Adipsic Diabetes Insipidus Research Articles

7422 Adipsic Diabetes Insipidus Following Pituitary Macroadenoma Resection

Abstract Disclosure: J. Chippior: None. L. Ghalib: None. A 44 year-old female with a PMH of primary hypothyroidism, and a pituitary macroadenoma that was lost to follow up, presented to the ED with a chief complaint of an acute headache and decreased visual acuity involving the right eye over the past year. Patient was found to have a large, homogenous suprasellar mass with the largest dimension measuring 6.3 cm in size, and causing displacement of the optic chiasm. Initial hormonal workup was most notable for elevated prolactin at 128 ng/ml (3.3-26.7 ng/ml), which was slightly lower than when it was previously measured 10 years ago. The patient was taken to the OR for transsphenoidal endoscopic partial resection, but within 24 hours of post-op, she developed hypernatremia at 153 mmol/dL (135-145 mmol/dL) that was suspected to be secondary to diabetes insipidus (DI). In addition, she also suffered several small strokes involving her right midbrain and basal ganglia. She required multiple doses of DDAVP throughout her stay and this was ultimately continued upon discharge. Unfortunately, as follow up MRI had shown residual tumor within the third ventricle, she returned for a 2nd resection but once again suffered additional post-operative complications. The patient experienced additional strokes involving the right frontal and parietal lobes, and her DI became more labile than before, with her serum sodium peaking at 172 mmol/dL. Despite such a high degree of hypernatremia, she did not demonstrate any signs of thirst or any desire to access free water, even after her post-stroke encephalopathy had largely resolved. With less than 100 severe cases documented over the past four decades, it was suspected that the patient had a rare form of DI known as “adipsic DI”. Osmoreceptors within the hypothalamus can trigger the release of ADH or stimulate thirst, with the former requiring a smaller change in serum osmolality to generate such a response. Pituitary surgery involves manipulation of the infundibulum and any inadvertent severing can prevent ADH’s ability to travel from the hypothalamus to the posterior pituitary, subsequently leading to DI. The majority of patients with post-op DI increase their free water intake and subsequently do not develop hypernatremia. Those with adipsic DI lack this compensatory mechanism which leads to the electrolyte disturbance, and carries a higher risk of morbidity and mortality than those with typical DI. Following her second surgery, our patient was discharged with DDAVP once again but this time with a higher dose and frequency. She’s had numerous hospital readmissions since then for altered mental status in the setting of hypernatremia as she still struggles to meet her free water intake and continuously relies on her family for reminders. Though her case is certainly not typical, it highlights the added complexity of managing adipsic DI as it relies not only on DDAVP replacement, but replicating what is supposed to be an innate behavior. Presentation: 6/3/2024

Double trouble: adipsic diabetes insipidus and panhypopituitarism in isolated hypothalamo-pituitary langerhans cell histiocytosis

Double trouble: adipsic diabetes insipidus and panhypopituitarism in isolated hypothalamo-pituitary langerhans cell histiocytosis

Hyponatremia due to preserved non-osmotic arginine vasopressin secretion in adipsic diabetes insipidus: a case report with review of literature.

Adipsic diabetes insipidus (ADI) is characterized by central diabetes insipidus and an impaired thirst response to hyperosmolality, leading to hypernatremia. Hyponatremia observed in patients with ADI has been considered a complication of desmopressin therapy. Herein, we present a case of impaired thirst sensation and arginine vasopressin (AVP) secretion without desmopressin therapy, in which hyponatremia developed due to preserved non-osmotic AVP secretion. A 53-year-old woman with hypopituitarism, receiving hydrocortisone and levothyroxine, experienced hyponatremia three times over 5 months without desmopressin treatment. The first hyponatremic episode (120 mEq/L) was complicated by a urinary tract infection with a plasma AVP level of 33.8 pg/mL. Subsequent hyponatremia episodes occurred after administration of antipsychotic (124 mEq/L) and spontaneously (125 mEq/L) with unsuppressed plasma AVP levels (1.3 and 1.8 pg/mL, respectively). Hypertonic saline infusion did not affect AVP or copeptin levels. Regulating water intake using a sliding scale based on body weight prevented the recurrence of hyponatremia without the use of desmopressin. Except during infection, plasma AVP levels (1.3 ± 0.4 pg/mL) were not significantly correlated with serum sodium levels (rs = -0.04, p = 0.85). In conclusion, we present a unique case of impaired thirst sensation and AVP secretion in which hyponatremia developed without desmopressin therapy. Preserved non-osmotic AVP secretion, possibly stimulated by glucocorticoid deficiency, may contribute to the development of hyponatremia in patients with ADI.

