Magnetic Resonance Imaging Spectrum of Pituitary Involvement in an Adolescent with Central Diabetes Insipidus: A Case Suggestive of Langerhans Cell Histiocytosis.

Disease Extent, Not Lesion Location, Determines Relapse Risk in Single-System Skeletal Langerhans Cell Histiocytosis.

Arginine vasopressin (AVP) and oxytocin (OXT) are both hormones released from the posterior pituitary. While AVP primarily regulates water reabsorption in the kidneys, OXT plays a key role in socioemotional functioning. Due to the anatomical proximity, disruptions of the AVP system leading to AVP deficiency (AVP-D) may also affect the OXT system, possibly resulting in an additional OXT deficiency. This hypothesis was recently proven by using the 3,4-methylenedioxymethamphetamine stimulation tests and identifying OXT deficiency in patients with AVP-D, linked to increased anxiety and impaired emotion recognition. Despite these findings, OXT replacement therapy is not currently established as a treatment for AVP-D and long-term replacement therapy remains unexplored. This is a randomised, double-blind, placebo-controlled, parallel-group trial enrolling adults with AVP-D. Participants are randomised 1:1 to receive intranasal OXT (Syntocinon, 24 IU twice daily) or placebo for 28 days. The primary endpoint is a composite binary outcome defined as a clinically meaningful improvement in either trait anxiety (≥5-point reduction in State-Trait Anxiety Inventory-Trait Score) or emotion recognition (≥4-point increase in EmBody/EmFace task performance). Secondary outcomes include empathy, stress reactivity, neuroimaging markers of amygdala activity, additional psychological measures, metabolic parameters and safety outcomes, including hyponatraemia. Analyses will follow the intention-to-treat principle, with Fisher's exact test used for the primary outcome and mixed-effects models for secondary endpoints. The study has been approved by the competent ethics committees and regulatory authorities in Switzerland and the European Union. The following institutions granted ethical approval: Ethikkommission Nordwest- und Zentralschweiz (EKNZ), project number EKNZ 2023-01010 and the Erasmus MC MERC, EU-CT number 2024-5 16 813-19-00. Results will be published in open-access, peer-reviewed journals and disseminated via scientific meetings, media communication and lay summaries provided to participants. De-identified individual participant data will be made available on reasonable request following publication. NCT06036004.

Chimeric antigen receptor (CAR) T-cell therapy is increasingly used for hematologic malignancies, yet the spectrum and clinical significance of its endocrine adverse events (AEs) remain poorly characterized. To identify and clinically contextualize signals of endocrine AEs associated with CAR T-cell therapy, with a focus on their overlap with cytokine release syndrome (CRS). We performed a retrospective disproportionality analysis using the FDA Adverse Event Reporting System (FAERS) database (2017 to Q2 2025). Reporting odds ratio (ROR) and information component (IC) were calculated for endocrine AEs associated with six CAR T-cell products. To validate and elaborate these pharmacovigilance signals, a structured literature review was conducted to identify published clinical evidence. The FAERS analysis identified 269 endocrine AE reports, yielding 14 significant disproportionality signals. Hyperglycemia was the most frequently reported event, while estrogen deficiency showed the strongest signal strength (ROR₀₂₅ = 14.93). Signals for adrenal insufficiency and hypothalamo-pituitary disorders were primarily associated with axicabtagene. Critically, mortality was frequently reported among cases with positive endocrine signals, particularly when the endocrine AE co-occurred with CRS. The literature review provided direct clinical validation: a retrospective study confirmed a 39% incidence of CRS-associated hyperglycemia, and independent case reports documented the first instances of CAR T-cell therapy-induced Hashimoto's thyroiditis and central diabetes insipidus. These clinical cases corroborated the FAERS signals and suggested immune-inflammatory mechanisms, often independent of corticosteroid use. This integrated analysis reveals a distinct spectrum of CAR T-cell-related endocrine toxicities, encompassing glucose and calcium dysregulation, pituitary axis disorders, and autoimmune phenomena. The frequent and potentially fatal overlap with CRS underscores the need for enhanced clinical vigilance. Proactive monitoring of endocrine function, especially in patients experiencing CRS, is warranted to mitigate these underrecognized complications.

