Idiopathic Central Diabetes Insipidus Research Articles

Diabète insipide central inaugural d'une leucémie myélomonocytaire chronique

Central diabetes insipidus is most frequently reported to occur after a trauma from surgery or accident. However, between 30 and 50% of cases are considered idiopathic. It's a rare complication of myelodysplastic syndrome. A 61-year-old patient presented central diabetes insipidus revealing, 17 months before, chronic myelomonocytic leukemia. Cytogenetics studies revealed monosomy 7. Acute myeloid leukemia appears 3 months after training rapid patient's death. Blood examination is necessary before to conclude idiopathic central diabetes insipidus. The discovery of chronic myelomonocytic leukemia implicates a rapid managing before its possible acute myeloid leukemia transformation. Indeed, prognosis of central diabetes insipidus and acute myeloid leukemia associated, in presence of monosomy 7, is very poor.

Corticotropin and cortisol response to maximal exercise testing in central diabetes insipidus

It is known that arginine vasopressin (AVP) has a stimulatory effect on corticotropin (adrenocorticotropic hormone; ACTH) and cortisol secretion especially during stress. The present study was designed to investigate the effect of stress on ACTH and cortisol levels in patients with central diabetes insipidus (DI) with endogenous AVP deficiency receiving AVP therapy, and to determine whether these children need steroid replacement during stress. Seven patients with a median age of 12 years (range 7-13 years) with idiopathic central DI on appropriate Desmopressin (DDAVP) therapy (group 1) and seven healthy controls with a median age of 15 years (range 13-20 years; group 2) were included in the study. Acute stress was produced in all children by treadmill exercise, assessed by maximal oxygen consumption and heart rate. ACTH and cortisol levels were determined before and after exercise. In group 1, median ACTH level after exercise (28.3 pg/mL) was not different from the median value (24.0 pg/mL) before exercise. However, median cortisol level (10.5 microg/dL) was significantly increased (14.9 microg/dL; P < 0.05) with exercise. In group 2, cortisol (median 9.3 microg/dL) and ACTH levels (median 6.3 pg/mL) were significantly increased after exercise (15 mug/dL and 13.6 pg/mL, respectively; P < 0.05). There was no significant difference between the groups with respect to cortisol levels before and after exercise, but the stimulated ACTH levels after exercise were higher in patients with DI than in the controls (P < 0.05). A positive correlation was observed between total daily DDAVP dose and cortisol level after exercise (r(s)= 0.786, P < 0.05). Cortisol response during acute stress is normal in children with DI and these patients do not need extra steroid treatment during stress. In contrast, the normal cortisol response obtained by increased ACTH levels in these patients indicates an increased sensitivity of corticotroph cells.

Clinical, endocrinological and radiological courses in patients who was initially diagnosed as idiopathic central diabetes insipidus

Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis. Results : Our study included 15 males and 5 females. Mean age of CDI diagnosis was 7.8±3.6 (2.1- 14.7) years. Mean follow-up duration was 8.6±5.1 (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor. Conclusion : About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.

A Case of Idiopathic Central Diabetes Insipidus together with Primary Empty Sella and Combined Pituitary Hormone Deficiency

Central diabetes insipidus is a heterogeneous condition that is characterized by polyuria and polydipsia, and this is due to a deficiency of arginine vasopressin. Central diabetes insipidus is rare in children and young adults, and up to 50 percent of cases are idiopathic. Genetic abnormalities in the homeobox genes have recently been shown, on sellar magnetic resonance imaging, to be associated with combined pituitary hormone deficiency with pituitary defect. We report here on a 44-year-old female who suffered from polydipsia, polyuria and primary amenorrhea since childhood. She was diagnosed with idiopathic central diabetes insipidus together with primary empty sella and combined pituitary hormone deficiency. On the genetic analysis, she was proven to have a point mutation of the PROP-1 gene, which is known as a cause of combined pituitary hormone deficiency.