An exhaustive battle of salt: A challenging case of Adipsic Diabetes Insipidus following resection of Craniopharyngioma

Introduction: Even though rare, Adipsic Diabetes Insipidus (ADI) is a potentially life-threatening complication of craniopharyngioma: due to the mass effect of the tumor or perioperative consequences leading to severe hypernatremic dehydration. Even in expert hands, management of water balance, in the absence of thirst is extremely challenging. Case Description: A 25-year-old gentleman, an electrician presented to the neurosurgical casualty with worsening headaches, impaired vision, and features of raised intracranial pressure over 2 months. He was found to have a large cystic suprasellar mass with well-defined margins measuring 2.7cm x 2.7cm x 3cm with chiasmal compression, suggestive of craniopharyngioma, complicating with bitemporal hemianopia, hypogonadotropic hypogonadism (LH 0.78IU/L, FSH 1.2IU/L, Testosterone 33ng/dL), secondary hypothyroidism, cranial diabetes insipidus, prediabetes sparing the cortisol axis. He underwent transcranial resection of the tumor, revealing WHO grade1 adamantinomatous craniopharyngioma followed by radiotherapy. The post-operative period was complicated with severe hypernatremia despite escalating doses and changing dosage forms of desmopressin from parenteral to nasal spray to oral tablets. Two weeks following discharge, he presented with severe hypernatremia (176mEq/L), polyuria, weight loss, hypotension, and altered sensorium where his lack of thirst was appreciated. Conclusions: In those with ADI, for the management of water balance, a “personalized approach” with the involvement of both patient and family is of paramount importance. With appropriate dose titration of desmopressin, careful fluid prescription, and frequent monitoring of electrolytes even at best centers, it is a battle that is hard to win.

Abstract #1404147: Adipsic Diabetes Insipidus in a Patient with Aggressive Suprasellar Mature Teratoma

Diabetes insipidus (DI) can be life-threatening if not properly diagnosed and managed. There are many etiologies of central diabetes insipidus including neoplasm, vascular disease, trauma, autoimmune disease, infection, granulomatous disease, drugs, and genetic disease. This report presents a patient who developed adipsic DI secondary to a rare etiology, a suprasellar mature teratoma. A 37-year-old man with no significant past medical history presented to the hospital for worsening altered mental status (AMS) for 5 months, headaches, and polydipsia. Hypernatremia with a sodium level of 175 was noted. Magnetic resonance imaging (MRI) of the brain showed a large, heterogenous, mixed solid cystic suprasellar mass measuring 3.4 x 2.6 x 2.7 cm invading the suprasellar structures and extending to the third ventricle with mass effect on the hypothalamus, thalami, and optic chiasm. Panhypopituitarism with DI, hypothyroidism, adrenal insufficiency, and hypogonadism was diagnosed. Open craniotomy for surgical resection of the mass was performed. Pathology revealed endodermal tissues representing small and large intestine and stratified keratinizing and nonkeratinizing squamous epithelium in a background of hemorrhage, fibrosis, chronic inflammation, and mucin consistent with a suprasellar mature teratoma. He was discharged on desmopressin 1 mcg subcutaneously twice daily, hydrocortisone, and levothyroxine. He presented 7 months later for AMS and severe hypernatremia on desmopressin 6 mcg subcutaneously twice daily. There was regrowth of the suprasellar mass with interval worsening in size (3.5 x 3.7 x 3.1 cm) and increased mass effect on the left internal carotid artery, anterior communicating artery, and third ventricle. Subsequently, he underwent endoscopic cyst fenestration and ventriculoperitoneal shunt placement. Panhypopituitarism persisted, and he remained obtunded. He was hospitalized for 2 months, and repeat MRI of the brain showed an interval increase in the size of the mass (4.7 x 3.6 x 3.3 cm) with surrounding vasogenic edema and mass effect on the optic chiasm, hypothalamus, and third ventricle concerning for right ventricular entrapment. Due to the aggressive nature of the tumor, it was deemed inoperable and the patient underwent comfort care measures. Suprasellar mature teratomas are rare tumors which respond to surgical resection with a 10-year survival rate of 93%. Hypothalamic damage from these tumors can lead to an inability to maintain thirst response which can be dangerous in central DI as severe hypernatremia can develop. In these patients, it is vital to evaluate for other pituitary hormone deficiencies.

