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1/55. Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis.

    OBJECTIVES: We retrospectively reviewed 5 patients with neurosarcoidosis, who all presented with central diabetes insipidus and hypogonadism. DESIGN: This was a single-centre, retrospective analysis of 5 cases with a minimum follow-up of 2 years. methods: Case analysis included clinical, biochemical, and endocrinological evaluation and frequent CT/MRI scans of involved organs as primary evaluation and in response to immunosuppressive therapy. RESULT: Neurosarcoidosis was diagnosed in all patients. Two patients had no proven extracerebral manifestation and had a stable disease over 3 and 5 years. One patient showed deterioration with corticosteroids alone but partial remission after additional cyclophosphamide. Pituitary dysfunction remained unchanged in all patients, despite total clinical and radiological remission in two patients. However, one of these patients died of acute granulomatous meningoencephalitis after two years of follow-up. CONCLUSION: Although the presenting symptoms of neurosarcoidosis may vary, the occurrence of central diabetes insipidus associated with typical radiological features is suggestive of neurosarcoidosis. However, there is an increasing number of case reports on lymphocytic hypophysitis. Without the bioptic diagnosis, the differentiation between potentially lethal isolated neurosarcoidosis and lymphocytic hypophysitis is difficult. These cases demonstrate the difficulties in diagnosing neurosarcoidosis and reflect experiences with follow-up parameters. ( info)

2/55. Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature.

    This study was conducted to evaluate the signs and symptoms on admission, diagnosis, localization, therapy, and survival of patients with primary intracranial germ cell tumors (PICGCT). Eight patients with surgically confirmed PICGCTs were treated and followed up at Hacettepe University's Department of Pediatric Oncology between 1974 and 1995. While one patient was admitted with a second recurrence of her disease, the others were admitted or referred primarily to our institution. In this period, 357 germ cell tumor and 684 primary intracranial malignant tumors were diagnosed and treated at our institution. Thus, PICGCTs comprised 1.1 percent of the primary intracranial malignant tumors and 2.2 percent of the germ cell tumors. There were four females and four males and the median age was eight years (13 months to 12 years). On admission, the most common symptoms were diabetes insipidus (3/8) and vomiting (3/8). One patient also and Down's syndrome. Locations of the tumors were suprasellar in three, in the third ventricle in two, and in the cerebral parenchyma, and pineal and hypothalamic regions in the remainder. There were germinomas, three malignant teratomas, and two mixed germ cell tumors. Only two patients could be treated with appropriate and adequate chemotherapy and radiotherapy. Three patients died: one in the postsurgical period, one after the third surgical approach and one 11 months after the diagnosis of progressive disease; three were lost to follow-up. The remaining two patients (with second recurrence and disseminated disease) are alive and without disease. Our experience with these patients demonstrated that appropriate and adequate chemotherapy is as effective a treatment as radiotherapy, even with recurrence of the disease. ( info)

3/55. Hyponatraemia associated with lamotrigine in cranial diabetes insipidus.

    We report the cases of two children with cranial diabetes insipidus who were treated with lamotrigine for seizures and who had accompanying changes in desmopressin requirements. Lamotrigine is a new anticonvulsant chemically unrelated to other existing antiepileptic drugs. Studies suggest it acts at voltage-sensitive sodium channels and also decreases calcium conductance. Both of these mechanisms of action are shared by carbamazepine, which can cause hyponatraemia secondary to inappropriate secretion of antidiuretic hormone. It is possible that the effect of lamotrigine on fluid balance in the cases described is also centrally mediated. ( info)

4/55. A missense mutation encoding cys(67) --> gly in neurophysin ii is associated with early onset autosomal dominant neurohypophyseal diabetes insipidus.

    Autosomal dominant neurohypophyseal diabetes insipidus (ADNDI) is an inherited disorder in which progressive degeneration of magnocellular neurons of the hypothalamus impairs production of arginine vasopressin (AVP). ADNDI is caused by mutations in the arginine vasopressin-neurophysin II (AVP-NPII) gene. These mutations are hypothesized to trigger neurodegeneration via disruption of preproAVP-NPII processing. Affected individuals usually develop diabetes insipidus between 1 and 6 years of age. Here we report a novel mutation of the AVP-NPII gene in a family with unusually early presentation of ADNDI. The index case developed symptoms of diabetes insipidus at 1 month of age, her mother at 9 months of age, and the maternal grandfather in early childhood. Each was found to be heterozygous for the missense mutation 1665T > G encoding the amino acid substitution C67G within NPII. This mutation helps to define two homologous regions of the AVP-NPII precursor bounded by disulfide bridges between C13 and C27 and between C61 and C73 that have structural homology and contain the majority of amino acid substitutions associated with ADNDI. The early onset of symptomatic diabetes insipidus in this family suggests that the C67G substitution may be particularly deleterious to magnocellular neurons and may provide a valuable model for study of dominantly inherited neurodegeneration. ( info)

5/55. A clinical feature of hyperlipidemia in patients with central diabetes insipidus.

