EXPRESS: Presumed Partial Aplasia of the Hypothalamus and Pituitary Gland Causing Central Diabetes Insipidus in an Adult Cat

88. New onset central diabetes insipidus in pediatric scoliosis fusions utilizing tranexamic acid

Case summaryA 1-year-old neutered male domestic shorthair cat was presented for polyuria and polydipsia which had progressed since adoption, 7 months previously. On admission, clinical examination did not reveal any remarkable features. Urinalysis showed marked hyposthenuria and calculated plasma osmolality was high, suggesting diabetes insipidus. A positive response to desmopressin administration appeared to confirm pituitary dysfunction. Brain MRI revealed a lesion compatible with a cyst or a neoplasm compressing the pituitary gland. A follow-up MRI performed 9 months later showed that the lesion was stable, which at first argued in favour of a congenital pituitary cyst. Intranasal administration of desmopressin was then used to achieve a long-term clinical response.Relevance and novel informationCentral diabetes insipidus (CDI) is a rare cause of polyuria and polydipsia in cats, resulting from inadequate or impaired secretion of antidiuretic hormone from the posterior pituitary gland. Recognised causes include head trauma, central nervous system (CNS) neoplasia, idiopathic CDI and congenital pituitary cysts. Apart from one cat with CNS lymphoma, the few previously reported feline cases have described CDI in young cats with a previous history of trauma, but brain imaging has rarely been performed to look for underlying anatomical abnormalities. This report describes the first case of CDI in a cat with a confirmed congenital pituitary cyst and, as in previous cases, demonstrates successful treatment with desmopressin.

A case of central diabetes insipidus after ketamine infusion during an external to internal carotid artery bypass

Cats are animals with highly efficient reproduction, clearly pointing to a need for suppression of fertility. Although surgical contraception is highly effective, it is not always the method of choice. This is predominantly because it is cost-intensive, time-consuming and irreversible, with the latter being of major importance for cat breeders. This article reviews the use of progestins, scleroting agents, immunocontraception, melatonin, GnRH antagonists and finally, GnRH agonists, in adult male and female cats in detail, according to the present state of the art. By now, various scientific and clinical options are available for the suppression of fertility in adult cats and the decision as to which should be chosen - independent of the legal registration of any state - depends on different facts: (i) feral or privately owned animal? (ii) temporary or permanent suppression of fertility wanted/needed? (iii) sex of the animal? New effective and available methods for hormonal contraception include melatonin implants for short-term post ponement of oestrus in adult queens and slow-release GnRH-agonist implants containing deslorelin (Suprelorin(®) ) for short- and long-term contraception in male and female companion and breeding cats.

Epidemiological data for central diabetes insipidus (CDI) are sparse. The purpose of this study was to provide accurate epidemiological data on CDI on a national level. This was a drug utilization and patient registry study during a 5-year period from 2007 to 2011. We used the Danish National Prescription Registry data linked with the Danish National Patient Registry to study the epidemiology of CDI using waiting time distribution and other pharmacoepidemiological methods. A total of 1285 patients with CDI were recorded in the observation period and given 9309 prescriptions for desmopressin in the nasal formulation, orodispersible tablet, or conventional tablet. The period prevalence rate of CDI in Denmark over the 5-year period investigated was 23 CDI patients per 100 000 inhabitants, with a higher prevalence in children and older adults (>80 years of age). The 1-year period prevalence rate of CDI decreased in Denmark over the 5 years from approximately 10 to 7 CDI patients per 100 000 inhabitants. The yearly incidence rate of new cases of CDI was found to be 3 to 4 patients per 100 000. The incidence of (presumable) congenital CDI was found to be 2 infants per 100 000 infants. Half of the patients with CDI prescribed as oral treatment were provided dosing instructions to only administer the drug before bedtime, and one third of the CDI patients either had no specific instructions or were instructed to use the drug as needed. Hospital admissions due to severe hyponatremia occurred in 0.9% of patients over a 5-year period, predominantly in females with an incidence ratio of women to men of 1.8:1. Half of the cases of CDI are acquired later in life. At least half of the patients with CDI are instructed to prevent nocturnal polyuria, but it is not clear whether their CDI remains uncontrolled during the daytime or, alternatively, whether they use desmopressin only as needed. Female patients with CDI had approximately twice the number of hospital admissions due to severe hyponatremia than male patients with CDI.

