deamino-arginine-vasopressin and Central-Nervous-System-Cysts

Presented case demonstrates a rare diencephalic pathology - adipsic diabetes insipidus (ADI) with severe hypernatremia in a 58-year-old woman after ttranssphenoidal removal of stalk intraventricular craniopharyngioma. ADI was diagnosed because of hypernatremia (150-155 mmol/L), polyuria (up to 4 liters per day) and absence of thirst. Normalization of water-electrolyte balance occurred on the background of desmopressin therapy and sufficient hydration in postoperative period. After release from the hospital, the patient independently stopped desmopressin therapy and did not consume an adequate amount of fluid of the background of polyuria. This led to severe hypernatremia (155-160 mmol/L) and rough mental disorders.Patients with ADI need closely monitoring of medical condition and water-electrolyte parameters, appointment of fixed doses of desmopressin and adequate hydration.

Topics: Central Nervous System Cysts; Craniopharyngioma; Deamino Arginine Vasopressin; Diabetes Insipidus; Diabetes Mellitus; Female; Humans; Hypernatremia; Middle Aged; Neurosurgical Procedures; Polyuria; Postoperative Complications

Rathke's cleft cyst (RCC) is a common incidental tumor in the hypothalamic-pituitary region. Some reports have shown that the clinical symptoms and endocrine functions of symptomatic RCCs are temporarily improved by glucocorticoid administration. However, it is still unknown whether glucocorticoid treatment is effective for symptomatic RCCs according to long-term observations. In this study, we describe the long-term clinical outcomes of two cases of glucocorticoid-treated biopsy-proven secondary hypophysitis caused by RCCs. We summarize the symptoms, imaging findings, and endocrine evaluations of two symptomatic RCC patients with concomitant hypophysitis before and after prednisolone treatment. In both evaluated cases, visual impairments and altered endocrine parameters were present due to chiasm and stalk compression; these outcomes improved after shrinkage of RCCs in response to prednisolone administration, and partial recovery of anterior pituitary hormone secretion was observed. However, in both cases, the deficits in anterior pituitary hormone secretion recurred, possibly due to persistent inflammatory infiltration in the RCCs and pituitary glands. After relapse of hypophysitis, anterior hormone secretion did not fully recover. In our cases of secondary hypophysitis caused by RCCs, prednisolone administration had an early effect of cyst shrinkage, followed by partial improvements in clinical symptoms and pituitary functions. However, long-term observation showed that prednisolone treatment did not contribute to complete improvement in anterior pituitary hormone dysfunction.

Topics: Antidiuretic Agents; Central Nervous System Cysts; Deamino Arginine Vasopressin; Female; Glucocorticoids; Hormone Replacement Therapy; Humans; Hydrocortisone; Hypophysitis; Hypopituitarism; Middle Aged; Pituitary Neoplasms; Prednisolone

We report a case of subclinical central diabetes insipidus (DI), due to Rathke's cleft cysts, that was initially misdiagnosed as transient DI of pregnancy because it presented in the third trimester of pregnancy. A 37-year-old primigravida visited the Department of Obstetrics in the 30th week of gestation due to polyuria. She was admitted due to oligohydramnios; the amniotic fluid index was 3.24. A vasopressin challenge test was performed and her urine osmolality increased by >100% from baseline after the administration of desmopressin. Because central DI or transient DI of pregnancy was suspected, we prescribed her a desmopressin nasal spray. She gave birth to a relatively healthy baby at 37 weeks and 4 days of gestation. Several months after delivery, discontinuation of desmopressin resulted in recurrence of her polyuria. Magnetic resonance imaging of her brain revealed Rathke's cleft cysts, and finally central DI was diagnosed.

Topics: Administration, Intranasal; Brain; Central Nervous System Cysts; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Diagnostic Errors; Female; Humans; Infant, Newborn; Magnetic Resonance Imaging; Nasal Sprays; Oligohydramnios; Polyuria; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Pregnancy Trimester, Third; Recurrence

To clarify the outcome of all cases of Rathke's cleft cysts (RCC) treated surgically and followed up in Oxford during a long-term period.. The records of all patients with RCC seen in the Department of Endocrinology between January 1978 and June 2009 were reviewed.. A total of 33 patients (20 females, median age 43 years) were identified. At presentation, major visual field defects were detected in 58% of patients and gonadotrophin, ACTH and TSH deficiency in 60, 36 and 36% of patients respectively. Desmopressin treatment was required in 18% of patients. Treatment consisted of cyst evacuation combined with or without biopsy/removal of the wall. Post-operatively, visual fields improved in 83% of patients with impairment, whereas there was no reversal of ACTH or TSH deficiency or of diabetes insipidus. All but one subject had imaging follow-up during a mean period of 48 months (range 2-267). Cyst relapse was detected in 22% of patients at a mean interval of 29 months (range 3-48 months); in 57% of them, the recurrence was symptomatic. Relapse-free rates were 88% at 24-months and 52% at 48-months follow-up. At last assessment, at least quadrantanopia was reported in 19% of patients, gonadotrophin, ACTH and TSH deficiency in 50, 42 and 47% of patients respectively. Desmopressin treatment was required in 39% of patients.. In this study of patients with RCC and long-term follow-up, we showed a considerable relapse rate necessitating long-term monitoring. Surgical intervention is of major importance for the restoration of visual field defects, but it does not improve endocrine morbidity, which in the long-term affects a substantial number of patients.

Topics: Adenoma; Adolescent; Adrenocorticotropic Hormone; Adult; Aged; Aged, 80 and over; Central Nervous System Cysts; Child; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Follicle Stimulating Hormone; Humans; Luteinizing Hormone; Male; Middle Aged; Neoplasm Recurrence, Local; Pituitary Neoplasms; Thyrotropin; Treatment Outcome; Visual Fields

Although intrinsic pituitary lesions are the most common cause of hypopituitarism, suprasellar masses can produce similar symptoms. The differential diagnosis of a suprasellar mass includes cystic lesions, tumors, granulomatous disease, and infection. The etiology is not always obvious, and despite extensive work-up, may remain elusive. A 28-year-old Mexican man presented with complaints of headache and weakness for two weeks duration. He became increasingly lethargic and an MRI revealed a two centimeter suprasellar mass. Testing of the hypothalamic-pituitary axis suggested panhypopituitarism. He was prescribed treatment with hydrocortisone, DDAVP, and levothyroxine. Open craniotomy and biopsy of the hypothalamus revealed marked inflammation with plasma cells, histiocytes, and small lymphocytes. Light microscopy revealed macrophage-contained leishmania-like organisms although results were not immediately available. Pathological data was consistent with acute infection by Trypanasoma cruzi. Despite supportive efforts, the patient expired two months after presentation. This case illustrates the difficulty of diagnosing and the potential rapid mortality of a suprasellar mass. Because of the wide consideration of etiologies, a tissue diagnosis is needed. However, as this case illustrates, a definitive tissue diagnosis is not always possible, even following biopsy during open craniotomy.

Topics: Adult; Animals; Central Nervous System Cysts; Chagas Disease; Deamino Arginine Vasopressin; Humans; Hydrocortisone; Hypopituitarism; Male; Thyroxine; Trypanosoma cruzi