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1/22. Thickened pituitary stalk with central diabetes insipidus: report of three cases.

    diabetes insipidus of central origin usually results from lesions in the hypothalamic neurohypophyseal system. Lymphocytic infundibuloneurohypophysitis is an uncommon cause. Cases of lymphocytic infundibuloneurohypophysitis with thickening of the pituitary stalk and enlargement of the neurohypophysis with no hyperintense signal in the posterior pituitary have been reported. Reported cases presenting with isolated thickening of the pituitary stalk are very rare. We report three such cases, one in a nulliparous woman and the other two in men. Magnetic resonance (MR) imaging in these patients revealed isolated thickening of the pituitary stalk, loss of the hyperintense signal of the posterior pituitary, and an adenohypophysis of normal size. All cases had abnormal nodular infundibular enlargement. One male patient had hypogonadism; the other patients showed no sign of adenohypophyseal deficiency on stimulation test. Serial follow-up MR imaging revealed that all three patients had persistent thickening of the pituitary stalk. diabetes insipidus was controlled by the administration of desmopressin acetate in all patients.
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2/22. A missense mutation encoding Cys73Phe in neurophysin II is associated with autosomal dominant neurohypophyseal diabetes insipidus.

    Autosomal dominant neurohypophyseal diabetes insipidus (ADNDI) is an inherited disease caused by progressive deficiency of the hormone arginine vasopressin (AVP) that typically becomes clinically apparent in the first decade of life. The genetic locus of ADNDI is the arginine vasopressin-neurophysin II (AVP-NPII) gene and mutations that cause ADNDI have been found in the nucleotides encoding the signal peptide, vasopressin, and neurophysin II peptides. In this study we have analyzed the AVP-NPII gene in a 20-year-old female who was diagnosed with ADNDI at 2 years of age. A heterozygous missense mutation (1684G>T) was found in exon 2 that predicts replacement of cysteine with phenylalanine at position 73 of neurophysin II. The mutation was confirmed by subcloning exon 2 PCR products to sequence each allele independently. Two out of four clones were found to have the missense mutation and two have the normal sequence, confirming the presence of the mutation and heterozygosity. Neurophysin II is an intracellular carrier protein for AVP during axonal transport from the hypothalamus to the posterior pituitary and contains 14 cysteine residues forming 7 disulfide bonds. This mutation is predicted to disrupt the disulfide bridge between Cys73 and Cys61 within the neurophysin II moiety. This finding of a novel mutation substituting cysteine with phenylalanine in one AVP-NPII gene allele supports the hypothesis that inability to form normal disulfide bonds in neurophysin II leads to ADNDI.
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3/22. pituitary apoplexy in association with lymphocytic hypophysitis.

    pituitary apoplexy has been recognised much more frequently since the introduction of CT and MRI scanning. Lymphocytic hypophysitis has been increasingly diagnosed in recent years. A case of pituitary apoplexy occurring in a patient with lymphocytic hypophysitis as part of a polyglandular syndrome is reported. This combination does not appear to have been previously reported. The pituitary haemorrhage was confirmed on MRI and at surgery. Lymphocytic hypophysitis was confirmed histologically. The apoplexy was accompanied by severe headache, elevation of the optic chiasm, developing field loss and onset of ptosis. The apoplexy was precipitated by neck extension.
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keywords = pituitary, gland
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4/22. survival with serum sodium level of 180 mEq/L: permanent disorientation to place and time.

    A 41-year-old woman who had undergone transfrontal craniotomy for a pituitary tumor 4 months before presentation was admitted with confusion and orientation only to self. She had a fever of 40 degrees C. serum sodium and chloride levels on admission were 180 and 139 mEq/L, respectively. Measured serum osmolality was 380 mOsmol/L with a urine osmolality of 360 mOsmol/L. magnetic resonance imaging revealed a 1.5-cm mass in the sella turcica, which was nonfunctioning on endocrine evaluation. The "bright spot" of a normal posterior pituitary was absent. Central diabetes insipidus was confirmed by a 300% increase in urine osmolality with desmopressin. The patient survived her severe hypernatremia, which has 70% mortality with a serum sodium level of 160 mEq/L or above. However, she developed permanent (6 months) disorientation to time and place even when hypernatremia was corrected, which has not been described previously.
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5/22. Langerhans' cell histiocytosis diagnosed through periodontal lesions: a case report.

    BACKGROUND: Langerhans' cell histiocytosis (LCH) is a rare disorder in which a neoplastic proliferation of cells similar to the Langerhans' cell of the epidermis is observed. The disease may affect different organs as solitary or multiple lesions with a complete dissemination in different organs such as the bone (jaws), lung, hypothalamus, skin and mucous membranes, lymph nodes, liver, and other tissues. Although LCH is considered a childhood or juvenile disease, the diagnosis is often made in adults, and in many cases, a juvenile form progresses into adult life. hand-Schuller-Christian disease (HSCD) is an LCH form in which the typical lesions Involve the cranial bones, the eyes, and the pituitary gland. methods: This article describes a case of a patient diagnosed, through periodontal lesions and diabetes insipidus, as having Langerhans' cell histiocytosis. CONCLUSION: As far as LCH lesions appearing in periodontal tissues, the periodontist should be involved in the detection of oral lesions.
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keywords = pituitary gland, pituitary, gland
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6/22. Transient central diabetes insipidus in pregnancy with a peculiar change in signal intensity on T1-weighted magnetic resonance images.