Introduction: Central Diabetes Insipidus (CDI) is the lack of antidiuretic hormone (ADH) leading to impaired urinary concentration and manifests with extreme thirst and excessive urination. Patients unable to drink fluids are at risk of severe dehydration and hypernatremia. CDI can be a rare complication of acute myeloid leukemia (AML). The occurrence of AML with CDI is extremely rare with only few case reports published. This combination of AML with CDI has been reported to be associated with monosomy 7 and inversion (3) (q21q26) and portends an overall a very poor treatment response resulting in poor outcomes.Case Presentation: 66-year-old female with hypertension, polycythemia vera diagnosed in 2006 with transformation to AML in 2020. FISH studies revealed monosomy 7. Cytogenetic studies showed inv (3)(q21q26.2). Remission induction chemotherapy was initiated. Subsequently, neutropenic fever and sepsis secondary to Clostridium difficile colitis lead to hospitalization. Her sodium (Na) level gradually trended up and reached a peak of 157 mmol/l (range 136-145) and elevated serum osmolality at 311 mOsm/K with low urine osmolality 135 mOsm/K, low urine sodium at 13 and low urine specific gravity 1.006 concerning for CDI. She developed polyuria and received desmopressin (DDAVP) leading to improvements in urine osmolality to 267 mOsm/K, 30 minutes indicating CDI diagnosis. Her Na gradually normalized to 144 mmol/l, urine osmolality improved to 580 mOsm/K and urine specific gravity to 1.025. She is now on DDAVP 0.05 mg oral twice daily and her Na is in normal range. MRI pituitary did not show any evidence of metastatic lesion with intact pituitary bright spot. Her other pituitary hormonal workup was normal except for hypogonadotropic hypogonadism.Discussion: The pathophysiology of AML and CDI is unclear. Leukemic cells infiltration of the neurohypophysis; thrombosis of small vessels in hypothalamic nuclei and the posterior pituitary; alterations of the neutrophil migration placed on the chromosome 7 leading to glycoprotein gp 130 production, a cell surface marker on granulocytes are some of hypothesis suggested. CDI has a variable onset in the course of myeloid malignancies. MRI pituitary can be normal in most of the cases.

