Cases reported "Pituitary Diseases"

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1/14. Hypertonic saline test for the investigation of posterior pituitary function.

    The hypertonic saline test is a useful technique for distinguishing partial diabetes insipidus from psychogenic polydipsia, and for the diagnosis of complex disorders of osmoreceptor and posterior pituitary function. However, there is little information concerning its use in childhood. The experience of using this test in five children (11 months to 18 years) who presented diagnostic problems is reported. In two patients, in whom water deprivation tests were equivocal or impractical, an inappropriately low antidiuretic hormone (ADH) concentration (< 1 pmol/l) was demonstrated in the presence of an adequate osmotic stimulus (plasma osmolality > 295 mosmol/kg). In two children--one presenting with adipsic hypernatraemia and the other with hyponatraemia complicating desmopressin treatment of partial diabetes insipidus--defects of osmoreceptor function were identified. Confirming a diagnosis of idiopathic syndrome of inappropriate ADH secretion (SIADH) was possible in a patient with no other evidence of pituitary dysfunction. The hypertonic saline test was well tolerated, easy to perform, and diagnostic in all cases.
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keywords = polydipsia
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2/14. An elderly patient with transient diabetes insipidus associated with lymphocytic infundibulo-neurohypophysitis.

    We present the eldest case ever reported of central diabetes insipidus (DI) associated with infundibulo-neurohypophysitis. A 77-year old woman, who complained of recent development of excessive thirst, polyuria and polydipsia, was referred to our hospital. The daily urine volume was markedly increased to 6 L. DDAVP administration effectively reduced urine volume and increased urine osmolality. The loading test using high-osmolar sodium chloride showed impaired excretion of vasopressin discordant with plasma osmolar changes. The anterior pituitary function was normal. Pituitary magnetic resonance imaging (MRI) showed thickening of the pituitary stalk and a lack of high-intensity signal of the neurohypophysis on T1-weighted images, suggestive of lymphocytic infundibulo-neurohypophysitis. The thickness of pituitary stalk on MRI improved 6 months later. DI was controlled with DDAVP for 40 days. This was followed by stabilization of the daily urine volume to less than 2.5 L without DDAVP. Our case is the eldest case of central DI associated with infundibulo-neurohypophysitis. The rapid remission of pituitary changes on MRI provides an insight that spontaneously partial remission of central DI may occur, resulting in transient polyuria and polydipsia.
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keywords = polydipsia
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3/14. Hypophyseal tuberculoma: direct radiosurgery is contraindicated for a lesion with a thickened pituitary stalk: case report.

    OBJECTIVE AND IMPORTANCE: Hypophyseal tuberculomas are extremely rare lesions. The recognition of hypophyseal tuberculomas in the differential diagnosis of pituitary tumors is important, even with no evidence of systemic tuberculosis. CLINICAL PRESENTATION: A 27-year-old female patient presented with continuous, dull, generalized headaches and amenorrhea, with no history of visual diminution, galactorrhea, or endocrinological abnormalities and no evidence of systemic tuberculosis. The patient exhibited a normal water balance, without polyuria or polydipsia. A gynecological examination, including an endometrial biopsy for amenorrhea, did not reveal any abnormalities. Perimetric and endocrinological examination results were normal. Contrast magnetic resonance imaging revealed a dense enhancing intrasellar mass, with thickening of the pituitary stalk. INTERVENTION: Sublabial rhinoseptal transsphenoidal decompression of the lesion was performed. The histopathological features were consistent with a diagnosis of tuberculoma, and acid-fast bacilli were demonstrated in the surgically removed tissue with Ziehl-Neelsen staining. As soon as the histopathological features were known, the patient underwent a lumbar puncture for cerebrospinal fluid analysis, which indicated normal findings. An intradermal tuberculin test yielded negative results. The patient was treated with medical therapy for 18 months, and complete resolution of the lesion was observed in follow-up examinations. CONCLUSION: Hypophyseal tuberculomas are often mistaken for pituitary adenomas. The finding of a thickened pituitary stalk in contrast magnetic resonance imaging scans may be useful for the differentiation of these lesions from pituitary adenomas. Direct radiosurgery is not an appropriate primary treatment method for pituitary adenomas and is principally restricted to elderly, medically unfit patients with microadenomas and patients with residual or recurrent tumors after microsurgery. It is contraindicated for patients who exhibit a thickened pituitary stalk in contrast magnetic resonance imaging scans.
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keywords = polydipsia
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4/14. A case of lymphocytic infundibuloneurohypophysitis: histophathological studies.

