Clinical presentation and outcome of children with central diabetes insipidus associated with a self‐limited or transient pituitary stalk thickening, diagnosed as infundibuloneurohypophysitis

Abstract

Conclusions | METHODS | Patients | RESULTS | Clinical presentationAsshowninTable1,medianageofthechildrenatdiagnosiswas6.7 | Laboratory findingsAllpatientshadabiochemicalandhaematologicalwork-up,including sedimentationrate,bloodcellcount,kidneyandliverfunctiontests, which were normal in all patients. All patients tested negatively for the classical tumour markers β-HCG and α-fetoprotein in blood and sixtestednegativelyinthecerebrospinalfluid. | Pituitary function | Autoimmunity parameters | MRI findingsAt presentation, all patients had a thickening of the pituitary stalk | Other complementary examinationsSix patients had cerebrospinal fluid examination and a chest X-ray, fivepatientshadawholeskeletalX-raysurvey,andonepatienthad abonescintigraphy.Alltheseexaminationswerenormal.Nopatient underwentastalkbiopsy. | TreatmentAllpatientsreceiveddesmopressinfromthediagnosisofCDI.Thepatientwithcentralhypothyroidismwastreatedwiththyroxinsupplementationandreceivedduring8weeksoralglucocorticoids,starting 4weeksafterdiagnosis,asanevolvinganteriorpituitaryinsufficiency was expected, given an initial increase in ST. None of the patients received surgical or any other medical treatment at diagnosis or at laterstage. | OutcomeFollow-updataforatleast18monthswereavailableforallpatients.Allpatientswerekeptundercloseclinical (3monthlyinthefirstyear) andMRI (6monthlyinthefirst2years) A limitation of our study is the retrospective study design. All children underwent different hormonal and biological testing at different moments in the follow-up, and in particular, the autoimmune parameterswereonlytestedinalimitedgroupofchildren Clin Endocrinol.2006;65:470-478. 4. Leger J,Velasquez A, Garel C, Hassan M, Czernichow P. Thickened pituitarystalkonmagneticresonanceimaginginchildrenwithcentral diabetesinsipidus.J Clin Endocrinol Metab.1999;84:1954-1960. 5. DiIorgiN,AllegriAE,NapoliF,etal.Centraldiabetesinsipidusinchildrenandyoungadults:etiologicaldiagnosisandlong-termoutcome ofidiopathiccases.J Clin Endocrinol Metab.201499:1264-1272. 6. HayashiY,OishiM,KitaD,WatanabeT,TachibanaO,HamadaJ.Pure lymphocyticinfundibuloneurohypophysitiscausedbytheruptureof rathke'scleftcyst:reportof2casesandreviewoftheliterature.Turk Neurosurg.201525:332-336