Background: Central diabetes insipidus (CDI) as a complication of acute myeloid leukemia (AML) is rare, occurring in less than 0.6% of AML cases. The mechanism is thought to involve leukemic infiltration in or around the pituitary gland, not always seen on imaging. In one study, as many as 61.4% of patients with CDI due to AML had no abnormalities on MRI, and at autopsy 46% of AML patients had perihypohyseal leukemic infiltration in the absence of overt CDI. CDI is also associated with AML in cases that involve monosomy 7 and inversion 3q21q26, both of which result in ectopic viral integration site 1 (EVI-1) overexpression. It is postulated that EVI-1 overexpression interferes with hypothalamic secretion of antidiuretic hormone (ADH) or may lead to its inactivation. We present a case of adipsic CDI due to AML in a patient with monosomy 7.Case: A 70-year-old female presented for routine follow-up and was found to have a white blood cell count of 2.6 K/µL with 29% blasts, anemia (Hgb 10.3 g/dL) and normal platelets (300 K/µL). She was diagnosed with AML and molecular evaluation showed del (3)(q21),-7,add(17(p13) consistent with monosomy 7. She was admitted for induction chemotherapy with cytarabine, daunorubicin and intrathecal methotrexate. She denied thirst. On physical exam she was euvolemic and visual fields were full on confrontation. Her admission sodium was 146 mmol/L, urine osmolality was 149 mOsm/kg H2O, urine sodium 14 mmol/L. Urine output was 5.1 L over the first 24 hours. She underwent a 6 hour water deprivation test, during which her urine output averaged 250 cc/hr. Her sodium increased to 158 mmol/L, serum osmolality 331 mOsm/kg H2O, urine osmolality 146 mOsm/kg H2O. She was then administered 100 µg of DDAVP PO and her serum sodium and osmolality decreased to 155 mmol/L and 326 mOsm/kg H2O, respectively, while her urine osmolality nearly doubled to 292 mOsm/kg H2O. Urine output decreased to 50-100 cc/hr. At no point during her testing did she report thirst. The patient’s pituitary laboratory profile did not show any other abnormalities. Her pituitary MRI revealed subtle thickening of the proximal infundibulum and hypothalamus but no definitive intra-sellar pathology. She was discharged on twice daily DDAVP with a sodium of 142. Unfortunately, her AML was refractory to treatment. She was transitioned to comfort care and died peacefully.Conclusion: CDI as a complication of AML is very rare and is a poor prognostic marker. Based on her MRI findings, the most likely mechanism in this case was infundibular/hypothalamic infiltration. Her adypsia is interesting and may point to more generalized hypothalamic involvement including thirst center. Her monosomy 7 mutation may have also played a role. We present this case to bring awareness to this etiology of DI and its proposed mechanisms.

Patient: Female, 31-year-oldFinal Diagnosis: Central diabetes insipidusSymptoms: Polydipsia • polyuriaMedication: —Clinical Procedure: —Specialty: Endocrinology and MetabolicObjective:Unusual clinical courseBackground:Nephrogenic diabetes insipidus is a well-known adverse effect of lithium use. Albeit rare, there have also been documented cases of central diabetes insipidus (CDI) associated with lithium use.Case Report:A 31-year-old woman with a past medical history of bipolar disorder, managed with lithium 300 mg by mouth every day for 3 years, was assessed for a 1-year history of polyuria with accompanying polydipsia. During her initial hospital stay, her estimated urine output was more than 4 L per day. Initial labs showed elevated serum sodium (149 mmol/L; reference range 135–145), elevated serum osmolality (304 mOsm/kg; reference range 275–295), urine osmolality of 99 mOsm/kg (reference range 50–1200), and urine specific gravity (1.005; reference range 1.005–1.030). Lithium was at a subtherapeutic level of 0.05 mEq/L (reference range 0.6–1.2). Magnetic resonance imaging of the brain revealed no abnormalities of the pituitary gland. Two different occasions of desmopressin administration resulted in >50% increase in urine osmolality, confirming the diagnosis of CDI. Common causes of CDI, including trauma, tumors, and familial CDI, were ruled out and chronic lithium use was determined as the most probable cause for the patient’s CDI.Conclusions:CDI in the background of chronic lithium use is rarely reported. We present this case to consider CDI as a differential diagnosis when evaluating polyuria and hypernatremia in patients with long-term lithium use. These presentations warrant the consideration of both types of diabetes insipidus in the differential diagnoses.