    A 38-year-old woman was admitted with severe thirst and polyuria at 31 weeks' gestation. The plasma concentration of vasopressin (AVP) was very low (0.73 pg/ml) under conditions of high plasma osmolality (316 mOsm/ kg). T1-weighted magnetic resonance (MR) images revealed enlargement of the pituitary posterior lobe with absence of the hyperintense signal. After delivery, restoration of the hyperintense signal was demonstrated. This depletion-repletion process, which reflects the decrease and increase in amount of neurosecretory granules, is recognized in the case of transient central diabetes insipidus during pregnancy. We consider that an increase in cystine-aminopeptidase (CAP) activity is implicated in the pathogenesis.
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7/22. Transient lymphocytic panhypophysitis associated with SIADH leading to diabetes insipidus after glucocorticoid replacement.

    A 52-year-old man presented with vomiting, general fatigue and hyponatremia. His symptoms and signs were consistent with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Endocrine studies revealed hypopituitarism and administration of hydrocortisone resulted in a marked polyuria. The patient was diagnosed as masked diabetes insipidus. The lymphocytic hypophysitis was also diagnosed on the basis of MRI findings and anti-pituitary antibody. Six months later, these abnormalities disappeared. diabetes insipidus may exist in a case of hyponatremia due to contrastive SIADH. Such patients may recover spontaneously and careful follow-up is required, avoiding a long-term treatment by monotonous continuation of hormonal replacement.
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8/22. A rare case of central post-gravid diabetes insipidus.

    Central diabetes insipidus (CDI) arising in the puerperal period has been attributed mainly to Sheehan's syndrome or to lymphocytic infundibulo-neurohypophysitis. We report the case of a 24-year-old woman who came to our observation for the appearance, 3 weeks after a normal delivery, of a polyuric-polydipsic syndrome. Measurements of urinary volumes, plasma osmolality and urinary osmolality, in conditions of free water intake, water deprivation and a water deprivation-vasopressin administration test, demonstrated CDI. brain magnetic resonance imaging showed a normal morphology of the adenohypophysis and total absence of the neurohypophysis. Assays of the pituitary hormones were found to be within normal limits. These results, incompatible with a diagnosis of Sheehan's syndrome and lymphocytic infundibulo-neurohypophysis, excluded all the other known causes of acquired CDI. Our diagnosis was therefore of post-gravid idiopathic CDI. Thus, it is possible that in the puerperal period other diseases of the posterior hypophysis may develop, of unknown etiopathogenesis but equally responsible for CDI.
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9/22. A case of anterior hypopituitarism showing recurrent pituitary mass associated with central diabetes insipidus.

    We report a case of anterior hypopituitarism showing recurrent pituitary mass associated with central diabetes insipidus. A 76-year old woman was hospitalized with general fatigue and 5 kg body weight loss. Endocrinological examinations and pituitary provocative tests demonstrated hypopituitarism and central diabetes insipidus. T1-weighted image of magnetic resonance imaging (MRI) revealed an intrasellar cystic mass with ring enhancement suggesting pituitary abscess. MRI films subsequently obtained from another hospital and studied retrospectively showed intrasellar cystic mass with ring enhancement 4 years earlier, and a mass shape that was decreased after 2 years. Over the subsequent years, the patient has remained asymptomatic with hormone replacement therapy only. Cystic pituitary adenoma or Rathke's cleft cyst with repeated infection may be involved in the repeated change of pituitary mass shape although neither pituitary surgery nor a pituitary biopsy was performed because of the patient's age and condition. It is reported that apparent recurrence of Rathke's cleft cysts after initially successful surgery was higher than suggested by previous reports, and that long-term follow-up with pituitary imaging and neuroophthalmological assessment is essential. Careful evaluation by follow-up brain MRI is needed in the present case to prevent future recurrence of pituitary abscess.
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ranking = 1.3
keywords = pituitary
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10/22. pituitary apoplexy due to prolactinoma in a Taiwanese boy: patient report and review of the literature.

    We report a Taiwanese boy who presented with apoplexy of a prolactinoma. A 12 9/12 year-old boy presented to our clinic with headache and visual deficit of bitemporal hemianopsia. skull X-ray showed an enlarged sella. Magnetic resonance imaging (MRI) of the sella turcica showed a 4 x 2.5 x 2.5 cm mass, located at the sella turcica and extending upward to compress the optic chiasm. Preoperative laboratory data showed hyperprolactinemia, hypothyroidism and hypocortisonism. After a stress dose of i.v. hydrocortisone was given, he underwent transsphenoid surgery to remove the tumor. Immunohistochemical stains were positive for PRL in the tumor cells. After surgery, he suffered from neurogenic diabetes insipidus, hypopituitarism and hyperprolactinemia, with serum PRL level of 491 ng/ml. Visual field examination was normal 4 months later. In conclusion, pituitary apoplexy is rare in children but should be considered if a patient suffers from headache, vomiting, and visual deficit. brain MRI is preferred for diagnosis. Dopaminergic agonists should be given if residual tumor or recurrence of prolactinoma is found after transsphenoidal surgery.
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