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Symptomatic Rathke's cleft cyst coexisting with central diabetes insipidus and hypophysitis: case report.

We describe a 48-year-old female with acute onset of central diabetes insipidus followed by mild anterior pituitary dysfunction. Magnetic resonance imaging (MRI) revealed enlargement of the hypophysis-infundibulum accompanied by a cystic component. She underwent a transsphenoidal exploration of the sella turcica. Histological examination showed foreign body type xanthogranulomatous inflammation in the neurohypophysis which might have been caused by rupture of a Rathke's cleft cyst. The MRI abnormalities and anterior pituitary dysfunction improved after a short course of corticosteroid administration, but the diabetes insipidus persisted. The histological findings in this case indicated the site of RCC rupture and the direction of the progression of RCC induced neurohypophysitis and adenohypophysitis.

Cysts↗

Central diabetes insipidus following intracranial hemorrhage due to vitamin K deficiency in a neonate.

A previously healthy 30-day-old girl presented with seizures, irritability and inability to sleep for three days. Vitamin K was not given just after birth. She was lethargic. A multifocal clonic seizure was evident during examination. Anisocoria was diagnosed on eye examination. Brain magnetic resonance imaging showed intracerebral hemorrhage, ventricular dilatation, and hematoma in the left temporofrontal region extending to the hypothalamus. Central diabetes insipidus was diagnosed by water deprivation due to dehydration and hypernatremia, and then desmopressin was added to phenobarbital. The possible mechanism of central diabetes insipidus in our patient is damage of vasopressin pathway resulting from compression of hemorrhage. An operation of ventriculoperitoneal shunt was also performed due to hydrocephalus. While she was symptom-free except for neurological sequel during routine control examinations after discharging from hospital, the parents said that she died, most probably from bronchopneumonia, at the age of 7.5 months. In conclusion, we emphasize that prophylactic vitamin K should be administrated to all babies just after birth, and infants with intracranial hemorrhage should carefully be monitored for central diabetes insipidus.

Anisocoria↗

Intracranial aggressive fibromatosis presenting as panhypopituitarism and diabetes insipidus.

Aggressive fibromatosis (AF) is a rare, locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often, of the limbs and trunk. Intracranial AF is extremely rare and requires aggressive treatment to prevent recurrence. We present a case of a 34 year-old male with AF involving intracranial structures causing panhypopituitarism and diabetes insipidus. Patient was admitted to hospital because of polyuria, polydipsia, and loss of libido, impotence, hearing loss, and gait disturbance. On cranial magnetic resonance imaging, the lesion extended through the sphenoid sinus into the both pterygoid recesses, destroying the left lateral wall of the sphenoid sinus and invading the retroorbital area. There was also a distinct lesion in the hypothalamic area. The tumor was markedly isointense on both T2- and T1-weighted images relative to gray matter, and enhanced strongly after administration of gadolinium. The patient underwent partial resection of the lesion via a transcranial approach. The pathological examination of the mass was reported as AF. No other sites were found to be involved by thorax and abdominal tomography. Hormonal assessment of hypothalamic-pituitary dysfunction revealed panhypopituitarism with central diabetes insipidus. Replacement therapy was instituted. In this case, standard treatment of wide-field surgical resection was impossible. On the basis of reports that radiotherapy is an effective treatment for this kind of tumor, we administered radiation to the affected area, since chemotherapy and hormonal treatment of non-resectable tumors are not satisfactory. To our knowledge, this is the first reported case of AF presenting as panhypopituitarism with central diabetes insipidus.

Adult↗

Anhidrosis: an unusual presentation of diabetes insipidus.

A 61-year-old man who had a 10-year history of anhidrosis was found to have idiopathic diabetes insipidus. He showed no spontaneous sweating or pilocarpine-induced sweat response. Skin pathology showed a normal eccrine gland. Microneurography detected no skin sympathetic nerve activity. Within a month of desmopressin treatment for diabetes insipidus, sweating and skin sympathetic nerve activity returned.

Deamino Arginine Vasopressin↗

Two cases of lymphocytic hypophysitis presenting with diabetes insipidus: a variant of lymphocytic infundibulo-neurohypophysitis.

BACKGROUND: It has been thought that neurohypophysial involvement manifesting as central diabetes insipidus in lymphocytic hypophysitis is uncommon. Although cases with such an association have been reported recently with increasing frequency, the relationship with lymphocytic infundibular neurohypophysitis remains unclear. METHODS AND RESULTS: Two postmenopausal women who presented with diabetes insipidus are reported. Adenohypophysial function was normal in both cases, apart from growth hormone secretion. Magnetic resonance imaging revealed a sellar mass lesion mimicking pituitary adenoma, with loss of the hyperintense signal of the neurohypophysis in both cases and thickening of the stalk in one case. The lesion was located behind the residual adenohypophysis during transsphenoidal biopsy; however, histologic examination showed chronic inflammation of the adenohypophysis, findings identical to those of lymphocytic adenohypophysitis. Although the principal site of the inflammation was considered to be the neurohypophysial system, adenohypophysis was also involved in the lesion. We suggest that these cases represent a variant of lymphocytic infundibular neurohypophysitis. CONCLUSION: To avoid unnecessary surgical intervention, it is important to note that some variants of lymphocytic infundibulo-neurohypophysitis may form a mass lesion not localized in the neurohypophysial system but involving the adenohypophysis, thus resembling adenomas and other tumors.