BACKGROUND Central diabetes insipidus (CDI) is a rare disorder characterized by large volumes of dilute urine because of a lack of antidiuretic hormone. Co-existing CDI and diabetes mellitus without inherited disorders such as Wolfram syndrome are rare. It is both important and challenging to diagnose this combination because the 2 conditions present with thirst, polydipsia, and polyuria. A few cases of CDI developing in patients with type 2 diabetes mellitus (T2D) have been reported. We report an unusual case of CDI that developed in an older patient with T2D. The aims of this report are to share the clinical course and discuss clues to the early diagnosis of CDI in T2D. CASE REPORT A 70-year-old Japanese woman developed T2D with hyperglycemia symptoms, including thirst, polydipsia, and polyuria. After starting medical treatment, the hyperglycemia and its symptoms improved. The glycated hemoglobin level decreased from 9% to 6%. However, 5 years later (at 75 years of age), she re-exhibited thirst, polydipsia, and polyuria despite stable glycemic control. Her urine volume was large (6.3 L/day). A urine glucose test was negative. The plasma osmolality was high (321 mOsm/kg), while the urinary osmolality was low (125 mOsm/kg). A significant increase in urinary osmolality following vasopressin administration indicated a diagnosis of CDI. Desmopressin therapy effectively relieved the symptoms. CONCLUSIONS This case highlights the need to consider CDI as a rare but important comorbid disorder in patients with diabetes mellitus, including T2D, particularly those presenting with thirst, polydipsia, and polyuria despite well-controlled glycemia.

Case report. To present a case of central diabetes insipidus (CDI) that developed after a gunshot injury to the thorax and thoracic spinal cord and to discuss the disease process in light of the relevant literature. Antidiuretic hormone (ADH) abnormalities may develop after spinal trauma and/or surgery. Although there are published reports of inappropriate ADH syndrome arising in this clinical picture, CDI is rare. A 33-year-old woman with hemopneumothorax and a gunshot wound to her thoracic spine was treated with chest tube drainage. No surgery was performed for the spinal injury. The patient was paraplegic on admission and rapidly developed excessive urine output. Testing revealed that her serum ADH level was low, consistent with CDI. Desmopressin acetate nasal spray was the prescribed treatment. The patient responded well to the desmopressin acetate spray. CDI is a complicated hormonal disorder characterized by excessive urine output. It is typically linked to an abnormality in the hypothalamohypophyseal axis that markedly reduces ADH production. The most common inciting causes are craniocerebral trauma, brain tumor and/or surgery, and central nervous system infection. Although uncommon, CDI should be considered when a spinal trauma patient develops excessive urine output.

Background Metastasis to the pituitary gland is rare encounter and is more common amongst the elderly population with advanced malignancy. An estimated 1% of all pituitary tumour resections are metastatic. Primary sites that frequently metastasize include breast and lung carcinomas. In the recent decade, advancement in the field of oncology with multiple modalities of therapy has led to prolonged survival of patients with advanced stages of malignancy.Herein, we present three cases and review of literature of pituitary metastasis presenting as central diabetes insipidus incidentally unmasked following administration of corticosteroids. Objective To establish the common clinical features, establish variations in clinical presentations and natural progression of disease in patients with pituitary metastasis. Methods Three cases of central diabetes insipidus unmasked by corticostetoroids in pituitary metastasis were presented. A total of 9 other cases with central diabetes insipidus as first clinical manifestation unmasked by corticosteroid published from 2007-2018 were reviewed. Pertinent references were searched using windows remote search model on PubMed. Non-English articles were excluded. Results A compilation of 9 previously reported cases of central DI unmasked by corticosteroids from 2007 to 2017 along with the present 3 cases were performed (Table 1). There was equal gender prevalence with a mean age of 61 (range 56-80 years old). More than 75% of the cases described here had previously been diagnosed with advanced malignancies of varying primary sites. The remaining 25% presented with varying symptoms of hypopituitarism as the harbinger to the discovery of the primary neoplasm. Amongst the literature review and cases presented, primary malignancies with pituitary metastasis included lung adenocarcinoma (33%), breast carcinoma (25%), nasopharyngeal carcinoma (16%), renal cell carcinoma (8%), hepatocellular carcinoma (8%) and gastric adenocarcinoma (8%). It is noteworthy that two of three present cases identified, were the result of directly infiltration of nasopharyngeal carcinoma to the pituitary gland. There is limited data documenting the prevalence of nasopharyngeal carcinoma with pituitary metastasis within the Asian population. Conclusion Central diabetes insipidus unmasked by corticosteroids is a less recognized, potentially lethal but fully reversible complication of pituitary metastasis. Symptoms or signs of central diabetes insipidus should be sought in all patient with advanced malignancies presenting with polyuria and hypernatremia. Prompt restoration of pituitary hormones is warranted in affected patients to allow timely restoration of hormonal balance and preventing endocrine emergencies.