    In this study, we analyzed plasma lipid and lipoprotein levels before and after treatment with 1-desamino-8-D-arginine vasopressin (DDAVP) in subjects with partial and complete central diabetes insipidus (DI) in order to determine how a shortage and supplement of this hormone affect plasma lipid metabolism. The subjects consisted of 6 patients with partial and 6 with complete central DI. After treatment with DDAVP through nasal cavity, plasma total cholesterol (TC) level did not decrease either in complete or partial form. plasma triglyceride (TG) levels decreased from 306 /-175 mg/dl to 198 /-91 (35% decrease, p=0.027) in complete form, while TG did not change significantly in partial form. A detailed investigation of plasma lipoprotein metabolism during treatment with DDAVP was carried out in 3 of the 6 subjects with complete form of DI. lipoprotein lipase activity and mass in post-heparin plasma from those three subjects tended to increase after treatment with DDAVP, along with the complete disappearance of an unusual lipoprotein between low density lipoprotein (LDL) and very low density lipoprotein (VLDL) as analyzed by polyacrylamide gel electrophoresis. These results suggest that the DDAVP treatment has a favorable effect on lipid and lipoprotein metabolism, especially triglyceride-rich lipoproteins, either directly or through modifying factors contributing to lipid metabolism. ( info)

6/55. hyponatremia and polyuria in children with central diabetes insipidus: challenges in diagnosis and management.

    Five patients with well-controlled, long-standing, central diabetes insipidus had acute development of dehydration, hyponatremia, and inappropriate natriuresis in the setting of polyuria resistant to exogenous antidiuretic hormone. hyponatremia and dehydration worsened with fluid restriction or use of exogenous antidiuretic hormone. We discuss the challenges in diagnosis and management of probable salt wasting in children with central diabetes insipidus. ( info)

7/55. Perioperative management of central diabetes insipidus in kidney transplantation.

    Central diabetes insipidus is clinically masked in dialysis patients. We report a 12-year-old girl receiving a living-related donor graft for renal failure from Alport syndrome, in whom a craniopharyngioma had been resected 6 months before transplantation. Pretransplant evaluation had documented central hypothyroidism, growth hormone deficiency, and presumptive hypogonadotropic hypogonadism. The corticotropin-releasing factor test had been normal. Four hours after transplantation, urine output exceeded 1,000 ml/h without diuretic therapy. serum sodium concentration was 155 mmol/l, serum osmolality 333 mmol/kg, and plasma antidiuretic hormone 4.9 ng/l, while urine osmolality was 233 mmol/kg. Desmopressin acetate was started by continuous intravenous infusion at 1 microgram/day. serum electrolytes rapidly normalized, urine output stabilized at 2 l/day. The patient was discharged 4 weeks after transplantation with good allograft function, receiving intranasal desmopressin acetate 10 micrograms twice daily. Pre-existing central diabetes insipidus is unmasked after successful kidney transplantation, leading to rapid dehydration and hypernatremia, which can be prevented by prompt institution of desmopressin therapy. ( info)

8/55. Central diabetes insipidus following carbon monoxide poisoning.

    diabetes insipidus is a rare complication after carbon monoxide poisoning. We report on a woman and her daughter who were noted to have polyuria and hypernatremia after exposure to carbon monoxide. Both patients were responsive to desmopressin therapy, and the diagnosis of central diabetes insipidus was made. The development and correction of hypernatremia further complicated the neurological damages caused by carbon monoxide poisoning. These cases illustrate the possibility of central diabetes insipidus and hypernatremia after carbon monoxide poisoning and the importance of immediate antidiuretic hormone treatment and careful fluid therapy. ( info)

9/55. 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. ( info)

10/55. Central diabetes insipidus due to herpes simplex in a patient immunosuppressed by Cushing's syndrome.

    OBJECTIVE: To describe a patient immunocompromised by Cushing's syndrome in whom central diabetes insipidus developed as a complication of herpes simplex involvement of the hypothalamus. methods: We present a case, including results of laboratory and histopathologic studies, in which herpes simplex was established as the causative agent for central diabetes insipidus. RESULTS: A woman with ectopic corticotropin-dependent Cushing's syndrome, diabetes mellitus, carcinoid tumor, and a history of thyroid cancer had the precipitous onset of seizure and fever, and hypotonic polyuria and progressive hypernatremia developed. Central diabetes insipidus was diagnosed and successfully treated with desmopressin. Nevertheless, the patient's condition deteriorated and she died. autopsy revealed herpes simplex encephalitis involving the magnicellular neurons of the hypothalamus. CONCLUSION: Central diabetes insipidus caused by viral infections has been reported in immunosuppressed patients, such as those with acquired immunodeficiency syndrome (AIDS). To our knowledge, this is the first report of a herpes infection causing diabetes insipidus in a patient immunosuppressed by Cushing's syndrome. This case demonstrates that, in patients with Cushing's syndrome, diabetes insipidus may develop as a result of herpes simplex infection of the hypothalamus. ( info)
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