Case series summary A 2-year-old neutered male domestic shorthair cat presented with severe lethargy, hyporexia, dehydration, and poor body condition. The incidence of polyuria/polydipsia was unknown because of the presence of multiple other cats in the household. Laboratory findings revealed severe hypernatremia, isosthenuria, hemoconcentration, and moderate azotemia. MRI of the head demonstrated partial hypoplasia/aplasia of the hypothalamus and pituitary gland, confirming congenital central diabetes insipidus; additional endocrine testing excluded other causes. Initial fluid therapy improved azotemia and stabilized the hematocrit, but hypernatremia persisted until desmopressin therapy was initiated. The cat was successfully managed long-term with gradually titrated oral desmopressin, achieving normalization of electrolytes. Relevance and novel information This report details the first documented case of central diabetes insipidus in an adult cat, attributed to presumed partial aplasia of the hypothalamus and pituitary gland, with no other hormonal deficiencies.

Primary hypothalamic involvement in primary central nervous system lymphoma is rare and can present with nonspecific neurological and endocrine manifestations, resulting in significant diagnostic delays. We report the case of an immunocompetent 29-year-old female who presented with intermittent fever, seizures, progressive behavioral changes, central diabetes insipidus, and hypopituitarism. She was initially treated empirically for encephalitis with anti-epileptics and corticosteroids. Neuroimaging subsequently demonstrated a homogeneously enhancing lesion along the chiasmatic-hypothalamic axis. Initial biopsy was nondiagnostic due to corticosteroid pretreatment, necessitating repeat biopsy via craniotomy, which confirmed diffuse large B-cell lymphoma. This case illustrates the importance of recognizing endocrine red flags in hypothalamic lesions, emphasizes the negative impact of prebiopsy steroid exposure on diagnostic yield, obscuring histopathology even in the absence of radiological regression.

Arginine vasopressin (AVP) regulates homeostasis and social behavior. Damage to AVP neurons causes central diabetes insipidus (CDI), now termed arginine vasopressin deficiency (AVP-D), a rare disorder potentially linked to social deficits. This study examined how AVP deficiency affects social, emotional, and psychological functioning in AVP-D patients. Twelve AVP-D patients and twelve matched controls participated. Blood samples measured copeptin and other hormones. Participants completed behavioral tasks assessing social exclusion (Cyberball paradigm), emotion recognition (Reading the Mind in Films), prosocial behavior (Social Discounting Task), fairness (Ultimatum Game), and approach-avoidance (Stop Distance Paradigm). Questionnaires assessed mood, anxiety, aggression, alexithymia, and empathy. Hormone analysis showed AVP-D patients had lower copeptin and lower ACTH levels. Psychometric tests indicated increased depression, aggression, and alexithymia. Both groups felt negative after social exclusion, but AVP-D patients adjusted less emotionally, showing smaller changes in insecurity and team spirit. AVP-D patients also struggled more with emotion recognition, linked to lower copeptin and higher alexithymia. Fairness sensitivity differences were minor and not significant. Both groups donated less as social distance grew, though AVP-D patients' decline was less steep. In approach-avoidance behavior, AVP-D patients kept greater distances from human faces with friendly or aggressive expressions, unlike with animal targets. AVP-D patients exhibit socioemotional impairments like poor emotion recognition and depression, highlighting the need for routine screening. Early detection enables targeted interventions, and addressing central AVP pathways is crucial, underscoring the need for treatments beyond hormone replacement.