    A 57-year-old woman presented with 2-year history of polyuria and polydipsia. Hormonal studies revealed almost normal anterior pituitary function and central diabetes insipidus. magnetic resonance imaging showed thickening of the pituitary stalk and enlargement of the neurohypophysis without high intensity of the posterior lobe on T1-weighted images, which were compatible with lymphocytic infundibuloneurohypophysitis. Transsphenoidal biopsy was done and histological examination disclosed moderate fibrosis and lymphocytic infiltration not only in the posterior pituitary, but also in the adjacent anterior pituitary part of the gland. The lymphocytes both in the anterior and posterior pituitary were mainly T cells that were positive for UCHL 1, CD 3, and CD 8. Immunofluorescence of frozen tissue detected immunecomplex deposition in small vessels and the interstitium. These findings suggested that allergic reactions may play an important role in the pathogenesis of lymphocytic infundibuloneurohypophysitis.
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ranking = 1
keywords = polydipsia
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5/14. Langerhans cell histiocytosis limited to the pituitary-hypothalamic axis--two case reports.

    Langerhans cell histiocytosis rarely presents as a solitary lesion in the pituitary-hypothalamic region, and is indistinguishable from germinoma, which occurs much more frequently, especially in Japanese. A 14-year-old girl and a 9-year-old girl presented with polydipsia and polyuria as the initial symptoms. Magnetic resonance (MR) imaging demonstrated a round mass at the pituitary stalk appearing as isointense on T1-weighted imaging and hyperintense on T2-weighted imaging. Endocrinological examination revealed mild hypopituitarism with central diabetes insipidus. Both patients underwent open craniotomy. Histological examination revealed granulomatous tissue with eosinophil infiltration and frequent Langerhans histiocyte clustering, compatible with the diagnosis of Langerhans cell histiocytosis. Low-dose local irradiation of 20 Gy was administered. First patient was followed up for 8 years, and her hypopituitarism gradually improved to a minimal level with only amenorrhea as the residual symptom. Recent MR imaging showed no residual mass at the region. Second patient was followed up for 15 months, and her diabetes insipidus is stable. MR imaging performed 5 months after the treatment showed marked reduction of the mass. These cases reemphasize the importance of histological diagnosis for lesions with similar neuroimaging appearances. biopsy and low-dose irradiation are an effective treatment for this rare and essentially benign lesion, as opposed to attempting total removal of the mass.
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keywords = polydipsia
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6/14. Neurohypophyseal germinoma traced from its earliest stage via magnetic resonance imaging: case report.

    BACKGROUND: We report a case in which magnetic resonance imaging (MRI) documented the formation of a neurohypophyseal germinoma from its earliest stage. CASE DESCRIPTION: A 9-year-old girl presented with polyuria, polydipsia, and growth retardation. Urinary and endocrinological examination indicated diabetes insipidus (DI) and growth hormone deficiency. No clear evidence of a mass in the hypothalamo-pituitary area was apparent on the first MRI scan. Follow-up MRIs and endocrinological examinations revealed that a tumor, arising from the stalk and posterior lobe of the pituitary gland, formed a mass and caused anterior pituitary function to deteriorate. Histologic diagnosis was germinoma based on open biopsy. CONCLUSION: Radiologic findings in this case indicated the primary site of this intrasellar germinoma to be the hypothalamo-neurohypophyseal region, designating this a "neurohypophyseal germinoma."
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ranking = 1
keywords = polydipsia
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7/14. Inflammatory pseudotumor of the pituitary: case report.

