scholarly journals SUN-282 Transient Infundibulohypophysitis in a Child with Central Diabetes Insipidus

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Shabana Kalladi Puthanpurayil ◽  
Ahsan Uddin ◽  
Peter Matthew Gerrits
Keyword(s):  
Diabetes Insipidus ◽  
Anterior Pituitary ◽  
Elevated Serum ◽  
Central Diabetes Insipidus ◽  
Pituitary Stalk ◽  
Bright Spot ◽  
Neoplastic Process ◽  
Anterior Pituitary Function ◽  
History Of ◽  
Pituitary Stalk Thickening

Abstract Case Description A 7-year-old previously healthy female presented with a 1-month history of tiredness, excessive thirst and increased urinary frequency. Initial studies showed an elevated serum osmolality with relatively dilute urine. She underwent a water deprivation study which confirmed central diabetes insipidus (CDI). An MRI of the brain was obtained which showed markedly thickened and homogeneously enhancing pituitary stalk thickening (17mm craniocaudal x10 mm anteroposterior diameter). Workup did not reveal any signs suggestive of Langerhan’s cell histiocytosis; a skin biopsy and bone survey were normal. A pituitary stalk biopsy was scheduled, however direct endoscopic visualization of the pituitary stalk was normal, so the procedure was aborted. A repeat MRI 7 weeks later showed complete resolution of the stalk thickening. An MRI done 4 months later again showed normal anterior pituitary and normal pituitary stalk, with persistent absent posterior bright spot. She remains on desmopressin, and anterior pituitary functions have been normal to date. Discussion CDI in children can be due to infectious, neoplastic, traumatic or genetic processes that disrupt the hypothalamus or pituitary stalk. One third of children with CDI present with pituitary stalk thickening (PST) on initial evaluation with MRI. There are wide variations in the degree of PST. Previous studies have shown that the degree of PST is different depending on the underlying etiology. Furthermore, regression of PST has been observed in many cases of CDI with PST which has been attributed to idiopathic or autoimmune process (infundibulohypophysitis). Most studies have demonstrated that PST > 4 mm is unlikely to regress and may be more likely to progress with eventual diagnosis of a neoplastic process such as germinoma or Langerhan’s cell histiocytosis. Based on review of the literature, the earliest resolution of PST was in 3 months in a patient who received steroid therapy at diagnosis. [1] Conclusion Our case illustrates that in children with central diabetes insipidus, isolated pituitary stalk thickening may resolve in as short a period as 7 weeks. While ongoing evaluation is warranted with serial pituitary imaging, unnecessary invasive procedures may be avoided in patients without evidence of other underlying anatomic pathology. Anterior pituitary function requires initial and continued surveillance. References 1. Shaefers J et al. Clinical presentation and outcome of children with central diabetes insipidus associated with a self-limited or transient pituitary stalk thickening, diagnosed as infundibuloneurohypophysitis. Clin Endocrinol (Oxf). 2017 Aug;87(2):171-176. doi: 10.1111/cen.13362. Epub 2017 Jun 5.

scholarly journals Central Diabetes Insipidus as the Inaugural Manifestation of Langerhans Cell Histiocytosis: Natural History and Medical Evaluation of 26 Children and Adolescents

2011 ◽  
Vol 96 (9) ◽  
pp. E1352-E1360 ◽  
Author(s):  
Isis Marchand ◽  
Mohamed Aziz Barkaoui ◽  
Catherine Garel ◽  
Michel Polak ◽  
Jean Donadieu ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Third Ventricle ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus ◽  
Pituitary Stalk ◽  
The Third ◽  
Mri Features ◽  
Pituitary Stalk Thickening