Idiopathic central diabetes insipidus in children and young adults is commonly associated with vasopressin‐cell antibodies and markers of autoimmunity

Idiopathic central diabetes insipidus in children and young adults is commonly associated with vasopressin‐cell antibodies and markers of autoimmunity

Idiopathic central diabetes insipidus in children and young adults is commonly associated with vasopressin‐cell antibodies and markers of autoimmunity

Autoimmune targeting of hypothalamic-neurohypophyseal structures in children and young adults with posterior pituitary and anterior pituitary dysfunction, as well as pituitary stalk involvement, are not yet completely understood. We aimed to (1) evaluate the presence of circulating vasopressin-cell autoantibodies (AVPc-Abs) in young patients with central diabetes insipidus (CDI), (2) detect organ-specific autoantibodies as markers of autoimmunity, and (3) define the relationship between immune markers and neuroimaging findings. Twenty patients were evaluated at a median age of 16.3 years. Twelve patients had idiopathic CDI, six had Langerhans cell histiocytosis (LCH) and two had germinoma. AVPc-Abs were evaluated in 40 healthy children. Magnetic resonance imaging (MRI) of the hypothalamic-pituitary region was performed longitudinally in all subjects. Circulating arginine vasopressin (AVP), protein tyrosine phosphatase (IA2), glutamic acid decarboxylase (GAD), 21-hydroxylase (21-OH), endomysium antibodies (EMA), parietal cell (PCA), thyroid peroxidase (TPO), thyroglobulin (TG) and TSH-receptor (TSHr) autoantibodies were evaluated. Circulating AVPc-Abs were found in 15 patients (75%), nine with idiopathic CDI, four with LCH and two with germinoma; the pituitary stalk was involved in most of them. Five patients with idiopathic CDI showed a persistence of AVPc-Abs during follow-up and one became positive subsequently. Serum IA2 autoantibodies were demonstrated in 14 patients (70%) and 21-OH autoantibodies in three of them. In idiopathic CDI, circulating AVPc-Abs suggest an autoimmune involvement of the neurohypophyseal system. The identification of AVPc-Abs in subjects who could have either idiopathic CDI or LCH or germinoma, however, indicates that AVPc-Abs cannot be considered a completely reliable marker of autoimmune CDI. Thus, close clinical and MRI follow-up are needed because AVPc-Abs may mask germinoma or LCH.

Clinical, hormonal and imaging findings in 27 children with central diabetes insipidus

Clinical, auxological, biological and neuroradiological characteristics of 27 children with central diabetes insipidus (CDI) were retrospectively analysed. Median age at diagnosis was 8.6 years (range: 0.3-16.1 years). Final aetiologies were postsurgical infundibulo-hypophyseal impairment (n=7), cerebral tumour (n=8), Langerhans cell histiocytosis (n=3), septo-optic dysplasia (n=1), ectrodactyly ectodermal dysplasia clefting syndrome (n=1), and idiopathic (n=7). In the non-postsurgical CDI patients, major cumulative and often subtle presenting manifestations were: polyuria (n=20), polydipsia (n=19), fatigue (n=11), nycturia (n=10), growth retardation (n=9), and headache (n=9). An associated antehypophyseal insufficiency, mainly somatotropic, was documented in 11 children. All patients except one who initially had a cerebral tomography, underwent magnetic resonance imaging revealing the lack of the physiological posterior pituitary hyperintense signal. One third of the idiopathic patients initially had a thickened pituitary stalk. All patients with idiopathic CDI were intensively followed up with 3-monthly physical examination, antehypophyseal evaluation, search for tumour markers, and cerebral MRI every 6 months. In one of them the pituitary stalk had normalized after 4.3 years. In one patient Langerhans cell histiocytosis was diagnosed after 7 months of follow-up, and in another patient a malignant teratoma was found after 2.4 years of follow-up. CDI may be the early sign of an evolving cerebral process. The association of polyuria-polydipsia should incite a complete endocrine evaluation and a meticulous MRI evaluation of the hypothalamo-hypophyseal region. A rigorous clinical and neuroradiologic follow-up is mandatory to rule out an evolving cerebral process and to detect associated antehypophyseal insufficiencies.