WCN26-7418 Eosinophilic Granulomatosis with Polyangiitis Following Central Diabetes Insipidus: A Rare Case Report and Literature Review

Transient Syndrome of Inappropriate Antidiuretic Hormone Secretion and Sustained Improvement in Central Diabetes Insipidus After Roux-en-Y Gastric Bypass

The incidence of pituitary stalk thickening (PST) has increased in recent years due to the increased utilization of diagnostic MRI of the brain. The clinical significance of PST, appropriate diagnostic workup, and clinical course for these patients is not well documented. After Institutional Review Board approval, we reviewed the pediatric tumor board and pediatric endocrinology clinic records between 2003 and 2024 to identify patients with PST. We followed the clinical course of these patients to collect applicable data. A total of 29 patients were identified with PST; 11 were excluded due to additional MRI findings, and out of 18 selected patients, 9 were females (50%). Initial diagnostic workup included complete blood count (CBC), basic metabolic panel, serum and cerebrospinal fluid (CSF) tumor markers (AFP, β-hCG), and skeletal survey. With the initial workup, no patient was diagnosed with neoplasm. Patients were followed with serial MRIs. Five patients (27.7%) displayed progression of PST, warranting biopsy (2 germinomas, 2 Langerhans cell histiocytosis, and 1 inconclusive). The median time to biopsy was 8 months (range 4-26 months). All the patients diagnosed with neoplasm had central diabetes insipidus at presentation, and 3 went on to develop additional endocrine abnormalities. Ten patients had stable PST over a median follow-up period of 19 months (range 0-81 months), and 3 patients had complete resolution of PST during the follow-up period. Most isolated PST among pediatric patients remain stable over time. Serial monitoring of patients with isolated PST can be safe, and not all cases of PST warrant upfront biopsy.

Three Years to Diagnosis: Idiopathic Central Diabetes Insipidus in a Healthcare Worker

Concurrent Central Diabetes Insipidus and Panhypopituitarism in Newly Diagnosed Acute Myeloid Leukemia

Endocrine Disruptions Leading to Hypernatremia and Fluid Imbalance in Neurosarcoidosis: The Challenge of Central Diabetes Insipidus and Adrenal Insufficiency

Background: Paraneoplastic limbic encephalitis (PLE) with anti-Ma2 antibodies is a rare immune-mediated disorder associated with testicular cancer, particularly in young males. While neurological manifestations are well documented, hypothalamic-pituitary dysfunctions remain underreported. We present a case of anti-Ma2 PLE associated with testicular cancer together with a systematic review of PLE associated with testicular cancer, selectively restricted to anti-Ma2 positive cases and focusing on hypothalamic-endocrine involvement. Case presentation: We describe a 21-year-old male diagnosed with anti-Ma2 PLE and intratubular germ cell neoplasia of the right testis. He underwent orchifunicolectomy and immunosuppressive therapy with neurological improvement. Four years later, he developed new-onset temporal seizures, decreased libido, and a polyuria-polydipsia syndrome. Dynamic endocrine testing, including a water deprivation test and copeptin measurement, supported a diagnosis of partial central diabetes insipidus (CDI). Methods: A systematic literature review was performed in accordance with PRISMA guidelines. PubMed was searched using predefined keywords without time restriction. Studies reporting PLE associated with testicular tumors in humans with confirmed anti-Ma2 antibody positivity were included. Results: Eleven studies were included, reporting a total of 38 patients with anti-Ma2-associated PLE and testicular cancer. Hypothalamic or diencephalic involvement was described in 16 patients (42.0%), while endocrine manifestations were explicitly reported in four cases. Only two previous reports mentioned CDI, without detailed diagnostic evaluation. Conclusions: This study highlights the importance of recognizing hypothalamic-endocrine manifestations in PLE. In patients presenting with polydipsia and polyuria, CDI should be carefully differentiated from primary polydipsia using dynamic testing. Hypothalamic involvement may emerge years after tumor treatment, warranting long-term endocrine surveillance.

