Diffuse malignant lymphoma type B with optic chiasm infiltration, visual disturbances, hypopituitarism, hyperprolactinaemia and diabetes insipidus. Case report and literature review

The case is reported of a 55-year-old man with diffuse malignant lymphoma type B associated with transient optic chiasm infiltration and visual disturbances but with persistent hypopituitarism, hyperprolactinaemia and diabetes insipidus. The patient was administered chemotherapy and radiotherapy. Repeated MR and CT scans showed optic chiasm infiltration, which disappeared in the course of the chemotherapy but then recurred, changed its appearance and finally disappeared again. In the meantime visual disturbances occurred and disappeared during the therapy. Hypopituitarism, diabetes insipidus and hyperprolactinaemia were diagnosed and replacement therapy was administered. Later on abdominal pain occurred, and a CT scan revealed bilateral kidney masses and enlarged retroperitoneal lymph nodes. These were diffuse malignant lymphoma with regional lymphonodulitis in histology. Finally, hydrothorax and hydroretroperitoneum were diagnosed. The patient died as a result of systemic complications of the disease. The length of survival time documented following the hypothalamochiasmatic infiltration and diagnosis of lymphoma makes the case an unusual one for patients with CNS lymphoma. Hormonal disturbances accompanying the suprasellar region infiltration are very important from the practical point of view.     

Xanthogranuloma as an Unsuspected Cause of Idiopathic Central Diabetes Insipidus

ObjectiveTo demonstrate that xanthogranuloma is a rare cause of idiopathic central diabetes insipidus in the early phase of the disease and that it presents as a suprasellar mass at a later stage. In addition, we emphasize the importance of identifying the cause of idiopathic central diabetes insipidus and review the literature concerning endocrine disturbance in central xanthogranuloma.MethodsReview of recently published case reports of central xanthogranuloma with endocrine disorders. The case of a 35-year-old man who presented with a very large suprasellar mass is also reported. The patient was diagnosed with idiopathic central diabetes insipidus 20 years ago with normal brain magnetic resonance imaging.ResultMost cases of this disease present as supra-or parasellar masses with endocrine involvement, the most common of which (in approximately 75% of patients) is sex hormone deficiency. Diabetes insipidus was found in 65% of patients.ConclusionXanthogranuloma should be in the differential diagnosis of idiopathic central diabetes insipidus and sellar and parasellar masses. A detailed skin examination is very important in making the diagnosis of central diabetes insipidus. (Endocr Pract. 2014;20:e42-e46)     

A case of pituitary stalk tumor diagnosed with magnetic resonance (MRI)]

Authors present a case of 28-year old female with anterior hypopituitarism and diabetes insipidus, with properly functioning anterior pituitary cells as showed by means of measuring pituitary hormones in response to neurohormones i.v. injections. Magnetic resonance imaging revealed neoplastic tissue in the pituitary stalk destroying supraopticohypophysial and paraventriculohypophysial tracts, as well as portal blood system, thus preventing release of vasopressin and these hypothalamic neurohormones from accessing anterior pituitary.     

Superiority of magnetic resonance imaging (MRI) over computerized tomography (CT) in diagnosis of hypopituitarism]

Hypopituitarism can be a result of various lesions of hypothalamus, pituitary stalk, or of the pituitary gland itself. The aim of the study was to assess the value of CT and MRI examinations in determination of the cause of hypopituitarism. Seventeen patients with hypopituitarism (9 women and 8 men) aged 22 to 61 years have been examined. In three cases growth deficiency was observed, 4 women had galactorrhoea, 4 patients had diabetes insipidus, 16 patients had supra-adrenal insufficiency, 14 had signs of hypogonadism and 10 hypothyroidism. In each case plasma concentrations of LH, FSH, PRL, TSH, alpha-subunit, ACTH before and after appropriate stimulation with TRH, metoclopramid, LH-RH, GRF or metyrapon were determined with RIA. Every patient was examined both with CT and MRI (0.5 T Toshiba MRT 50a). All 17 patients had abnormal MR images of hypothalamo-pituitary area, while only 10 of them had abnormalities in their CT scans. In remaining 7 patients the MRI revealed: three cases of congenital malformation of hypophyseal stalk, two cases of empty sella, one posttraumatic lesion of the stalk and one case of granulomatous infiltration of the stalk. We found MRI superior to CT in establishing the case of hypopituitarism.     