LBMON139 A Case of Adipsic Diabetes Insipidus In Patient With Third Ventricular Tumor And Right Frontal Craniotomy.

Abstract Introduction Adipsic diabetes insipidus (ADI) is a rare hypothalamic disorder consisting of central diabetes insipidus (CDI) and loss of thirst. In hyperosmolar state, signals from osmosensors integrate in the central thirst center resulting in stimulatory signals between subfornical-oragan, organum-vasculosm of lamina terminalis and median preoptic nucleus increasing thirst and release of vasopressin. Disruption in this mechanism leads to adipsia. Case presentation 27 yr old male with history of prediabetes, 4.6cm third ventricular epidermoid cyst s/p endoscopic ventriculosmoy, hypogonadotropic hypogonadism, central hypothyroidism, and CDI underwent right frontal craniotomy for residual third ventricular cyst. Developed worsening DI, new onset hyperglycemia, and secondary adrenal insufficiency. Discharged with oral DDAVP 300mcg oral twice daily for CDI. 1 week later presented to ED with weakness. BP 132/112, HR 139, RR 38, SPO2 94%. Labs significant for corrected sodium of 172 (136-145mmol/L), glucose 699 (70-99mg/dl), calcium 11.8 (8.3-10.6 mg/dl). Discharged after 3 weeks with intranasal DDAVP 10mcg twice daily (BID). Again presented 1 week after for weakness. Vitals BP 105/64, HR 134, F 37.3 C, RR 31, SPO2 96%. Corrected sodium of 174 (136-145mmol/L) and glucose 708 (70-99mg/dl). Denied missing medication. Reported no increased water intake. Patient was diagnosed with adipsic DI based on subjective lack of thirst during period of significant hyperosmolar hypernatremia state. Overtime switched to 10mcg BID intranasal DDAVP and started on 4L fixed water intake. Had 5L urine output daily. Sodium levels ranged from 132-137. Discharged on intranasal DDAVP 10mcg AM/20mcg PM, 4L daily fixed water intake, and twice weekly sodium checks as outpatient. Goal sodium 135-140. DDAVP adjusted after sodium rose to 145. At recent clinic visit continued fixed 4L fluid intake with extra 500-1L intake for increased sweating/exercise, and sodium level 134. A1c 10% on insulin, and weight gain of 70lbs since surgery (8mon piror), BMI of 45. Started on GLP-1RA and sleep study. Learning points: Less than 100 cases of ADI reported worldwide and associated with increased morbidity/mortality1. This patient likely developed ADI due to disruption of central thirst center from epidermoid cyst and trans frontal craniotomy. We must be hypervigilant for ADI in patients with third ventricular tumors who undergo frontal craniotomy. Management is complex and requires patients are followed closely with weekly labs, either fixed water intake or fixed DDAVP dose and adjustment depending on labs. Important to screen these patients for obesity and sleep apnea given increased likelihood of hypothalamic dysfunction1. Reference: Cuesta, M., Hannon, M. J. & Thompson, C. J. Adipsic diabetes insipidus in adult patients. Pituitary 20, 372-380 (2017). https://doi.org/10.1007/s11102-016-0784-4 Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Relationship between postoperative hypothalamic injury and water and sodium disturbance in patients with craniopharyngioma: A retrospective study of 178 cases.