Magnetic resonance imaging of thickened pituitary stalk proceeding to Langerhans cell histiocytosis in a child

Magnetic resonance imaging has shown isolated pituitary stalk thickening in certain cases of idiopathic or secondary central diabetes insipidus (DI) due to infiltrative processes. We present a 4-year-old boy who was initially diagnosed as having central DI. The MRI showed isolated pituitary stalk thickening with prominent homogeneous contrast enhancement. The remaining findings on MRI were within normal limits. The patient's personal and family history and laboratory and clinical findings were unremarkable; therefore, he was initially diagnosed as having idiopathic DI. Since central DI and isolated pituitary stalk thickening may be considered to be the first manifestations of Langerhans cell histiocytosis, we decided to follow up the patient. After 5 months, following the initial diagnosis, on skeletal X-ray survey, the patient did indeed develop multiple lytic skull lesions which, on biopsy, were histologically typical bone lesions of Langerhans cell histiocytosis.

Primary central nervous system lymphoma of the pituitary stalk presenting with central diabetes insipidus: a case report

Isolated thickening of the pituitary stalk accompanied with central diabetes insipidus provides a diagnostic challenge. We report an unusual case of a 37-year-old woman presenting with a short history of polyuria and polydipsia. Endocrine evaluation confirmed the diagnosis of isolated central diabetes insipidus. Treatment with desmopressin relieved the symptoms. The initial MR imaging revealed an enhancement and thickening of the pituitary stalk suggesting hypophysitis. A high-dose prednisolone therapy showed no beneficial effect. The follow-up imaging showed a progressive thickening of the stalk and a polycyclic mass stretching towards the hypothalamic region. Renewed endocrine evaluation showed a partial insufficiency of the somatotrope, thyrotrope, and gonadotrope axes, and a hyperprolactinaemia. We did not detect any pathological findings in blood count, protein electrophoresis, inflammatory and tumour markers or cerebrospinal fluid examination. Neurosurgical biopsy and further imaging, including FDG-PET and MRI follow-up, suspected primary central nervous system lymphoma (PCNSL) as diagnosis. The patient was treated with high-dose methotrexate chemotherapy. Recent reassessment including FDG-PET and MRI scan showed a response with subsequent tumour reduction after 4 cycles of chemotherapy. To our knowledge central diabetes insipidus caused by a PCNSL located in the pituitary stalk is extremely rare, with only one reported case in the literature [Baleydier 2001]. Persistence of idiopathic central diabetes insipidus or progression of pathological MR imaging should prompt the clinician to initiate repeated search for an underlying cause. This case highlights the rare manifestation of PCNSL and the difficulties that may be encountered. In addition it points out the need for vigilance in follow-up brain imaging and hormonal evaluation in patients with idiopathic diabetes insipidus.

Retrospective Observation of Long-Term Clinical Courses of Idiopathic Central Diabetes Insipidus in Adults

Background: Idiopathic central diabetes insipidus (CDI) can be diagnosed when it occurs in the absence of a genetic or secondary cause known to be responsible for diabetes insipidus (DI). Some studies have reported that idiopathic CDI in adults shows a more benign clinical course than in children and young patients. However, the clinical characteristics and progress of this disorder have not been fully described. Therefore, we investigated the clinical courses of adult patients over the age of sixteen years with idiopathic central DI. Methods: We reviewed the medical records of all patients who had documented cases of idiopathic CDI from 1989 to 2005, and studied clinical features, hormone data, and imaging studies at diagnosis and during at least 1-year of follow-up. Results: There were 9 male (30.0%) and 21 female (70.0%) patients with a mean age of 39.3 years at diagnosis and a mean follow-up duration of 6.9 years. At diagnosis, deficits in anterior pituitary hormones were documented in 6 patients (20%), hyperprolactinemia in 4, and hypogonadism in 2. Two patients had an anterior pituitary hormone deficiency that was newly detected at a mean 3.4 years after the onset of DI. On initial MRI, the posterior pituitary was not hyperintense in 7 of the 30 patients (23.3%), but pituitary stalk thickening was observed in 15 (50.0%). After a mean follow-up of 6.9 years (range: 1 to 18), follow-up pituitary MRI showed improvement or no changes in patients with initial MRI findings of a pituitary abnormality, and no development of new lesions in 7 patients with a normal pituitary finding on initial MRI. Conclusion: Two of the 30 patients with idiopathic CDI developed an anterior pituitary hormone deficiency during follow-up, but no subject showed any aggravation on follow-up MRI. No patient showed a newly developed pituitary abnormality on follow-up MRI after a negative finding on the initial MRI (J Kor Endocrinol Soc 21:482~489, 2006)

