigid, we postulate that tumor growth within the sella increases intrasellar pressure (ISP), which in turn impairs portal blood flow, resulting in mild hyperprolactinemia and hypopituitarism. We also postulate that increased mean ISP (MISP) contributes to the development of HAs. Normal MISP is not known but is unlikely to exceed normal intracranial pressure of less than 10-15 mm Hg. We determined MISP in 49 patients who had transsphenoidal surgery for pituitary adenomas. MISP was measured using a commonly available intracranial monitoring kit where a fiberoptic transducer was inserted through a 2-mm dural incision at the time of adenomectomy. Patients with deficient FSH, LH, ACTH, or TSH secretion were considered hypopituitary. Data on serum PRL levels were included for analysis only in patients whose adenomas had negative immunostaining for the hormone. MISP measurements ranged from 7-56 mm Hg, with a mean (+/-SD) of 28.8 +/- 13.5 and a median of 26 mm Hg. The pressure measurements were higher in patients with hypopituitarism than in those with normal pituitary function (P = 4.6013 x 10(-6)). Patients presenting with HAs had higher MISP than those who did not (P = 5.44 x 10(-7)), regardless of their pituitary function or tumor sizes. PRL levels correlated positively with MISP values (r = 0.715, P < 0.0001). Tumor size did not correlate with MISP or PRL levels. The findings of increased MISP in hypopituitary patients and the documented correlation with PRL levels, suggest that ISP is a major mechanism involved in the pathogenesis of hypopituitarism and hyperprolactinemia. Similarly, the increased MISP in patients with HAs, irrespective of tumor size or pituitary function, suggest that increased ISP is a major mechanism involved in the pathogenesis of this symptom. The data support the hypothesis that in patients with pituitary adenomas increased ISP is a major mechanism contributing to the development of hyperprolactinemia, hypopituitarism, and HAs. Increased ISP in these patients leads to compression of the portal vessels and the associated interruption of the delivery of hypothalamic hormones to the anterior pituitary. This would explain the reversibility of pituitary function observed in most patients after adenomectomy. However, increased ISP may also lead to decreased blood supply, resulting in ischemic necrosis in some regions of the pituitary. The latter could limit potential recovery of pituitary function after adenomectomy.
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PMID:The dominant role of increased intrasellar pressure in the pathogenesis of hypopituitarism, hyperprolactinemia, and headaches in patients with pituitary adenomas. 1084 53

We report a case of a seventy-year-old woman with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by Rathke's cleft cyst. She experienced nausea, vomiting, diarrhea, and headache and disturbance of consciousness induced by hyponatremia at a serum sodium level of 100 mEq/l. In spite of severe hyponatremia, urinary sodium excretion was not suppressed and serum osmolality (270 mOsm/kg) was lower than urine osmolality (304 mOsm/kg), and arginine vasopressin (AVP) remained within normal range. SIADH was diagnosed because she was free from other diseases known to cause hyponatremia such as dehydration, cardiac dysfunction, liver dysfunction, renal dysfunction, hypothyroidism, and adrenal insufficiency. Cranial computed tomographic (CT) scan and cranial magnetic resonance (MR) imaging showed a cystic lesion of approximately 2 cm in diameter in the pituitary gland. These images suggested that the cystic lesion was a Rathke's cleft cyst, which was the cause of SIADH. Water restriction therapy normalized her serum sodium concentration and improved her symptoms. After one year, she suffered from general fatigue, appetite loss, fever, and body weight loss (5 kg/2 months). She had neither hypotension nor hypoglycemia, but her serum sodium level was low and serum cortisol, ACTH, and urine free cortisol were very low. Therefore, secondary adrenal insufficiency was suspected and diagnosed by stimulation tests. After start of hydrocortisone replacement therapy (10 mg/day), her symptoms disappeared. In conclusion, Rathke's cleft cyst should be kept in mind as a potential cause in a patient with SIADH, hypopituitarism, and/or adrenal insufficiency.
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PMID:Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by rathke's cleft cyst: a case report. 1107 19