ObjectiveTo investigate the relationship between postoperative hypothalamo–hypophyseal injury (HHI) and postoperative water and sodium disturbances in patients with craniopharyngioma.MethodsThe medical records, radiological data, and laboratory results of 178 patients (44 children and 134 adults) who underwent microsurgery for craniopharyngioma in a single center were reviewed. Postoperative HHI was assessed using magnetic resonance imaging. Structural defects of the hypothalamo–hypophyseal system (pituitary, pituitary stalk, floor and lateral wall of the third ventricle) were assessed in four standard T1-weighted images. The defect of each structure was assigned 1 score (0.5 for the unilateral injury of the third ventricle wall), and a HHI score was calculated.ResultsThe number of patients with HHI scores of 0-1, 2, 2.5-3, and >3 was 35, 49, 61, and 33, respectively. Diabetes insipidus (DI) worsened in 56 (31.5%) patients with preoperative DI, while 119 (66.9%) patients were diagnosed with new-onset DI. Hypernatremia and hyponatremia developed in 127 (71.3%) and 128 (71.9%) patients after surgery, respectively. Syndrome of inappropriate antidiuresis occurred in 97(54.5%) patients. During hospitalization, hypernatremia recurred in 33 (18.5%) patients and in 54 (35.7%) during follow-up, of which 18 (11.9%) were severe. DI persisted in 140 (78.7%) patients before discharge. No relationship was found between the HHI score and incidence of early DI, hyponatremia, syndrome of inappropriate diuretic hormone, or prolonged DI. Compared with patients with a score of 0–1, those with scores =2.5-3 (OR = 5.289, 95% CI:1.098-25.477, P = 0.038) and >3 (OR = 10.815, 95% CI:2.148-54.457, P = 0.004) had higher risk of developing recurrent hypernatremia. Patients with a score >3 had higher risk of developing severe hypernatremia during hospitalization (OR = 15.487, 95% CI:1.852-129.539, P = 0.011) and at follow-up (OR = 28.637, 95% CI:3.060-267.981, P = 0.003).ConclusionsThe neuroimaging scoring scale is a simple tool to semi-quantify HHI after surgery. Recurrent and severe hypernatremia should be considered in patients with a high HHI score (>2.5). An HHI score >3 is a potential predictor of adipsic DI development. Preventive efforts should be implemented in the perioperative period to reduce the incidence of potentially catastrophic complications.

Diagnosis and Management of Central Diabetes Insipidus in Adults.

Central diabetes insipidus (CDI) is a clinical syndrome which results from loss or impaired function of vasopressinergic neurons in the hypothalamus/posterior pituitary, resulting in impaired synthesis and/or secretion of arginine vasopressin (AVP). AVP deficiency leads to the inability to concentrate urine and excessive renal water losses, resulting in a clinical syndrome of hypotonic polyuria with compensatory thirst. CDI is caused by diverse etiologies, although it typically develops due to neoplastic, traumatic, or autoimmune destruction of AVP-synthesizing/secreting neurons. This review focuses on the diagnosis and management of CDI, providing insights into the physiological disturbances underpinning the syndrome. Recent developments in diagnostic techniques, particularly the development of the copeptin assay, have improved accuracy and acceptability of the diagnostic approach to the hypotonic polyuria syndrome. We discuss the management of CDI with particular emphasis on management of fluid intake and pharmacological replacement of AVP. Specific clinical syndromes such as adipsic diabetes insipidus and diabetes insipidus in pregnancy as well as management of the perioperative patient with diabetes insipidus are also discussed.