Abstract Context: Isolated central diabetes insipidus (CDI) can be the first manifestation of Langerhans cell histiocytosis (LCH), creating diagnostic dilemmas such as dysgerminoma and other inflammatory lesions. Method: In 2010, the French national LCH registry had enrolled 1236 LCH patients under 18 yr of age. Isolated CDI was the initial presentation of LCH in 26 patients. We reviewed their clinical and magnetic resonance imaging (MRI) features. Results: Median age at the diagnosis of CDI was 9.6 yr (1.8–16.3), and median follow-up after CDI diagnosis was 9.9 yr (3.5–26.6). In addition to CDI, two patients had visual field defects, four had secondary amenorrhea, and 11 had anterior pituitary deficiency. Cerebral imaging (including computed tomography in two cases), performed in 22 patients within 3 months of CDI diagnosis, showed pituitary stalk thickening in 14 patients, which was moderate (3.0–7 mm) in nine cases and marked (>7 mm) in five cases. In eight cases, the lesion extended to the floor of the third ventricle. One child with LCH presented with a mild enlarged sellar content. During follow-up, 22 patients developed extrapituitary involvement, mainly of bone (n = 15), lung (n = 9), and skin (n = 9). Pituitary biopsy was performed in eight cases and was conclusive in six cases. Conclusions: Pituitary stalk thickening can be observed in LCH as well as lesions extending to the floor of the third ventricle. In all cases but one, the intrasellar content was not enlarged. Long-term follow-up with close attention to bone, skin, and lung disorders may lead to the diagnosis of LCH.

scholarly journals Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis

2017 ◽  
Vol 177 (2) ◽  
pp. 127-135 ◽  
Author(s):  
Isabella Lupi ◽  
Mirco Cosottini ◽  
Patrizio Caturegli ◽  
Luca Manetti ◽  
Claudio Urbani ◽  
...  
Keyword(s):  
Prognostic Factor ◽  
Diabetes Insipidus ◽  
Anterior Pituitary ◽  
Glucocorticoid Therapy ◽  
Pituitary Function ◽  
Pituitary Stalk ◽  
Autoimmune Hypophysitis ◽  
Negative Prognostic Factor ◽  
Anterior Pituitary Function ◽  
Design And Methods

Introduction Autoimmune hypophysitis (AH) has a variable clinical presentation and natural history; likewise, its response to glucocorticoid therapy is often unpredictable. Objective To identify clinical and radiological findings associated with response to glucocorticoids. Design and methods 12 consecutive patients with AH, evaluated from 2008 to 2016. AH was the exclusion diagnosis after ruling out other pituitary masses and secondary causes of hypophysitis. Mean follow-up time was 30 ± 27 months (range 12–96 months). Results MRI identified two main patterns of presentation: global enlargement of the pituitary gland or panhypophysitis (n = 4, PH), and pituitary stalk abnormality only, or infundibulo-neuro-hypophysitis (n = 8, INH). Multiple tropin defects were more common in PH (100%) than those in INH (28% P = 0.014), whereas diabetes insipidus was more common in INH (100%) than that in PH (50%; P = 0.028). All 4 PH and 4 out of 8 INH were treated with glucocorticoids. Pituitary volume significantly reduced in all PH patients (P = 0.012), defective anterior pituitary function recovered only in the two patients without diabetes insipidus (50%) and panhypopituitarism persisted, along with diabetes insipidus, in the remaining 2 (50%). In all INH patients, either treated or untreated, pituitary stalk diameter reduced (P = 0.008) but diabetes insipidus persisted in all. Conclusions Glucocorticoid therapy may improve anterior pituitary function in a subset of patients but has no effect on restoring posterior pituitary function. Diabetes insipidus appears as a negative prognostic factor for response to glucocorticoids.

scholarly journals Central diabetes insipidus and pituitary stalk thickening in adults: distinction of neoplastic from non-neoplastic lesions

2020 ◽  
Vol 183 (1) ◽  
pp. 95-105
Author(s):  
France Devuyst ◽  
Paraskevi Kazakou ◽  
Danielle Balériaux ◽  
Orsalia Alexopoulou ◽  
Agnès Burniat ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Prolactin Level ◽  
Anterior Pituitary ◽  
Male Gender ◽  
Adult Patients ◽  
Pituitary Stalk ◽  
Serum Prolactin ◽  
Serum Prolactin Level ◽  
Neoplastic Lesions ◽  
Pituitary Stalk Thickening