    BACKGROUND: Inflammatory pseudotumor of the pituitary is a very rare nonneoplastic lesion. We describe a case of a patient with past history of lymphocytic meningitis. CASE DESCRIPTION: A 32-year-old man presented with polyuria, polydipsia, anorexia, abdominal discomfort, and panhypopituitarism. He had 2 episodes of lymphocytic meningitis in the last two years. Magnetic resonance image (MRI) disclosed a sellar and suprasellar mass with extension to the pituitary stalk. The patient underwent transnasal-transsphenoidal surgery to remove the lesion. Histopathological findings revealed mixed inflammatory cells composed mainly of lymphocytes, macrophages and extensive fibrosis. CONCLUSIONS: Inflammatory pseudotumor of the pituitary, although rare, should be included in the differential diagnosis of a sellar and suprasellar mass.
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ranking = 1
keywords = polydipsia
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8/14. Delayed lymphocytic infundibuloneurohypophysitis following successful transsphenoidal treatment of Cushing's disease.

    Lymphocytic infundibuloneurohypophysitis is a rare disorder in which neurohypophyseal function is impaired by an autoimmune process. Although several etiologies for this rare entity have been suggested, its occurrence following transsphenoidal adenomectomy has not been reported. A 20-year-old man presented with diabetes insipidus - seven years after successful transsphenoidal microadenomectomy for Cushing's disease, first diagnosed at the age of 13. Seven years later, he developed fairly rapid onset of polydipsia and polyuria. magnetic resonance imaging demonstrated swelling of the posterior pituitary gland with thickening of the pituitary stalk. Endocrinological evaluation revealed neurohypophyseal dysfunction without the adenohypophysis being affected. On the basis of these findings, a diagnosis of lymphocytic infundibuloneurohypophysitis was made. The mass lesion of the posterior pituitary resolved after the administration of corticosteroids for two months and no operation was required. Lymphocytic infundibuloneurohypophysitis should be considered in the differential diagnosis of pituitary mass lesions following transsphenoidal surgery, especially when the mass is confined to the posterior pituitary gland with neurohypophyseal function being compromised.
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ranking = 1
keywords = polydipsia
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9/14. Empty sella turcica in intracranial sarcoidosis. Pituitary insufficiency, primary polydipsia, and changing neuroradiologic findings.

    A 37-year-old man with visual loss was found to have hypopituitarism and primary polydipsia associated with sarcoidosis. Neuroradiologic studies demonstrated a dramatic evolution of CNS lesions, including a left thalamic infarct, an enhancing suprasellar mass, and ultimately an empty sella turcica. The patient has been clinically stable in spite of these changes. This case is likely to be the first reported of CNS sarcoidosis with an empty sella turcica documented by computed tomography.
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ranking = 5
keywords = polydipsia
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10/14. Neurosarcoidosis presenting as secondary amenorrhea in a teenager.

    sarcoidosis is a systemic inflammatory disease of unknown etiology characterized by non-caseating epithelioid cell granulomata. The lungs and reticulo-endothelial system are typically involved, and virtually any organ system may be affected. sarcoidosis involving the central nervous system is relatively uncommon, estimated to occur in approximately 5% of patients with sarcoidosis in the united states, while the incidence throughout the world may be as high as 15%. Hypothalamic dysfunction is the most common manifestation of central nervous system parenchymatous disease in neurosarcoidosis. polyuria and polydipsia are the most frequently occurring symptoms in patients with sarcoidosis who have dysfunction of the pituitary and hypothalamus. We describe a patient with secondary amenorrhea resulting from neurosarcoidosis involving the pituitary and hypothalamus.
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keywords = polydipsia
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