Adipsic diabetes insipidus and SGLT2 inhibitor: A perplexing conundrum

Adipsic diabetes insipidus and SGLT2 inhibitor: A perplexing conundrum

Chapter 19 - Adipsic diabetes insipidus

Adipsic diabetes insipidus (ADI) is a rare but devastating disorder of water balance with significant associated morbidity and mortality. Most patients develop the disease as a result of hypothalamic destruction from a variety of underlying etiologies. Damage to osmolar-responsive neuroreceptors, primarily within the supraoptic and paraventricular nuclei, results in impaired production and release of arginine vasopressin (AVP). Important regulating circuits of thirst sense and drive are regionally colocalized with AVP centers and therefore are also injured. Patients with central diabetes insipidus with impaired thirst response, defined as ADI, suffer from wide swings of plasma osmolality resulting in repeated hospitalization, numerous associated comorbidities, and significant mortality. Treatment recommendations are based largely on expert advice from case series owing to the rarity of disease prevalence. Acute disease management focuses on fixed dosing of antidiuretic hormone analogues and calculated prescriptions of obligate daily water intake. Long-term care requires patient/family education, frequent reassessment of clinical and biochemical parameters, as well as screening and treatment of comorbidities.

Adipsic diabetes insipidus patient in postoperative pituitary macroadenoma.

Adipsic diabetes insipidus is a rare condition secondary to injury to osmoreceptors in the anterior hypothalamic area. Only two cases have been published secondary to pituitary tumor surgery. A 43-year-old man, postoperative of a non-functioning pituitary macroadenoma invading the third ventricle and compressing the hypothalamus. Reoperated for headache and rhinorrhachia, developing diabetes insipidus in the postoperative period was discharged with 20 μg/day nasal desmopressin. He came again due to sensorial disorder and hypernatremia, managing to control with intravenous hydration and desmopressin. It presents with recurrence of hypernatremia every time intravenous hydration is suspended and taken orally. With high sodium levels, there is an absence of thirst. A diagnosis of adipsic diabetes insipidus is made, indicating supervised administration of water orally with favorable evolution. Adipsic diabetes insipidus is a rare variant of central diabetes insipidus caused by damage to osmoreceptors in the hypothalamus. It manifests with absence of perception of thirst, hypernatremia and polyuria. Its management is complex and requires strict control of the water balance and adherence to treatment.

SUN-284 Rare Case of Adipsic Diabetes Insipidus Post Astrocytoma Resection: Case Report

Background:Adipsic diabetes insipidus (ADI) is a rare disorder triggered by injury to the hypothalamus impairing thirst osmoreceptors and the antidiuretic hormone syntheses leading to absence of thirst.We are reporting a rare case of ADI Post astrocytoma resection who presented with a severe hypernatremia postoperatively.Clinical Case:A 16-year-old female known to have hypothalamic pilocystic astrocytoma post incomplete resection in 2014.She presented in 2018 with one week history of sudden onset heachache, progressively worsen overtime, worst in the morning, there were no relieving factors. On examination she was conscious alert oriented GCS 15/15, Vital signs were normal, fundus exam within normal, pupil equal bilateral reactive to light, there was no focal neurological deficit. Systemic examination was unremarkable. Radiological imaging revealed interval size progression of previously seen suprasella region with extension into the floor of the third ventricle. Investigations: Renal profile & pituitary hormonal profile were normal. She underwent second stage resection.Postoperatively, she developed asymptomatic hypernatremia with inability to sense thirst. On examination: Conscious, alert, BP:100/65, no postural drop, Pulse:95, Fluid balance negative 1L. Systemic examination was unremarkable. Laboratory investigations: Creat 0.77mg/dl (0.6–1), Na 173mEq/dl(136–145), K 3.3mEq/dl(3.5–5.1), Cl 134mEq/dl(98–107), Serum osmolality 363mOs/kg(285 -295), Urine osmolality 468mOs/kg(300 -1600), Urine Na 29mEq/L(20- 110) & Urine volume 1900. Diagnosis of ADI was made where she was treated with desmopressin. Her response was good with reduction in Na level of around 7- 9 mEq/L/Day. She was maintained on twice daily vasopressin, instructed to maintain her fluid intake 1–1.5 L/day and good urine output.Conclusions:Astrocytoma rarely linked with development of central diabetes insipidus and even fewer with adipsia. The absence of polydipsia and polyuria can be misleading. Long-term therapy should consider the benefits of vasopressin and fluid therapy against the risk of brain edema/herniation. Body weight and electrolytes monitoring essential & should be maintained a long with strict fluid intake.