Xanthoma Disseminatum: Case Report and Literature Review

ObjectiveTo report a case of a young normolipidemic woman with mucocutaneous xanthomas who developed neurogenic diabetes insipidus and hyperprolactinemia because of an inflammatory pituitary stalk lesion.MethodsThe clinical features, laboratory results, magnetic resonance imaging, and pathology findings are presented. In addition, the pertinent literature is reviewed.ResultsA 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas. Laboratory tests showed hyperprolactinemia, normal electrolytes, and a normal lipid profile. The results of a water deprivation test were compatible with neurogenic diabetes insipidus, and cerebral magnetic resonance imaging showed pituitary stalk thickening. Histologic findings on a skin biopsy specimen supported the diagnosis of non-Langerhans histiocytosis. Treatment was initiated with cabergoline, nasally administered desmopressin, radio-frequency ablation of facial skin lesions, and surgical excision of other accessible lesions.ConclusionXanthoma disseminatum is a rare, benign proliferative disorder characterized by extensive cutaneous and mucous membrane xanthomas in normolipidemic patients. Central nervous system involvement is rare and usually occurs in the systemic variety. Pituitary stalk disease commonly causes hyperprolactinemia, diabetes insipidus, and various degrees of hypopituitarism. The natural history of xanthoma disseminatum usually is benign, but lesions in critical anatomic sites may result in morbidity and mortality. (Endocr Pract. 2010;16:1003-1006)     

Hypothalamo-pituitary dysfunction in patients with chronic subdural hematoma

Relatively frequent pituitary hormone deficiencies are observed after traumatic brain injury (TBI) and subarachnoid hemorrhage (SAH) and according to the published studies the neuroendocrine consequenses of traumatic brain injury are underdiagnosed. In a cohort of 59 patients (49 males, mean age 68.3 years, 36-88 years) after evacuation of subdural hematoma (SDH) were evaluated hypothalamo-pituitary functions one week after surgery, after three months and after one year. Hypogonadism was present in 26 % of patients in an acute phase, but in the majority had a transient character. Less than half of patients was GH deficient (GHD) according to the GHRH+arginine test. We did not find any serious case of hypocortisolism, hypothyroidism, diabetes insipidus centralis nor syndrome of inappropriate secretion of ADH (SIADH). Transient partial hypocortisolism was present in two cases, but resolved. We did not find relation between extension of SDH or clinical severity and development of hypopituitarism. In conclusion, in some patients with SDH growth hormone deficiency or hypogonadism was present. No serious hypocortisolism, hypothyroidism, diabetes insipidus nor SIADH was observed. The possibility of neuroendocrine dysfunction should be considered in patients with SDH, although the deficits are less frequent than in patients after TBI or SAH.     

The Clinical Physiology of Water Metabolism: Part II: Renal Mechanisms for Urinary Concentration; Diabetes Insipidus

The renal reabsorption of water independent of solute is the result of the coordinated function of the collecting duct and the ascending limb of the loop of Henle. The unique juxtaposition of the ascending and descending portions of the loop of Henle and of the vasa recta permits the function of a counter-current multiplier system in which water is removed from the tubular lumen and reabsorbed into the circulation. The driving force for reabsorption is the osmotic gradient in the renal medulla which is dependent, in part, on chloride (followed by sodium) pumping from the thick ascending loop of Henle. Urea trapping is also thought to play an important role in the generation of a hypertonic medullary interstitium. Arginine vasopressin (AVP) acts by binding to receptors on the cell membrane and activating adenylate cyclase. This, inturn, results in the intracellular accumulation of cyclic adenosine monophosphate (AMP) which in some fashion abruptly increases the water permeability of the luminal membrane of cells in the collecting duct. As a consequence, water flows along an osmotic gradient out of the tubular lumen into the medullary interstitium.Diabetes insipidus is the clinical condition associated with either a deficiency of or a resistance to AVP. Central diabetes insipidus is due to diminished release of AVP following damage to either the neurosecretory nuclei or the pituitary stalk. Possible causes include idiopathic, familial, trauma, tumor, infection or vascular lesions. Patients present with polyuria, usually beginning over a period of a few days. The diagnosis is made by showing that urinary concentration is impaired after water restriction but that there is a good response to exogenous vasopressin therapy. Nephrogenic diabetes insipidus can be identified by a patient''s lack of response to AVP. Nephrogenic diabetes insipidus is caused by a familial defect, although milder forms can be acquired as a result of various forms of renal disease. Central diabetes insipidus is eminently responsive to replacement therapy, particularly with dDAVP, a long lasting analogue of AVP. Nephrogenic diabetes insipidus is best treated with a combination of thiazide diuretics as well as a diet low in sodium and protein.     