Context Association of central diabetes insipidus (CDI) and pituitary stalk thickening (PST) may have several etiologies (including malignancies) and differential diagnosis remains often difficult. Objective The purpose of this study was to identify which clinical, biochemical or radiological features could help clinicians to make an etiological diagnosis, especially distinguishing neoplastic from non-neoplastic pituitary stalk lesions. Design and methods We retrospectively analyzed clinical, biochemical, radiological and histological data of 38 adult patients diagnosed with CDI and PST of proven etiology. Results Of the 38 pituitary stalk lesions included, 11 (29%) were neoplastic. A histopathological diagnosis was obtained in 22/38 (58%) patients. The three most frequently observed etiologies of PST were neuroinfundibulitis (34%), germinoma (21%) and histiocytosis (18%). Pituitary stalk thickness was larger for neoplastic lesions, particularly germinomas. Male gender and a very young age were statistically associated with a risk of germinoma. At least one anterior pituitary deficit was observed in nearly 60% of patients. Patients with neoplastic PST were more affected by multiple anterior pituitary dysfunction than patients with benign PST. A high serum prolactin level was individually the best predictor of a neoplastic origin (90% sensitivity and 60% specificity for a serum prolactin level 1.27-fold above the normal upper limit (ULN)). Conclusion We confirm a relatively high risk of malignancy in adult patients presenting with the association of CDI and PST. Young age, male gender, a very large thickening of the stalk, multiple anterior pituitary deficits and prolactin above 1.3× ULN increase the likelihood of a neoplastic origin.

scholarly journals Central Diabetes Insipidus: A Previously Unreported Side Effect of Temozolomide

2013 ◽  
Vol 98 (10) ◽  
pp. 3926-3931 ◽  
Author(s):  
Alexander T. Faje ◽  
Lisa Nachtigall ◽  
Deborah Wexler ◽  
Karen K. Miller ◽  
Anne Klibanski ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Side Effect ◽  
Central Diabetes Insipidus ◽  
Bright Spot ◽  
Patient Database ◽  
Anterior Pituitary Function ◽  
The Central Nervous System ◽  
Effect Of Treatment ◽  
Case Presentations ◽  
Index Cases

Abstract Context: Temozolomide (TMZ) is an alkylating agent primarily used to treat tumors of the central nervous system. We describe 2 patients with apparent TMZ-induced central diabetes insipidus. Using our institution's Research Patient Database Registry, we identified 3 additional potential cases of TMZ-induced diabetes insipidus among a group of 1545 patients treated with TMZ. Case Presentations: A 53-year-old male with an oligoastrocytoma and a 38-year-old male with an oligodendroglioma each developed symptoms of polydipsia and polyuria approximately 2 months after the initiation of TMZ. Laboratory analyses demonstrated hypernatremia and urinary concentrating defects, consistent with the presence of diabetes insipidus, and the patients were successfully treated with desmopressin acetate. Desmopressin acetate was withdrawn after the discontinuation of TMZ, and diabetes insipidus did not recur. Magnetic resonance imaging of the pituitary and hypothalamus was unremarkable apart from the absence of a posterior pituitary bright spot in both of the cases. Anterior pituitary function tests were normal in both cases. Using the Research Patient Database Registry database, we identified the 2 index cases and 3 additional potential cases of diabetes insipidus for an estimated prevalence of 0.3% (5 cases of diabetes insipidus per 1545 patients prescribed TMZ). Conclusions: Central diabetes insipidus is a rare but reversible side effect of treatment with TMZ.

scholarly journals Neuroinfundibulitis: Pituitary Stalk Thickening Presenting as Diabetes Insipidus

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A593-A593
Author(s):  
Payal P Patel ◽  
Susan Leanne Samson ◽  
Sarika Rao
Keyword(s):  
Diabetes Insipidus ◽  
Hormone Replacement ◽  
Treatment Options ◽  
Head Injuries ◽  
Urine Osmolality ◽  
Pituitary Stalk ◽  
Bright Spot ◽  
Thyroid Function Tests ◽  
Normal Thyroid Function ◽  
Pituitary Stalk Thickening