Serial T1-weighted magnetic resonance imaging changes in a patient with central diabetes insipidus, possibly due to lymphocytic infundibuloneurohypophysitis

Central diabetes insipidus (CDI), characterized by polydipsia and polyuria due to vasopressin deficiency, is familial, idiopathic or secondary. Idiopathic CDI, which accounts for 10% to 30% of cases of CDI (1), is characterized by selective hypofunction of the hypothalamic‐neurohypophysial system. Idiopathic CDI could be an autoimmune disorder (2). Imura et al. (3) studied the processes of idiopathic CDI by magnetic resonance imaging (MRI), found abnormalities in the pituitary stalk and neurohypophysis, and performed biopsies that demonstrated lymphocytic inflammation. Their cases were diagnosed with CDI due to lymphocytic infundibuloneurohypophysitis. Their study provided new insight into the pathogenesis of CDI. However, they did not demonstrate the serial changes by MRI. We demonstrate the serial T1-weighted MRI changes in a 52-year-old woman with CDI possibly due to lymphocytic infundibuloneurohypophysitis. The initial MRI findings on admission were prominent pituitary-stalk thickening and neurohypophysial enlargement. These findings suggested that the patient had lymphocytic infundibuloneurohypophysitis (3‐5). The serial T1weighted MRI changes which occurred over the years were (i) prominent pituitary-stalk thickening and neurohypophysial enlargement, (ii) their improvement, and finally (iii) empty sella. The MRI changes are associated with the clinical course of the disease. Steroids were not administered to the patient; she recovered from CDI spontaneously. This is the first report to demonstrate the natural course of serial MRI changes in a patient with CDI possibly due to lymphocytic infundibuloneurohypophysitis. A 52-year-old woman had been well until 27 October 2002, when she began to have extreme thirst and excessive urine output. She visited us on 2 December 2002. She had extreme thirst and had started to drink more than 2literswater aday.Shehad decreased anti-diuretic hormone (ADH) levels (0.3ng/l, reference range 0.3‐ 3.5ng/l) with a low urine osmolality of 100mOsm/kg, high plasma osmolality of 310mOsm/kg (reference range 278‐305mOsm/kg), and a high serum

Etiologies of Central Diabetes Insipidus in Thai Children

In order to determine the etiologies of central diabetes insipidus (DI) among Thai children, we retrospectively reviewed records of 67 patients (44 male and 23 female) between the age of 0.1 and 15.6 years (mean age 5.5 years) who were diagnosed with central DI at the Pediatric Endocrinology Unit, Siriraj Hospital, Bangkok, Thailand between 1996 and 2003. Etiologies were found to be central nervous system (CNS) malformations in 20 patients (29.8%), post resection of intracranial tumors and cysts in 17 patients (25.4%), intracranial tumors in 11 patients (16.4%), cerebral palsy of unknown cause in five patients (7.5%), infection in four patients (6%), and Langerhans cell histiocytosis in three patients (4.5%). In another seven patients (10.4%), the cause was considered to be idiopathic. This study revealed that CNS malformation was the most common etiology, followed by intracranial tumors and their treatment. A thorough investigation to rule out CNS lesions needs to be done before considering the diagnosis of idiopathic central DI.

Idiopathic central diabetes insipidus is associated with abnormal blood supply to the posterior pituitary gland caused by vascular impairment of the inferior hypophyseal artery system.