Abstract #805605: Adipsic Diabetes Insipidus in a Patient with a Non-Functioning Pituitary Macroadenoma

Abstract #805605: Adipsic Diabetes Insipidus in a Patient with a Non-Functioning Pituitary Macroadenoma

SUN-407 A Clinical Lesson in Adipsic Diabetes Insipidus

Background: Adipsic diabetes insipidus (ADI) is a rare condition with approximately 100 reported cases word wide. Inpatient management can be challenging as patients are predisposed to dysnatremia during this time. Societal guidelines offer little guidance in management of this complex disease. Case: A 62-year-old woman with a history of acromegaly with transphenoidal resection more than 20 years ago, complicated by post-operative hypothyroidism, adrenal insufficiency and adipsic diabetes insipidus presented for admission for a planned cycle on of chemotherapy for her recently diagnosed diffuse large B-cell lymphoma. At admission her initial serum sodium was 130 mmol/L (reference 135-145 mmol/L) near her baseline level. During her 8 day stay she experienced significant fluctuations of serum sodium with a peak level of 151 mmol/L and a nadir of 123 mmol/L. Despite these shifts she experienced no adverse neurological symptoms. 3 weeks later she was again admitted for planned chemotherapy. During this admission she received the same medication treatment protocol as her prior stay, however, the management principles for ADI suggested by Cuesta et al. were applied. The result was a more stable serum sodium with a range of 130-145 mmol/L and no change in concentration by more than 5 mmol/L in a single 24 hour period. Patient was clinically stable and asymptomatic throughout her hospital stay Discussion: When compared to diabetes insipidus with intact thirst sensation, patients with ADI have been shown to be at increased risk for both hypernatremia and hyponatremia during inpatient admission. The risk is further increased when the admission is for an illness not related to pituitary dysfunction. This case represents an important clinical lesson in ADI where simple management principles led to a drastic change in the patient’s serum sodium. Fortunately, this patient tolerated the sodium shifts well, and did not require critical care interventions to correct her dysnatremia. Among the most important principles were transition from intranasal to oral DDAVP for a more predictable treatment response and the use of oral fluids instead of IV whenever possible. Applying these principles at the time of admission allowed for a better understanding of the treatment goals by the multidisciplinary healthcare team. Although ADI is rare, applying these simple interventions at the time of admission is likely to benefit all patients with diabetes insipidus.

SAT-484 Pituitary Microadenoma and Cerebral Venous Thrombosis in a Patient with Transient Adipsic Diabetes Insipidus after Treatment for Craniopharyngioma

Background Craniopharyngioma is a rare embryonic sellar malformation, that can present with headache, motor and cranial nerve palsies. Treatment of large masses involves surgical excision followed by radiation therapy. Hypopituitarism and diabetes insipidus are some of the post-operative complications. Rarely, patients may develop inability to appreciate fluid losses, resulting in marked hypernatremia and dehydration associated with life-threatening lower extremity thrombosis and pulmonary embolism. We present a patient developing cerebral venous thrombosis resulting from transient adipsic diabetes insipidus accompanied by new-onset pituitary microadenoma. Clinical A 39-year-old female post-excision and radiation therapy for craniopharyngioma developed hypopituitarism and diabetes insipidus. Three months after initiating radiotherapy, she presented with headache and inability to sense thirst. She took desmopressin 100 mcg tablet only three times for the past month. There was hypernatremia at 154 meq/L (Normal Value 135-145 meq/L) and hyperprolactinemia at 235 ng/mL (NV 5.11-26.19 ng/ml). Contrast-enhanced MRI revealed cerebral venous thrombosis and a pituitary microadenoma. Thrombosis has been attributed to plasma volume contraction and consequent plasma hyperviscosity. She was started on enoxaparin, hydration with hypotonic solution, desmopressin 100 mcg tablet and bromocriptine 2.5 mg tablet for the possibility of new-onset prolactin secreting pituitary microadenoma. There was decrease in headache severity and normalization of sodium level. She was discharged on apixaban 2.5 mg tablet twice a day, bromocriptine, levothyroxine 100 mcg tab daily, desmopressin daily or at least twice a week and hydrocortisone 20 mg tab daily. After three months, she had increased ability to sense thirst but still had occasional headaches. Imaging showed partial resolution of thrombosis. Prolactin decreased to 0.925 ng/mL (NV 3-20 ng/ml). In general, prolactinomas have serum prolactin that parallel tumor size. Rarely, minimal prolactin elevations may be consistent with a prolactinoma, but a non-prolactin secreting mass should first be considered in the presence of a microadenoma. The initially elevated prolactin has been attributed to disruption of dopamine inhibition to the pituitary lactotrophs. Serial imaging of the pituitary microadenoma appears to be a reasonable follow-up. Prompt evaluation must be done should there be any signs and symptoms of hormonal hypersecretion or if there is increase in size of the microadenoma. Conclusion This is the first case to demonstrate cerebral venous thrombosis and new-onset pituitary microadenoma in a patient developing transient adipsic diabetes insipidus after treatment for craniopharyngioma. These entities must be included in the workup of patients presenting with headache. No relevant disclosures.