Background: Craniopharyngiomas are benign tumor of skull base which often follows an aggressive clinical course. Adamantinomatous subtype are frequently observe in age group 5-15 years. Currently, treatment strategy has relied exclusively on surgery and radiotherapy. Materials and Methods: All pediatric Craniopharyngiomas operated at National Neurosurgical Referral Center, Bir hospital from Nov 2021 to Dec 2023 were included in this study. Results: A total of 17 patients underwent surgical excision of craniopharyngioma. The male to female ratio was 4.6:1. The mean age of the patients was 10.88 ± 4.18 (Range 2 to 16 years). The most common presenting symptoms were headache (64.7%) followed by visual disturbances.12 patients exhibited preoperative hypothalamic pituitary axis dysfunction including central hypothyroidism (35.6%), central diabetes insipidus (29%), growth hormone deficiency (35.6%), adrenocortical insufficiency (23.5%). The Gross total resection was achieved in 6 patients (45%) while subtotal resection was done in remaining 11 patients (55%). Modified Rankin scale score (mRS) on discharge was favorable outcome (mRS 0,1,2,3) in 75% and unfavorable outcome (mRS 4,5) in 19%. There was one mortality. The overall rate of postoperative Diabetes insipidus (DI) was 35.3 % (6/17), visual deterioration (6%), meningitis (11.7%) behavioral disturbances (6%). On 6 month follow up MRI, recurrence was noted in 5 cases, 3(50%) in cases with gross total resection and 2(18.2%) in subtotal resection with adjuvant radiotherapy group. Conclusion: We conclude that Surgical excision of pediatric craniopharyngioma caries a substantial risk of postoperative hypothalamic pituitary dysfunction which significantly impair the quality of life and functional recovery of these patients. A high index suspicion of hypothalamic involvement by tumor and its preservation can improvise patient functional outcome. Postoperative radiotherapy is advised in patient with subtotal resection to improve recurrence free survival.

Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis driven by activating mutations of the MAPK signaling pathway, most commonly BRAF V600E. Pediatric cases are exceedingly rare and often present with atypical, multisystem manifestations, resulting in significant diagnostic delay. Renal and retroperitoneal involvement may mimic primary renal malignancies, particularly Wilms tumor. We report a case of Erdheim–Chester disease in a child treated in Lviv, Ukraine. A male child who initially presented at two years of age with central diabetes insipidus and recurrent urinary tract infections. Over the following two years, the patient developed bilateral obstructive uropathy, retroperitoneal and paravertebral masses, and multifocal central nervous system and orbital involvement, raising concern for Wilms tumor. Recurrent urinary tract infections and progressive hydronephrosis necessitated multiple surgical interventions. Histopathological findings were initially non-specific. Due to clinicoradiologic–pathologic discordance, tissue-based molecular analysis was performed, which identified a pathogenic BRAF V600E mutation, confirming the diagnosis of Erdheim–Chester disease. Targeted therapy with the BRAF inhibitor dabrafenib was initiated, resulting in clinical and radiological stabilization. This case highlights the diagnostic complexity of Erdheim–Chester disease in children and emphasizes the importance of considering histiocytic disorders in the differential diagnosis of renal and retroperitoneal masses. Multisystem involvement, atypical imaging findings, and molecular testing are key elements for accurate diagnosis and appropriate management, particularly in cases mimicking Wilms tumor, as misdiagnosis may lead to inappropriate surgical or chemotherapeutic interventions, delay targeted treatment, and adversely affect prognosis.