Panhypopituitarism associated with diabetes insipidus in a girl with a suprasellar arachnoid cyst

We report on a female patient with a large suprasellar arachnoid cyst (3.5 x 2.5 cm) combined with right optic nerve hypoplasia. She developed growth hormone deficiency and hypothyroidism at the age of 8.5 years, adrenal insufficiency at the age of 11 years, diabetes insipidus and hypogonadotropic hypogonadism at the age of 15 years. When last seen at the age of 19 years she was extremely obese (+5.9 BMI SDS). The endocrine picture suggests that arachnoid cysts might be involved in far more complex hypothalamic-pituitary disturbances than previously thought.     

Primary Pituitary Lymphoma As Rare Cause Of A Pituitary Mass And Hypopituitarism In Adulthood

Objective: Differential diagnosis of nonadenomatous sellar masses causing hypopituitarism is still a challenge. Among these masses, growing evidence has demonstrated that primary pituitary lymphoma is a specific and emerging entity. The aim of our study was to describe our experience with a case of primary pituitary lymphoma and to perform a review of the available literature.Methods: We searched relevant databases up to March 2020, identifying 36 suitable articles basing on inclusion criteria (primary pituitary lymphoma in adult immunocompetent subjects). Overall, 43 cases were included in the review, adding a new case diagnosed and treated in our hospital. Epidemiologic data, clinical presentation, hormonal status, radiologic findings, pathology, treatment, and outcome were extracted.Results: Mean age at diagnosis was 58.9 years, without gender difference. Symptoms related to mass were common (52.3%), in particular cranial nerve palsy (70.5%), headache (56.8%), and alteration in visual field (40.9%). Impaired hormonal status was detected in 89.7% of patients; of them, 58.9% presented with anterior pituitary failure (partial or total), while 25.6% presented with panhypopituitarism. Overall, diabetes insipidus was present in 30.8% and hyperprolactinemia in 41.0% of patients. The majority of patients presented a radiologically invasive mass in the suprasellar region and cavernous sinus (65.9% and 40.9%, respectively) and histologic diagnosis of diffuse B-cell lymphoma (54.5%).Conclusion: The differential diagnosis of sellar and parasellar masses causing hypopituitarism should include primary pituitary lymphoma, even in absence of systemic symptoms or posterior pituitary dysfunction. The disease has a heterogeneous pattern, so a collaboration between endocrinologists, neuroradiologists, neurosurgeons, and hematologists is desirable.     

The Emerging Role of Copeptin

Direct measurement of the nonapeptide vasopressin has been limited by analyte instability ex vivo and in vivo rapid degradation, low serum concentrations requiring a sensitive assay and inherent secretory pulsatility. Copeptin is a 39 amino acid glycopeptide cleavage product of vasopressin synthesis with high stability, providing a marker of vasopressin secretion. Copeptin measurement has applications in diagnosis of diabetes insipidus and other diseases with altered vasopressin secretion. This review summarises our current understanding of serum copeptin measurement in diabetes insipidus and possible future applications of copeptin assays. As vasopressin is a stress hormone, there is emerging evidence on the use of copeptin for diagnosis and prognostication of disorders such as syndrome of inappropriate anti-diuretic hormone secretion, diabetes mellitus, critical illness, stroke, cardiovascular disease, respiratory disease, renal disease and thermal stress. Copeptin concentration measurement is likely to improve the diagnostic reliability of diabetes insipidus and, as a marker of stress, may have diagnostic or prognostic utility in specific clinical circumstances. Further studies are needed to determine if goal-directed therapy using plasma copeptin concentrations may improve patient outcomes.     