Abstract Background: Isolated pituitary stalk thickening (PST) from neuroinfundibulitis is a rare entity that requires thorough evaluation of secondary causes and understanding of treatment options. Clinical Case: A 55-year-old male presented with progressive polyuria and polydipsia over several months. He had no significant head injuries, headache, weight loss, fatigue or inability to exercise. His serum sodium level was 148 (135 - 145 mmol/L) with a serum osmolality 310 (275-305 mOsm/kg), and urine osmolality was 246 (40-1200 mOsm/kg) suggestive of partial diabetes insipidus (DI). A 24-hour urine collection had a volume of 9.2 liters. He had a normal hemoglobin A1c. Hormonal work-up was unremarkable with normal range his prolactin, IGF-1, and a.m. cortisol (19 mcg/dl). He was already on levothyroxine with normal thyroid function tests and negative thyroid antibodies. Water deprivation testing confirmed a diagnosis of central DI and he was started on desmopressin. Magnetic resonance imaging (MRI) of the pituitary demonstrated diffuse PST with T2 hyperintensity and loss of normal T1 hyperintensity (“bright spot”) of the posterior pituitary. Repeat imaging 3 months later showed progressive thickening. He underwent full body positron emission tomography-computerized tomography (PET-CT) which demonstrated increased metabolism of the pituitary stalk, but no other suspicious uptake in other areas. Chest X-ray, tuberculin screen, angiotensin converting enzyme, and calcium were all normal. Patient underwent transsphenoidal stalk biopsy. Pathology demonstrated fibrosis and chronic inflammation without malignancy but otherwise was nondiagnostic. Based on these findings, he was presumed to have neuroinfundibulitis and is being actively followed with MRIs and labs. Conclusion: Isolated PST is exceedingly rare, especially in adults. The differential diagnosis includes neoplastic (germinomas, lymphoma, metastatic disease), autoimmune, and infiltrative disease (sarcoidosis, histiocytosis). One review of 38 adult patients with central DI and PST showed that almost half had neuroinfundibulitis. Of those, 4 were treated with corticosteroids and 7 were not. All patients showed reduction or resolution of PST on subsequent MRIs indicating that resolution of thickening can be spontaneous without steroid use, and can be followed by MRI and with hormone replacement for deficiencies (1). Because of the rarity of isolated neuroinfundibulitis, it is important that neoplastic and systemic diseases be ruled out in all patients with PST, as in the case of our patient. 1.Devuyst F, Kazakou P, Baleriaux D, Alexopoulou O, Burniat A, Salenave S, et al. Central diabetes insipidus and pituitary stalk thickening in adults: distinction of neoplastic from non-neoplastic lesions. European Journal of Endocrinology. 2020;183(1):95-105.

scholarly journals Difficulties in cerebrospinal fluid βhCG interpretation in a patient with an infundibular lesion

2018 ◽  
Vol 2018 ◽  
Author(s):  
Alicia R Jones ◽  
Alan McNeil ◽  
Christopher Yates ◽  
Bala Krishnamurthy ◽  
Peter S Hamblin
Keyword(s):  
Germ Cell ◽  
Diabetes Insipidus ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Pituitary Stalk ◽  
Bright Spot ◽  
List Type ◽  
Pituitary Stalk Thickening ◽  
Serial Mri

Summary A variety of neoplastic, inflammatory and congenital conditions can cause pituitary stalk thickening. Differentiating between these causes is important as targeted treatment may be offered. Diagnostic work-up consists of a thorough history, examination, biochemical analysis and imaging. We present the case of a 33-year-old male who presented with diabetes insipidus and had pituitary stalk thickening on magnetic resonance imaging. Further investigations revealed an elevated CSF βhCG, which raised the possibility of an intracranial germ cell tumor. However, when repeated on four different assays, the βhCG levels were discordant. On serial imaging, the pituitary stalk thickening reduced slightly, which would be unexpected for a germ cell tumor. This case raises the difficulties interpreting CSF βhCG, as not all immunoassays for βhCG have been validated for use in CSF. The Roche Diagnostics Elecsys and Siemens Centaur assays have been validated for CSF βhCG, and so we advocate using one of these methods. If unavailable or serum/CSF results are ambiguous, serial MRI is appropriate, with pituitary stalk biopsy considered if the stalk measures >6.5 mm or other imaging abnormalities are present. Learning points: Most adult patients with central diabetes insipidus have imaging abnormalities on a pituitary MRI. The most common abnormalities are loss of the posterior pituitary bright spot and pituitary stalk thickening, both of which are non-specific. Causes of pituitary stalk thickening include neoplastic, inflammatory, infective and congenital lesions. Investigation of pituitary stalk thickening should encompass the many possible causes and include biochemical analyses as well as imaging of the chest, abdomen and pelvis. Further investigations should be guided by the clinical context, but may include testicular ultrasound, CSF analysis and pituitary stalk biopsy. Germ cell tumors involving the pituitary stalk may be suspected on clinical grounds, but in the absence of a tissue diagnosis (biopsy) confirmation may be difficult and relies on biochemical assessment of blood and possibly CSF as well as serial MRI imaging. CSF βhCG levels should be analyzed on an instrument validated for use in CSF or on multiple instruments, and the pitfalls of testing this marker (false negative in some germ cell tumors, false positives in other conditions, lack of internationally agreed reference ranges for diagnosing germ cell tumors) should be considered when interpreting the results.

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