Central diabetes insipidus (CDI) has been linked to vascular central nervous system damage, although the pathophysiology of the mechanism has never been perfectly understood. Indeed, the vascular system of human pituitary gland has rarely been the subject of rigorous investigation except at postmortem. Recently, studies of pituitary gland blood supply have been carried out by means of a time evaluation of pituitary gland enhancement with noninvasive dynamic magnetic resonance (MR) imaging after contrast medium injection. In the present study, we decided to investigate the status of posterior pituitary blood supply by evaluating vascular pituitary patterns in a group of 19 patients with idiopathic CDI in whom previous standard MR imaging had failed to identify causal specific lesions. The control group was composed of 55 subjects with a median age of 12 yr (range, 4.2-17 yr) who had idiopathic isolated GH deficiency and normal pituitary morphology and 15 young adults (18-25 yr) who had normal pituitary gland and no endocrine dysfunction. Nineteen patients (12 females and seven males), ranging in age at the time of diagnosis of CDI from 0.5-14.9 yr (median, 5 yr), were examined with dynamic MR imaging between 1990 and 1997 at a median age of 14.1 yr (range, 5.0-26.3 yr). CDI was diagnosed according to clinical findings of polyuria and polydipsia, water deprivation test, and desmopressin acetate therapeutic trial. All of the patients had permanent CDI and were being treated with satisfactory results with desmopressin, two to three times daily, either intranasally or orally. The previous MR imaging findings of the 19 CDI patients had shown the absence of posterior pituitary hyperintensity, normal pituitary stalk, and normal anterior pituitary size. Enhancement of the straight sinus, representing a temporal reference point and occurring in normal subjects simultaneously to that of the posterior pituitary gland, was observed in all subjects after iv gadopentetate dimeglumine administration, with no substantial differences between patients and controls. However, the enhancement of the posterior pituitary lobe occurred simultaneously with the enhancement of the straight sinus in all of the controls but in only 14 of the 19 patients with CDI. In the remaining five patients, the enhancement of the straight sinus was not associated with the expected contrast enhancement of the posterior pituitary gland, suggesting abnormal blood supply to the posterior pituitary lobe. This is in keeping with vascular impairment of the inferior hypophyseal artery system and suggests that abnormal blood supply to the posterior pituitary gland is associated with what, until now, has been considered idiopathic CDI.

Central diabetes insipidus and autoimmunity: relationship between the occurrence of antibodies to arginine vasopressin-secreting cells and clinical, immunological, and radiological features in a large cohort of patients with central diabetes insipidus of known and unknown etiology.

Central diabetes insipidus (CDI) is a rare hypothalamus-pituitary disease due to the deficiency of arginine vasopressin (AVP) synthesis from the hypothalamus and/or secretion from the neurohypophysis. The etiology of CDI is unknown in over one third of cases, classified as idiopathic CDI. The aim of this study was 2-fold: 1) to evaluate the occurrence of circulating autoantibodies to AVP-secreting cells (AVPcAb), and 2) to correlate it to clinical (sex, age of disease onset, disease duration, and degree), immunological (clinical history of autoimmune diseases and presence of related organ-specific autoantibodies), and radiological features (neurohypophyseal bright spot, pituitary stalk thickening, and empty sella) in a large cohort of patients with apparently idiopathic CDI or CDI of known etiology. To this purpose, 150 patients with CDI were studied: 64 idiopathic, 6 familial, 12 associated to granulomatous diseases, and 68 secondary to cranial trauma, tumor, or surgery. AVPcAb were measured by an indirect immunofluorescence method. AVPcAb were found in 23.3% of CDI patients: 21 idiopathic (32.8%) and 14 nonidiopathic (16.3%; chi(2) = 13.1; P < 0.001). AVPcAb were independently associated with age less than 30 yr at disease onset (P = 0.001) in patients with idiopathic CDI and with history of autoimmune diseases (P = 0.006 and P = 0.02, respectively) and radiological evidence of pituitary stalk thickening (P = 0.02 and P = 0.003, respectively) in both idiopathic and nonidiopathic CDI. The likelihood of autoimmunity in one patient with apparently idiopathic CDI with age of disease onset less than 30 yr was 53%, it increased to 91% when history of autoimmune diseases was associated and to 99% when pituitary stalk thickening was further associated. In conclusion, autoimmunity is associated with one third of patients with apparently idiopathic CDI, which should therefore be classified as autoimmune CDI. Autoimmune CDI is highly likely in young patients with a clinical history of autoimmune diseases and radiological evidence of pituitary stalk thickening. Conversely, autoimmunity probably represents an epiphenomenon in patients with nonidiopathic CDI.