Diabetes Insipidus Associated with Hehmophagocytic Lymphohistiocytosis: First Case Report

ObjectiveTo report the first case of central diabetes insipidus associated with hemophagocytic lymphohistiocytosis.MethodsWe describe the clinical course of a 75-yearold woman who developed febrile illness 1 month after receiving the H1N1 influenza vaccination. Shortly thereafter, she developed central diabetes insipidus, which responded to treatment with intranasal desmopressin acetate. She was then hospitalized with another febrile illness and diagnosed with hemophagocytic lymphohistiocytosis.ResultsMagnetic resonance imaging of the pituitary gland was significant for plaquelike meningeal enhancement and thickening surrounding the infundibulum, which has been previously reported as a manifestation of histiocytosis.ConclusionWe believe this is the first case of central diabetes insipidus associated with hemophagocytic lymphohistiocytosis. (Endocr Pract. 2011;17:e118-e122)     

Pituitary Stalk Thickening in a Large Cohort: Toward More Accurate Predictors of Pituitary Dysfunction and Etiology

Objective: To summarize the characteristics of patients with pituitary stalk thickening, analyze the association between pituitary stalk width and hypopituitarism, and develop a diagnostic model to differentiate neoplastic and inflammatory origins.Methods: A total of 325 patients with pituitary stalk thickening in a tertiary teaching hospital between January 2012 and February 2018 were enrolled. Basic characteristics and hormonal status were evaluated. Indicators to predict etiology in patients with histologic diagnoses were analyzed.Results: Of the 325 patients, 62.5% were female. Deficiency in gonadotropin was most common, followed by corticotropin, growth hormone, and thyrotropin. The increase in pituitary stalk width was associated with a risk of central diabetes insipidus (odds ratio &lsqb;OR], 3.57; P<.001) and with a combination of central diabetes insipidus and anterior pituitary deficiency (OR, 2.28; P = .029). The cut-off pituitary stalk width of 4.75 mm had a sensitivity of 69.2% and a specificity of 71.4% for the presence of central diabetes insipidus together with anterior pituitary deficiency. Six indicators (central diabetes insipidus, pattern of pituitary stalk thickening, pituitary stalk width, neutrophilic granulocyte percentage, serum sodium level, and gender) were used to develop a model having an accuracy of 95.7% to differentiate neoplastic from inflammatory causes.Conclusion: Pituitary stalk width could indicate the presence of anterior pituitary dysfunction, especially in central diabetes insipidus patients. With the use of a diagnostic model, the neoplastic and inflammatory causes of pituitary stalk thickening could be preliminarily differentiated.Abbreviations: APD = anterior pituitary dysfunction; AUC = area under the curve; CDI = central diabetes insipidus; GH = growth hormone; MRI = magnetic resonance imaging; OR = odd ratio; PHBS = posterior hypophyseal bright spots; PST = pituitary stalk thickening; PSW = pituitary stalk width     

Diabetes Insipidus in Pregnancy: Etiology,Evaluation, and Management

ObjectiveTo review the approach to a patient with diabetes insipidus during pregnancy.MethodsThis review examines the normal physiology of water homeostasis, the related changes that occur during pregnancy, and the pathophysiology of diabetes insipidus in pregnancy. Associated complications, evaluation, and management are discussed.ResultsDiabetes insipidus can complicate up to 1 in 30000 pregnancies. Diabetes insipidus during pregnancy has a variety of causes, some that predate the pregnancy and others that begin during gestation. Polyuria and polydipsia can occur or be exacerbated in women with overt or subclinical central or nephrogenic diabetes insipidus. These women have either decreased central secretory reserve or impaired renal responsiveness to vasopressin. In addition, women can experience diabetes insipidus de novo in pregnancy through the actions of placental vasopressinase, which causes accelerated degradation of vasopressin. This form of diabetes insipidus may be associated with increased complications of pregnancy, including preeclampsia. Management of central diabetes insipidus and transient diabetes insipidus of pregnancy can be achieved with 1-deamino-8-D-arginine vasopressin (desmopressin acetate) (DDAVP), a vasopressin analogue. Nephrogenic diabetes insipidus is typically resistant to both DDAVP and vasopressin and underlying causes should be addressed.ConclusionsIncreased awareness of diabetes insipidus in pregnancy may lead to early diagnosis and appropriate treatment that will reduce the risks of maternal and fetal morbidity. Overall, growing experience with DDAVP has shown that it is a safe and effective treatment for diabetes insipidus caused by a variety of factors. (Endocr Pract. 2009;15:377-382)     

Important Management Considerations In Patients With Pituitary Disorders During The Time Of The Covid-19 Pandemic