Diabetes Insipidus↗

Primary cerebral lymphoma presenting with cranial diabetes insipidus.

A 30 year old woman with an 8-year history of thirst and polyuria was found to have cranial diabetes insipidus. There were no neurological abnormalities at presentation but she subsequently developed diverse signs and died 26 months later. Autopsy revealed a diagnosis of diffuse primary cerebral lymphoma. Cranial diabetes insipidus with otherwise minimal abnormality of hypothalamic/pituitary function has not previously been reported as a presentation of this neoplasm.

Adult↗

Isolated central diabetes insipidus in a newborn with congenital toxoplasmosis.

We present a 5 day-old male newborn with isolated central diabetes insipidus due to congenital toxoplasmosis. This patient was referred to us for hydrocephalus. As we investigated the aetiology of the hydrocephalus, the patient's serum and cerebrospinal fluid tested positive for toxoplasmosis via ELISA and polymerase chain reaction. Computed tomography showed obstructive hydrocephalus and disseminated cranial calcifications. Central diabetes insipidus developed on the 10th day, apparently as a result of the toxoplasmosis infection, and was treated successfully with oral desmopressin.

Administration, Oral↗

[Pregnancy in diabetes insipidus--a case report with review of the literature].

A 32 year old I P II G with preexisting diabetes insipidus was treated with 1-(3-mercaptopropionic acid)-8-d-arginine vasopressin (DDAVP) during pregnancy. An otherwise normal pregnancy was marked only with an excessive weight increase. A healthy girl was delivered by secondary cesarean section at term. Postoperative the mother developed a water intoxication accompanying oxytocin-infusion. During nursing the diabetes insipidus improved significantly whereby DDAVP doses could be reduced to 20-10 percent. We suppose an overreaction to endogene oxytocin with an antidiuretic effect.

Administration, Intranasal↗

Serum Neurophysins in familial central diabetes insipidus.

Antibovine neurophysin antibodies (anti-bNpI and/or anti-bNpII) are present in certain patients with familial central diabetes insipidus; these are exogenous origin, as they are not present in patients who have not received treatment with crude posterior pituitary extracts over the years preceding the analysis. Immunoreactive neurophysins were detectable in the blood of five patients with familial central diabetes insipidus, and in two of them, the levels increased after a short period of water restriction. There is marked polymorphism of these neurophysins from one serum to another: neurophysin I was consistently absent, while neurophysin II, accessory neurophysins, and other immunoreactive substances not present in normal sera were sometimes present in variable amounts. Immunoreactive AVP was undetectable in the urine of all patients, while immunoreactive OT was found in three of them; the latter substance could, however, be arginine vasotocin. Data are presented suggesting that the association between the biosynthesis of neurophysin I and AVP on the one hand, and neurophysin II and OT on the other hand is maintained in patients with isolated AVP deficiency on the basis of a congenital defect.

Adolescent↗

[Diabetes insipidus after endoscopic third ventriculostomy. A case report and review of the literature].

Endoscopic third ventriculostomy has been found to be successful for treating occlusive hydrocephalus. The complication rate ranges from 6 to 12%. Intraoperative bleeding is the most common incident. Endocrinological failures are rare, mainly due to the proximity of the hypothalamic structures. We report the case of a 33-year-old man who was referred in emergency for subacute hydrocephalus related to a tentorium meningioma. The hydrocephalus was treated by endoscopic third ventriculostomy. During the procedure, the floor of the third ventricle was found to be thick but fenestration was performed without incident. After surgery, the clinical signs of hydrocephalus disappeared but diabetes insipidus was diagnosed the same day. There were no other endocrinology disorders. Medical treatment with vasopressin allowed resolution of the diabetes insipidus in fifteen days. Surgical debulking of the meningioma was then achieved via a subtemporal approach. There was no recurrence of the endocrinology disorder. Diabetes insipidus is an unpredictable complication of third ventriculostomy. The mechanism is not well known. It is however a transient disorder that can easily be treated with vasopresin and therefore should not modify the indications of third ventriculostomy, especially in tumor-related hydrocephalus.

Adult↗

Immune complexes in diabetes insipidus syndrome of rabbits immunized with vasopressin.

Rabbits immunized with lysine-vasopressin bovine serum albumin conjugate showed the diabetes insipidus syndrome intermittently manifested by polyuria and polydipsia with various degrees of peaking from the eighth to fourteenth day post injection during boosters. The antibody-antidiuretic hormone immune complexes which may interfere with the action of the endogenous vasopressin on the kidney were found in the diabetes insipidus rabbits. However, the degree of the polyuria was not necessarily related to the quantities of the formed immune complexes, the titer, nor the affinity of the antiserum. It is suggested that the degree of the polyuria is related not only to the binding ability of the antiserum to the endogeneous vasopressin, but also to other factors.

Animals↗

[Diabetes insipidus and pregnancy (author's transl)].

Reported in this paper are peculiarities relating to pregnancy and labour of eight patients with diabetes insipidus. The disease took different courses. Pregnancy in such cases was found to be endangered quite often by abortion and premature birth. Adiurekrin was used in the treatment of diabetes insipidus. Developmental disorders were not recorded from the infants born by the above women.

Abortion, Spontaneous↗