We report a patient with primary hypothyroidism associated with an aberrant ACTH response to the LH-RH test. A 40-year-old woman was admitted to our hospital displaying headache, nausea, and numbness on the left side of her face, upper limbs, and tips of her toes. Computed tomography and magnetic resonance imaging revealed a mass-like lesion in the pituitary. A high serum TSH concentration with concomitant low thyroid hormone concentrations resulted in a diagnosis of primary hypothyroidism. To exclude the possibility of a coexisting pituitary tumor including a TSH-secreting tumor, we performed dynamic TSH secretion tests. TRH testing showed an excessive, delayed TSH response, typical of primary hypothyroidism. Serum TSH decreased not only after administration of CRH, octreotide, or L-DOPA, but also after administration of LH-RH. In this case, LH-RH testing induced ACTH secretion. To determine if aberrant ACTH secretion in response to LH-RH loading is a common phenomenon in severe primary hypothyroidism, we performed the LH-RH test on 4 additional patients with pituitary enlargement due to primary hypothyroidism. Two patients demonstrated aberrant ACTH secretion in response to LH-RH loading, but the others did not. To our knowledge, this is the first report of aberrant LH-RH-stimulated ACTH secretion in primary hypothyroidism.
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PMID:Aberrant luteinizing hormone-releasing hormone-stimulated adrenocorticotropic hormone secretion in a patient with pituitary hyperplasia due to primary hypothyroidism. 1107 31

We report a man in whom a 15 cm. renal tumor was excised at the age of 49. The pathological examination showed a clear cell carcinoma. Five years later, he presented with headache, vomiting and unilateral palpebral ptosis. Imaging studies showed a sellar tumor with pituitary apoplexy. The tumor was excised and the pathological study disclosed a clear cell tumor, positive for vimentin, cytokeratins AE1 and AE3 and immunohistochemically negative for LH, TSH, ACTH and GH. Considering the similar histopathological features, it was considered as a metastasis of the renal tumor. The patient was supplemented with thyroid, adrenal and gonadal hormones. Seven years later, he presented a new tumor in the remaining kidney, that corresponded to a cystic papillary renal cell carcinoma. Afterwards, he presented a transitional urinary bladder tumor. Mortality associated to renal cell tumors is 90% at 5 years, and pituitary metastases are extraordinarily uncommon.
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PMID:[Apoplexy in pituitary metastasis of renal cell carcinoma. Clinical case followed for 7 years]. 1134 89

Lymphocytic hypophysitis is classically defined as an inflammatory disorder confined to adenohypophysis. However, it has recently been indicated that infundibuloneurohypophysitis underlies some subsets of central diabetes insipidus (DI). Therefore, lymphocytic hypophysitis can be considered a syndrome including disorders of both the anterior pituitary (lymphocytic adenohypophysitis) and the posterior pituitary (lymphocytic infundibuloneurohypophysitis). We describe a 77-yr-old woman with lymphocytic hypophysitis presenting with headache, diplopia, general malaise and appetite loss. Head magnetic resonance imaging (MRI) demonstrated pituitary swelling and dura mater thickening on the dorsum sella. Endocrinological investigations revealed both anterior and posterior pituitary dysfunction associated with primary hypothyroidism due to Hashimoto's thyroiditis. Headache and diplopia spontaneously disappeared, and anterior pituitary dysfunction, general malaise and appetite loss improved after taking 10 mg hydrocortisone daily, although ACTH hyposecretion persisted. Pituitary swelling was thereafter reduced but the dura mater thickening persisted. We suggest that this case may represent a variant of lymphocytic hypophysitis in which chronic inflammatory process involves both the anterior and the posterior pituitary gland, infundibulum, dura mater on the dorsum sella and cavernous sinus. Regarding expanding features of lymphocytic hypophysitis, it may be considered a syndrome including heterogeneous disorders, of which the pathogenesis remains to be elucidated.
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PMID:Lymphocytic hypophysitis: its expanding features. 1138 13

We evaluated the sensitivity of 5-HT1D receptors in patients with migraine using sumatriptan as a pharmacological probe. The drug inhibits the release of ACTH, cortisol and prolactin and this effect may be used to explore the function of serotoninergic systems in vivo. We administered sumatriptan (6 mg sc) and placebo to 15 migraineurs, during the headache-free period, and to 10 healthy controls. Blood samples were collected -15, 0, 15, 30, 45, 60 and 90 min after injections. Sumatriptan induced a significant (p<0.01) decrease of ACTH, cortisol and prolactin concentrations both in patients with migraine and in controls. The neuroendocrine response was not significantly different in the two groups. Our results suggest that 5-HT1D receptor sensitivity is not altered in migraine.
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PMID:Neuroendocrine effects of subcutaneous sumatriptan in patients with migraine. 1140 49