Introduction: Pseudo-subarachnoid hemorrhage (PSAH) is a radiographic mimic of subarachnoid hemorrhage (SAH), characterized by symmetrical hyperattenuation of the basal cisterns and subarachnoid spaces on computed tomography (CT). We present a case of PSAH in a patient who developed global anoxic encephalopathy following cardiac arrest. Description: A 24-year-old man presented to an outside hospital after being found unresponsive, apneic, and pulseless. Return of spontaneous circulation was achieved after two cardiopulmonary arrests. Initial CT head interpretation raised concern for diffuse bilateral SAH involving the suprasellar cisterns, prompting transfer to our tertiary center for possible neurosurgical intervention. The patient arrived at our intensive care unit with a Glasgow Coma Scale score of 3T and absent brainstem reflexes. Upon review by in-house neurologists noting no aneurysm on CT angiography, CT findings were determined to be more consistent with PSAH, notable for blurring of grey-white matter differentiation and diffuse sulcal effacement, along with hyperdense skull base/sulcal subarachnoid spaces without intraventricular hyperdensities. A positive fentanyl screen raised suspicion for opioid-induced pulseless electrical activity arrest. Electroencephalography revealed complete isoelectric suppression. Desmopressin was given for central diabetes insipidus and targeted temperature management started. The patient soon exhibited stereotyped decorticate posturing and clonus. Apnea testing was highly suggestive of brain death. Cerebral scintigraphy confirmed absence of intracerebral perfusion, diagnostic of brain death. Discussion: Approximately 3% of all CT findings initially suspected to be SAHs are actually PSAHs. In this vignette, clinical context—presumed opioid overdose with subsequent cardiac arrest—and the radiographic features detailed above, were incredibly important when discerning between PSAH and SAH. Prompt distinction between the two pathologies is crucial as clinical implications and prognoses vary vastly, with SAH being a surgical emergency with the potential for neurologic recovery while PSAH in the setting of anoxic brain injury invariably has a devastating outcome. Early recognition of PSAH can also save medical resources by avoiding transfers to tertiary care centers.

Central diabetes insipidus (CDI) is a rare condition characterized by arginine vasopressin deficiency, leading to persistent polyuria and polydipsia. Although it can present at any age, idiopathic CDI in young adults is uncommon and diagnostically challenging. Inflammatory conditions such as infundibuloneurohypophysitis must be considered, particularly when magnetic resonance imaging (MRI) reveals a thickened pituitary stalk and absence of the posterior pituitary bright spot. Early recognition and differentiation from diabetes mellitus, nephrogenic diabetes insipidus, and primary polydipsia are crucial for effective management. We report the case of a 20-year-old male who presented with excessive urination and thirst for 6 weeks. Physical examination was unremarkable, and the patient had no history of trauma or systemic illness. Urine osmolality was markedly low, and MRI of the brain showed absence of the posterior pituitary bright spot and thickening of the infundibulum, suggestive of infundibuloneurohypophysitis. The patient responded well to desmopressin therapy with symptomatic improvement, thus supporting the diagnosis of CDI. This case highlights the importance of considering idiopathic CDI in young adults presenting with unexplained polyuria and polydipsia. Infundibuloneurohypophysitis should be suspected in patients with characteristic MRI findings even in the absence of overt systemic autoimmune disease. A methodical diagnostic approach, including hormone assays, imaging, and therapeutic trials, can enable early diagnosis and effective long-term management.

Risk Factors for Central Diabetes Insipidus After Pituitary Surgery: Insights from a Multi-Institutional Analysis of 9,290 Patients

Background. Central diabetes insipidus (CDI) is a common complication following transsphenoidal surgery for pituitary adenomas, but CDI as an initial presentation in pituitary adenomas is extremely rare. We report a case of a 67-year-old Filipino male with pituitary macroadenoma presenting as central diabetes insipidus, manifesting as a two-month history of severe frontotemporal headache, increased thirst, and polyuria, which was managed with desmopressin followed by transsphenoidal surgery. Three months postoperatively, the thyroid and adrenocorticotropic axis remained intact, and pituitary bright spot recovery was observed. He was clinically stable; hence, desmopressin was gradually tapered and discontinued. This case report presents a unique case of a pituitary adenoma that initially presented with central DI but later showed a complete resolution of symptoms along with the normalization of the "bright spot" seen on MRI, a hallmark of the posterior pituitary. Treatment options for preoperative CDI may include surgical or medical management, with some cases reported as self-limiting. However, the rarity of such cases underscores the urgent need for more clinical studies to fully understand the course of this condition. This case highlights a unique presentation of central diabetes insipidus in a pituitary macroadenoma and the possibility of complete resolution of symptoms coinciding with pituitary bright spot recovery post operatively. Keywords. central diabetes insipidus, pituitary macroadenoma, pituitary bright spot