Longitudinal study of vasopressin-cell antibodies and of hypothalamic-pituitary region on magnetic resonance imaging in patients with autoimmune and idiopathic complete central diabetes insipidus.

Diagnosis of autoimmune central diabetes insipidus (CDI) is based on the presence of autoantibodies to AVP-secreting cells (AVPcAb) or the coexistence of other autoimmune polyendocrine syndromes; moreover, it can be also suggested by the presence of lymphocytic infundibulo-neurohypophysitis, evidenced by biopsy of pituitary stalk and/or by pituitary stalk thickening on magnetic resonance imaging (MRI). However, so far, in clinical CDI patients with lymphocytic infundibulo-neurohypophysitis, AVPcAb have not been investigated and in those with or without autoimmune polyendocrine syndromes (APS), longitudinal studies on the behavior of AVPcAb alone, or of both AVPcAb and hypothalamic pituitary imaging on MRI are lacking. Aim of this work was to investigate in these patients the occurrence of AVPcAb (by indirect immunofluorescence) and of pituitary stalk thickening (by MRI) and their longitudinal changes during a follow-up period. We studied 22 patients, aged 29-53, with APS and complete CDI, grouped as follows: 10 with recent onset (< or =1.5 yr) of CDI (group 1a) and 12 with CDI of long-term duration (> or = 7 yr) (group 1b); moreover, a group of 13 patients with apparent idiopathic CDI of recent onset (<1.5 yr) were studied. They were divided, on the basis of the detection of AVPcAb as follows: 5 AVPcAb positive patients (aged 19-26) classified as isolated autoimmune CDI (group 2) and 8 AVPcAb negative patients (aged 21-26), classified as true idiopathic CDI (group 3). All patients were evaluated yearly, along 5 yr, for AVPcAb and for hypothalamic-pituitary region imaging. At study entry, 8/10 (80%) of patients in group 1a and 7/12 (58.3%) in group 1b were positive for AVPcAb and persisted positive subsequently, during all the follow-up period, even if at lower titers. All patients in group 2 were positive and all those in group 3 were negative for AVPcAb and persisted positive and negative, respectively, for all the follow-up study. Among the AVPcAb-positive patients, only 5 in group 1a and 2 in group 2 showed also pituitary stalk thickening at the first observations, which however spontaneously disappeared subsequently indicating a possible lymphocytic infundibulo-neurohypophysitis. All patients in the studied groups showed loss of the hyperintense signal of the neurohypophysis on MRI at entry and during all the follow-up period. Results of this longitudinal study suggest: 1) AVPcAb, frequently present at high titers in recent phases of CDI, persist subsequently, even if at lower titers, several years after the onset of disease. 2) The occurrence of a lymphocytic infundibulo-neurohypophysitis suggested by the pituitary stalk thickening on MRI only in patients positive for AVPcAb confirms a further autoimmune variant of CDI also in these cases. 3) The longitudinal behavior of patients in group 3 suggests that the absence of AVPcAb at the onset of clinical idiopathic CDI is able to exclude a subsequent appearance of these antibodies and consequently an autoimmune involvement in CDI of these patients. Instead the finding of AVPcAb in several patients with only CDI, thought at first clinical observation as idiopathic, indicates that the prevalence of autoimmune CDI must be considered much higher than that so far reported.