Objective: In December 2019, a novel coronavirus called severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) caused an outbreak of coronavirus disease 2019 (COVID-19) that resulted in a global pandemic with substantial morbidity and mortality. Currently, there is no specific treatment or approved vaccine against COVID-19. The underlying associated comorbidity and diminished immune function of some pituitary patients (whether caused by the disease and its sequelae or treatment with excess glucocorticoids) increases their risk of contracting and developing complications from COVID-19 infection.Methods: A review of studies in PubMed and Google Scholar published between January 2020 to the time of writing (May 1, 2020) was conducted using the search terms ‘pituitary,’ ‘coronavirus,’ ‘COVID-19’, ‘2019-nCoV’, ‘diabetes mellitus’, ‘obesity’, ‘adrenal,’ and ‘endocrine.’Results: Older age and pre-existing obesity, hypertension, cardiovascular disease, and diabetes mellitus increase the risk of hospitalization and death in COVID-19 patients. Men tend to be more severely affected than women; fortunately, most men, particularly of younger age, survive the infection. In addition to general comorbidities that may apply to many pituitary patients, they are also susceptible due to the following pituitary disorder–specific features: hypercortisolemia and adrenal suppression with Cushing disease, adrenal insufficiency and diabetes insipidus with hypopituitarism, and sleep-apnea syndrome and chest wall deformity with acromegaly.Conclusion: This review aims to focus on the impact of COVID-19 in patients with pituitary disorders. As most countries are implementing mobility restrictions, we also discuss how this pandemic has affected patient attitudes and impacted our decision-making on management recommendations for these patients.Abbreviations: ACE = angiotensin-converting enzyme; AI = adrenal insufficiency; ARB = angiotensin receptor blocker; ARDS = acute respiratory disease syndrome; COVID-19 = coronavirus disease 2019; CPAP = continuous positive airway pressure; DI = diabetes insipidus; DM = diabetes mellitus; SARS-CoV-2 = severe acute respiratory syndrome coronavirus 2     

Pulmonary Atypical Carcinoid Tumor with Metastatic Involvement of the Pituitary Gland Causing Functional Hypopituitarism

ObjectiveTo report a unique case of a peripherally located pulmonary atypical carcinoid tumor with metastatic involvement of the pituitary, manifesting with evidence of functional hypopituitarism and compressive symptoms of dysarthria, bitemporal loss of vision, and ataxia.MethodsWe present a case report, including detailed laboratory, radiologic, and pathologic findings in a 50-year-old woman with a peripherally located pulmonary atypical carcinoid tumor and a lesion metastatic to the pituitary gland. The pertinent literature is also reviewed.ResultsA 50-year-old woman with a medical history of metastatic pulmonary atypical carcinoid tumor presented with symptoms of bitemporal hemianopia, ataxia, and dysarthria. Laboratory evaluation revealed functional hypopituitarism, and magnetic resonance imaging of the brain with use of gadolinium contrast demonstrated a pituitary lesion measuring 3.5 by 2.5 by 2.5 cm. Visual field testing revealed bitemporal superior quadrantanopia, consistent with a pituitary lesion of the chiasm. Computed tomographic angiography excluded the presence of an aneurysm of the internal carotid artery. Subsequently, the patient underwent subtotal transsphenoidal hypophysectomy. Findings on pathology examination were consistent with a lesion metastatic from the primary pulmonary atypical carcinoid tumor. The patient subsequently received monthly octreotide injections as well as chemotherapeutic regimens of capecitobine and temozolomide. At 18-month follow-up, disease had not recurred.ConclusionThis case of metastatic involvement of the pituitary gland from a peripherally located pulmonary atypical carcinoid tumor manifesting with evidence of functional hypopituitarism is highly uncommon. To our knowledge, such a case has not been previously reported. The presentation of an advanced pituitary metastatic lesion can be particularly dramatic, and the course can be potentially complicated. Therefore, heightened awareness of the possibility of a pituitary lesion metastatic from a pulmonary atypical carcinoid tumor is important. (Endocr Pract. 2007;13:291-295)     

ADHD symptoms in a young patient with central diabetes insipidus

Diabetes insipidus is known to be associated with neurodevelopmental disorders. In this case report, we present a child suffering from a central diabetes insipidus (DI) and an attention-deficit/hyperactivity disorder (ADHD). The DI was due to a mutation on the vasopressin gene, impairing its secretion. We discuss the effects of this impairment on the central nervous system and how it might be linked to ADHD symptoms.     

