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1/8. 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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2/8. 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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3/8. hypopituitarism and diabetes insipidus with localized hypertrophic pachymeningitis (tolosa-hunt syndrome) associated with Hashimoto thyroiditis.

    We report a 69-year-old woman with intracranial pachymeningitis showing hypopituitarism, diabetes insipidus, and tolosa-hunt syndrome associated with Hashimoto thyroiditis confirmed by autopsy. A large tumorous lesion of the hypothalamo-pituitary gland was revealed on magnetic resonance imaging, after the patient complained of gait and visual field disturbance. These symptoms subsided after thyroid hormone supplementation. hypopituitarism and diabetes insipidus were diagnosed after cessation of the treatment by the patient herself. Multiple cranial nerve palsies and orbito-frontalgia appeared. methylprednisolone pulse therapy improved the symptoms, but they recurred when the dose of glucocorticoid was decreased. The patient died of brain thrombosis. autopsy revealed typical findings of Hashimoto thyroiditis and intracranial pachymeningitis involving the cranial base and pituitary gland. The high titer of rheumatoid factor and Hashimoto thyroiditis in this patient suggest an immunological role in the pathogenesis of pachymeningitis.
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4/8. Serial MR intensity changes of the posterior pituitary in patients with diabetes insipidus after transsphenoidal surgery for pituitary adenomas: report of two cases.

    This is the first report describing magnetic resonance (MR) intensity changes of the posterior pituitary gland in the patients suffering from the classical "triphasic" diabetes insipidus (DI) after transsphenoidal surgery for pituitary adenomas. A 21-year-old female and a 54-year-old female were admitted to our hospital with the diagnosis of Cushing's disease and acromegaly due to pituitary microadenomas, respectively. No evidence of DI was found, and T1-weighted MR images exhibited "bright spot" corresponding to the posterior pituitary in both cases. Both experienced the classical "triphasic" pattern of water metabolism disturbance after successful transsphenoidal resection of pituitary adenomas, that is, polyuria-oliguria-polyuria. The MR signal hyperintensity in posterior pituitary was detected during the first polyuric phase, but the hyperintensity disappeared during the second polyuric phase. In addition, "bright spot" was restored along with the recovery from DI in the chronic phase. These findings of serial MR images supported that the first DI phase of the classical triphasic course of water metabolism disturbance was caused by secretional dysfunction of stored vasopressin from the posterior gland, whereas the second DI phase was due to impairment in the functional integrity producing vasopressin-containing granules after depletion of vasopressin in the oliguric phase.
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5/8. pituitary apoplexy precipitating diabetes insipidus.

    OBJECTIVE: To describe a case of pituitary apoplexy complicated by diabetes insipidus and to review management of patients with pituitary apoplexy and water excretion disturbances associated with transsphenoidal surgery. methods: We describe clinical, laboratory, and radiologic findings in a patient with pituitary apoplexy and central diabetes insipidus. RESULTS: A 74-year-old woman presented with severe headache and sudden loss of vision for 12 hours, accompanied by thirst and frequent urination. Visual field examination demonstrated bitemporal hemianopsia. Her laboratory findings were significant for a serum sodium level of 152 mEq/L and urine specific gravity of <1.005. A magnetic resonance imaging scan of her pituitary gland identified a 3.5-cm suprasellar mass compressing the optic chiasm. She subsequently underwent transsphenoidal pituitary surgery with subtotal resection of this mass. Microscopic evaluation of tumor tissue revealed a pituitary adenoma with evidence of recent infarct and hemorrhage. Her clinical and biochemical course was consistent with the triphasic response that may occur after pituitary surgery related to damage to the hypothalamus and supraopticohypophyseal tract. CONCLUSION: pituitary apoplexy may be rarely associated with diabetes insipidus.
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6/8. Novel mutant vasopressin-neurophysin II gene associated with familial neurohypophyseal diabetes insipidus.

    We describe a novel missense mutant of arginine vasopressin (AVP)-dependent neurohypophyseal diabetes insipidus in an autosomal dominant family. A 54-year-old woman was admitted to our hospital because of thyroidectomy for thyroid cancer. After thyroidectomy she was found to have hypernatremia and polyuria and polydipsia both of which had been present from childhood. She had no obstructive hydronephrosis. Her father, father's younger sister and her third son also had polyuria and polydipsia. Basal plasma AVP concentration at normal plasma osmolality was normal but did not respond to increased plasma osmolality despite hyperosmolality during infusion of hypertonic saline infusion, indicating that plasma AVP secretion was impaired. sodium concentration in urine and urine osmolality were low and increased after nasal administration of DDAVP. There was a diminished but bright signal of pituitary posterior gland on magnetic resonance T1 weighted image. Molecular genetic analysis demonstrated that the patient and her son had a single heterozygous missense mutation (G-->A) at nucleotide 1829 in 1 AVP allele, yielding an abnormal AVP precursor with lacking Glu-47 in its neurophysin II moiety. The abnormal AVP precursor may be related to the impaired AVP secretion.
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7/8. Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl.

    CASE REPORT: We report a case of central diabetes insipidus, hypothyroidism, and subsequent hypopituitarism due to lymphocytic hypophysitis masking a germinoma in a 13-year-old pubertal girl. Magnetic resonance revealed an enlarged pituitary gland and a mass lesion in the pituitary stalk and inferior hypothalamus. Open cranial surgery of the anterior pituitary showed active hypophysitis with lymphocytic infiltrates but without necrosis. Despite prednisolone therapy, 1 year later an enlarged, irregular cystic mass lesion had developed; in the pituitary stalk and inferior hypothalamus, a endoscopic biopsy revealed germinoma. CONCLUSION: Lymphocytic hypophysitis in children may be the first sign of a host reaction to an occult germinoma. The diagnosis of central diabetes insipidus with a thickened pituitary stalk requires long-term follow-up to establish the underlying cause.
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8/8. Wegener's granulomatosis presenting as diabetes insipidus.

    pituitary gland involvement in Wegener's granulomatosis (WG) occurs most commonly in the form of central diabetes insipidus (CDI). However, CDI as a presenting manifestation of WG is very rare. We report two such cases; one of them had multi-organ involvement at presentation, while other developed it during follow-up. CDI was reversible following cytotoxic drug therapy in one of them.
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