Longitudinal Study of Vasopressin-Cell Antibodies and of Hypothalamic-Pituitary Region on Magnetic Resonance Imaging in Patients with Autoimmune and Idiopathic Complete Central Diabetes Insipidus

Longitudinal Study of Vasopressin-Cell Antibodies and of Hypothalamic-Pituitary Region on Magnetic Resonance Imaging in Patients with Autoimmune and Idiopathic Complete Central Diabetes Insipidus

Central diabetes insipidus due to lymphocytic infundibuloneurohypophysitis

Lymphocytic infundibuloneurohypophysitis was recently reported by Kojima et al (1) and Imura et al (2) as a cause of idiopathic central diabetes insipidus. We report 2 patients with central diabetes insipidus who had abnormal findings on magnetic resonance imaging (MRI) that were compatible with this syndrome. One of the patients had bilateral enlarged salivary glands that showed an infiltration of activated T lymphocytes, as has been reported in the pituitary stalk among patients with lymphocytic infundibuloneurohypophysitis. Both patients had antibodies for the p24 antigen of the human T lymphocytic leukemia virus-1 (HTLV-1), as well as having the human leukocyte antigen (HLA)-A24 haplotype.

Thickened Pituitary Stalk on Magnetic Resonance Imaging in Children with Central Diabetes insipidus

Magnetic resonance imaging (MRI) has revealed isolated pituitary stalk thickening (PST) in certain cases of idiopathic or secondary central diabetes insipidus (DI) due to infiltrative processes. Twenty-six children with DI and PST underwent cerebral MRI at the age of 8 ± 4 years and were followed (n = 25) by clinical and MRI evaluation for 5.5 ± 3.6 and 3.0 ± 2 years, respectively, but given no treatment other than hormonal substitutive therapy. Patients were subdivided into groups according to the etiology of the DI: germinoma (n = 4), Langerhans’ histiocytosis (n = 5) or ‘idiopathic’ DI with PST (n = 17). Complete anterior pituitary evaluation in 24 of the 26 patients revealed that 14 children were suffering from associated growth hormone deficiency and 7 had multiple hormone deficiencies. At the first MRI evaluation, pituitary stalk enlargement varied from 2.2 to 9.0 mm. The anterior pituitary gland was found to be normal (n = 12), small (n = 8) or enlarged (n = 6). At the final evaluation, a change in MRI features had occurred in 16 patients: morphological and/or signal changes in the PST (n = 16; 6 of whom showed an increase in PST) and changes in anterior pituitary gland size (n = 8; 3 of whom had increased and 5 had decreased). The presence of a growing suprasellar mass with a progressively enlarging pituitary stalk was demonstrated in the 6 patients who had shown increased pituitary stalk enlargement 1.8 ± 1.6 years after the first MRI. In 4 of these patients, a diagnosis of germinoma was made 1.3 ± 0.6 years after PST identification by MRI at the onset of DI, but the other 2 patients showing a suprasellar mass were still categorized as ‘idiopathic’ at the final clinical evaluation performed 7.8 and 12.3 years after DI onset. In 10 patients (all but 1 with Langerhans’ histiocytosis, showing ‘idiopathic’ DI) the pituitary stalk enlargement was diminished after 2.0 ± 1.9 years of MRI follow-up, and there was a complete reversal of pituitary stalk enlargement in 5 patients. We conclude that the natural history of ‘idiopathic’ isolated central DI with PST is unpredictable. Although germinoma should always be considered during the first 3 years of follow-up in patients presenting isolated DI with PST by repeated investigations every 3–6 months, it remains an infrequent etiology, affecting only 15% of cases in our study.

Diabetes insipidus.

Diabetes insipidus.

20 years of experience in idiopathic central diabetes insipidus

Review of the notes of 120 patients with central diabetes insipidus (CDI) referred to the London Centre for Paediatric Endocrinology at The Middlesex Hospital and Great Ormond Street Hospital for Children from May, 1977, to October, 1997, identified 16 patients (13 males and three females) in whom repeated endocrinological and neuroradiological investigations failed to reveal an underlying cause. Their age at presentation ranged from 6 months to 17 years and 3 months (median 6 years 7 months).