La potomanie: réévaluation des epreuves d'investigation et présentation inhabituelle avec hydronéphrose et méqavessie

Three cases of compulsive polydipsia previously diagnosed as diabetes insipidus are presented. Abnormally dilated bladder and pyelocalyceal systems were accompanying features, as previously described for diabetes insipidus, particularly of renal orign. Results of the hypertonic saline (Hickey-Hare) test were positive in only one case. Results of restriction of liquids followed by intravenous injection of vasopressin (Miller test) favoured a diagnosis of complete diabetes insipidus. These two tests cannot, therefore, exclude compulsive polydipsia. The features suggesting a diagnosis of compulsive water drinking are low plasma osmolality, a decrease in 24-hour urine output following water restriction, and abnormal behaviour. The diagnosis is confirmed by an 18-hour dehydration test done after gradual fluid restriction, which favours partial restoration of the papillary osmotic gradient.     

Diabetes Insipidus,Panhypopituitarism, and Severe Mental Status Deterioration in a Patient with Chordoid Glioma: Case Report and Literature Review

ObjectiveTo describe a rare progressive case of chordoid glioma clinically masquerading as idiopathic diabetes insipidus (DI).MethodsWe describe the clinical, radiographic, and laboratory findings of the study patient and briefly review the relevant literature.ResultsA 41-year-old woman was referred to our center for evaluation of worsening mental status changes, a newly diagnosed suprasellar mass, and possible endocrine dysfunction. Three years earlier, a physician at another institution diagnosed idiopathic DI and prescribed desmopressin. At that time, laboratory workup and magnetic resonance imaging (MRI) revealed no brain lesions or other hormonal irregularities. Slow, progressive symptomatology in the following 3 years included mental status changes, nonhealing skin lesions, recurrent infections, temperature dysregulation, and midsection weight gain. She became withdrawn and emotionally labile and developed a flat affect, short-term memory loss, poor concentration, and sleep disturbance. MRI revealed a 2.2 × 2.1 × 1.9-cm suprasellar region lesion. Biopsy samples from the third ventricular lesion revealed a circumscribed glial tumor. Chordoid glioma is a rare tumor, and the 50 previously reported cases have been located in the suprasellar region. This is the third reported case of a chordoid glioma positive for neurofilament protein, which brings into question the hypothesis of a single phenotype for glial tumors. Tumors in this region frequently result in endocrine dysfunction that prompts patients to seek medical attention.ConclusionsThere is no formally recognized treatment protocol for chordoid glioma, and postoperative mortality and morbidity is high. Our report emphasizes the necessity of close follow-up of patients after a diagnosis of idiopathic DI. Early detection of any evolving occult hypothalamic-pituitary stalk lesion may improve outcome. (Endocr Pract. 2009;15:240-245)     

Langerhans cell histiocytosis of the thyroid gland. A case report

BACKGROUND: The variant of Langerhans histiocytosis commonly encountered in adults is the benign, localized form (eosinophilic granuloma). The more-aggressive or diffuse type (Letterer-Siwe disease) is rare in adults. CASE: A 28-year-old woman presented with enlargement of the thyroid gland three years after she had been diagnosed with and placed on treatment for diabetes insipidus. Thyroidectomy was performed following an initial fine needle aspiration cytology report of either papillary thyroid carcinoma or Langerhans cell histiocytosis. The latter diagnosis was confirmed on histopathology and immunohistochemical staining for S-100 protein. Intracellular Birbeck granules were also demonstrated by electron microscopy. The disease progressed over a 10-week period to involve the kidneys, resulting in renal dysfunction. CONCLUSION: Synchronous or metachronous involvement of the hypothalamus and thyroid gland by Langerhans histiocytosis could not be excluded in the present case, with subsequent progression to involve other organs. This was an unusual presentation of the disease in an adult.     

Central diabetes insipidus as the first manifestation of neurosarcoidosis in a 10-year-old girl

Central diabetes insipidus with thickening of the pituitary stalk has been demonstrated in adults with Langerhans cell histiocytosis (LCH), tuberculosis or sarcoidosis, and in children with LCH. We present a 10.5-year-old girl with central diabetes insipidus. Magnetic resonance imaging (MRI) revealed thickening of the central part of the pituitary stalk and multiple hyperintense lesions in the frontal white matter on T(2)-weighted images. Laboratory findings were normal except for an elevated serum angiotensin-converting enzyme (ACE) level. The MRI findings together with the elevated serum ACE level highly suggest the diagnosis of sarcoidosis. We conclude that central diabetes insipidus can be the first clinical manifestation of sarcoidosis in children.