A Rare Case of Adipsic Diabetes Insipidus Associated with Langerhans Cell Histiocytosis

Objective: We describe a case of adipsic diabetes insipidus, hypopituitarism, and cognitive impairment in a patient with a hypothalamic mass diagnosed with Langerhans cell histiocytosis. We aim to ...

Adipsic Diabetes Insipidus: A Single-Center Case Series

: Adipsic diabetes insipidus (ADI) is a rare and challenging complication secondary to several neurosurgical procedures. It can lead to severe hyperosmolarity and is associated with high rates of morbidity and mortality. Considering the variable time of thirst sensation recovery, long-term treatment is accompanied with the risk of extreme extracellular osmolality fluctuations. Herein, we report 3 cases of this rare presentation of hypothalamic injury.

Adipsic diabetes insipidus revealing a bifocal intracranial germinoma

Adipsic diabetes insipidus is a rare complication of intracranial tumors in which impaired antidiuretic hormone secretion is associated with the loss of thirst sensation. Here, we present the case of a patient with bifocal intracranial germinoma, diagnosed due to symptoms mainly caused by adipsic diabetes insipidus. This is, to our knowledge, the first case of adipsic diabetes insipidus revealing an intracranial germinoma reported in the literature. We describe the diagnostic procedures and the three-year follow-up of this patient. Management of intracranial germ-cell tumors is made complex by the wide range of histological features. Although germinomas have a generally better prognosis than most nongerminomatous tumors, they can have severe or even life-threatening presentations. Adipsic diabetes insipidus is one such severe presentation and its rarity can make it difficult to recognize and manage. Awareness of this potential entity is therefore important for clinical practice.

Adipsic diabetes insipidus in adult patients.

Adipsic diabetes insipidus (ADI) is a very rare disorder, characterized by hypotonic polyuria due to arginine vasopressin (AVP) deficiency and failure to generate the sensation of thirst in response to hypernatraemia. As the sensation of thirst is the key homeostatic mechanism that prevents hypernatraemic dehydration in patients with untreated diabetes insipidus (DI), adipsia leads to failure to respond to aquaresis with appropriate fluid intake. This predisposes to the development of significant hypernatraemia, which is the typical biochemical manifestation of adipsic DI. A literature search was performed to review the background, etiology, management and associated complications of this rare condition. ADI has been reported to occur in association with clipping of an anterior communicating artery aneurysm following subarachnoid haemorrhage, major hypothalamic surgery, traumatic brain injury and toluene exposure among other conditions. Management is very difficult and patients are prone to marked changes in plasma sodium concentration, in particular to the development of severe hypernatraemia. Associated hypothalamic disorders, such as severe obesity, sleep apnoea and thermoregulatory disorders are often observed in patients with ADI. The management of ADI is challenging and is associated with significant morbidity and mortality. Prognosis is variable; hypothalamic complications lead to early death in some patients, but recent reports highlight the possibility of recovery of thirst.

Bifocal intracranial germinoma presenting as adipsic diabetes insipidus

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