Histologically, cholesterol clefts are often observed in craniopharyngioma, Rathke's cleft cyst, and various granulomas. However, pituitary adenomas with cholesterol clefts are rare. A 46-year-old woman developed visual field disturbance. She had no history of severe headache that would suggest pituitary apoplexy. She presented with homonymous bitemporal hemianopsia and galactorrhea. Blood prolactin level was 63.1 ng/mL Other hypophysial hormone levels were within normal range. Magnetic resonance imaging revealed a pituitary tumor with intratumoral cyst. The cyst showed high intensity on T1- and T2-weighted images. The tumor was demonstrated with iso intensity on T1-weighted image and with high intensity on 12-weighted image. She underwent trans-sphenoidal surgery. The tumor was soft, with yellowish, oily fluid, probably the cyst content. By light microscopy with hematoxylin and eosin staining, a typical chromophobic adenoma of the pituitary was identified. Immunostaining revealed immunoreactivity for ACTH in several cells. Many cholesterol clefts and several hemosiderin pigment containing macrophages were observed. Electron microscopy demonstrated a pituitary adenoma with sparse and small secretory granules and numerous lysosomes. The cyst was most likely caused by focal hemorrhagic infarction, followed by the formation of cholesterol crystals, the appearance of hemosiderin containing macrophages, foreign body product cells, and accumulation of lysosomes.
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PMID:Pituitary Adenoma with Cholesterol Clefts. 1211 66

The majority of pituitary adenomas are solitary and monohormonal, producing only one hormone. Double or multiple adenomas are rare. Plurihormonal adenomas may be monomorphous consisting of one cell type producing more than one hormones or plurimorphous composed of two or more distinct cell populations each producing different hormones. Primary pituitary hyperplasia is uncommon and transformation to adenoma has rarely been documented. We describe a unique case of somatotroph adenoma combined with ACTH-immunoreactive cell hyperplasia and focal transformation to adenoma. The 53-yr-old man was presented with a 2-yr history of headaches, enlargement of the hands and feet and coarsening of facial features. His blood GH was 17.5 ng/ml and he had absence of GH suppressional oral glucose tolerance testing. MRI demonstrated a mass with maximum diameter of 1.5 cm, on the left side of the pituitary, without invasion of surrounding tissues. Transsphenoidal surgery was performed. Morphology disclosed a mostly chromophobic tumor, immunoreactive for GH with ultrastructural characteristics of sparsely granulated somatotroph adenoma. The adenoma cell population was focally admixed with hyperplastic PAS positive and ACTH immunoreactive cells showing the electron microscopic features characteristic of corticotrophs. In these areas the acini were enlarged with distorted architecture of the reticulin pattern. Dissolution of the reticulin fiber network and transformation of hyperplastic ACTH-immunoreactive cells to adenoma was evident in small areas. The hyperplastic and adenomatous ACTH-immunoreactive cells were admixed with somatotroph adenoma cells. Due to lack of biochemically obvious cortisol hypersecretion, this ACTH-immunoreactive adenoma was classified as silent "corticotroph" subtype 1. This is an unusual case of composite pituitary adenoma consisting of somatotroph cells and hyperplastic ACTH-immunoreactive cells transforming to a frank adenoma.
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PMID:Composite somatotroph--ACTH-immunoreactive pituitary adenoma with transformation of hyperplasia to adenoma. 1250 71

Lumbar puncture (LP) is a routine technique performed for a variety of procedures, e.g. diagnosis, administration of drugs, myelography and spinal anaesthesia. Postdural puncture headache is a common complication (30-40% in diagnostic LP). Prevention can be accomplished by using small-gauge needles (< or = 25 G) or pencil-point needles (22 G). Therapy should be carried out in a stepwise approach. The first step is bedrest, use of analgetics, i.v. fluids and an adequate guidance of the patient. The second step comprises special drug therapy. Several methods of pharmacologic management have been presented in the literature, but most of these are case reports. There is a lack of large double-blind placebo-controlled studies. Theophylline, caffeine, ACTH and sumatriptan are potentially promising agents for the treatment of postdural puncture headache. The efficacy of theophylline has been proven in a double-blind and placebo-controlled study. There are a few studies and case reports reporting that caffeine p.o. and i.v. is effective in the treatment of postdural puncture headache, but recurrence of headache after caffeine therapy is frequent. ACTH acts on a complex hormonal system. The treatment with sumatriptan has been reported in only a few case reports. The third step, and one of the most effective treatments of postdural puncture headache, is the epidural blood patch. The success rate ranges between 80 and 97%.
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PMID:[Postdural puncture headache: diagnosis, prevention and therapy]. 1279 21


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