Background: Glial fibrillary acidic protein (GFAP) specific IgG autoantibody has been identified as a potential biomarker of immunotherapy responsive, sometimes paraneoplastic autoimmune meningoencephalomyelitis. We present one of the first cases of central diabetes insipidus (DI) in a patient with myxopapillary ependymoma and related GFAP paraneoplastic autoimmune meningoencephalomyelitis. Clinical case: A 23 year old female presented with progressive left facial and right sided weakness, extremity parasthesias, diplopia and word finding difficulty over four months. MRI brain showed 3cm left frontal periventricular white matter lesion. Stereotatic brain biopsy revealed a demyelinating process. She was found to be GFAP IgG positive concerning for GFAP astrocytopathy and glucocorticoid treatment was initiated. Four months later, she developed new polydipsia (22L water/day) and polyuria with elevated sodium 151mmol/L, serum osmolality 329 mOsm/kg (normal range 285-310), low urine osmolality 122 mOsm/kg (normal range 300-900), dilute urine specific gravity 1.003 and documented urine output greater than 4L/24hrs. Clinical presentation was consistent with central DI as the patient had an appropriate response to DDAVP administration. MRI pituitary revealed absence of the posterior pituitary bright spot with increased fullness of the posterior aspect of the pituitary gland and prior evidence of pituitary stalk thickening, suggestive of lymphocytic infundibuloneurohypophysitis. Baseline anterior pituitary function remained intact. MRI spine obtained one month later for acute worsening lower extremity weakness, urinary retention and incontinence noted diffuse leptomeningeal enhancement, nodularity and an irregular L2/L3 mass-like lesion. Biopsy confirmed a WHO grade I myxopapillary ependymoma and chemotherapy was initiated. Conclusion: While the underlying etiologies can be identified in approximately 50-70% of central DI cases, the remaining are deemed idiopathic with speculation that autoimmunity involving autoantibodies may be involved. Lymphocytic infundibuloneurohypophysitis has been linked to central DI in several case reports, albeit few. Interestingly, although various autoimmune endocrinopathies have been noted in cases of GFAP autoimmune meningoencephalomyelitis, to our knowledge, this is the first case report of associated central DI.

Langerhans cell histiocytosis (LCH) is a rare disease that usually affects children and young adults. Sclerosing cholangitis (SC) can occur in 10-15% of patients with disseminated form of the disease. Central diabetes insipidus (CDI) is a rare disorder that may be caused by a variety of diseases mainly LCH and germinoma especially in children. In this case report, a- 4-year-old girl who is a known case of CDI and a single bone lesion in the left humerus, presented with jaundice, abdominal distention and itching. The diagnosis of SC was made by histopathology on liver biopsy. In this case, we found a link between CDI and SC through LCH, the diagnosis of which was made by histopathology of the explanted liver. The combination of CDI, liver involvement with SC and a single bone lesion is remarkable, since the histological diagnosis of LCH was made outside the biliary tract in the liver parenchyma.

Introduction: Central diabetes insipidus (CDI) is an uncommon complication of ischemic stroke and usually occurs when the hypothalamic–pituitary axis is affected. Its development following a middle cerebral artery (MCA) infarct is rare but clinically significant, as delayed recognition can lead to dehydration and hypernatremia. Objective: To present a rare case of transient CDI following a right MCA infarction and highlight the importance of early diagnosis in post-stroke patients presenting with polyuria. Material and Methods: This case was evaluated through retrospective review of hospital records. Clinical data, including neurological findings, urine output, serum sodium, serum and urine osmolality, and fluid balance, were collected. Imaging studies (CT and MRI) were reviewed to assess the infarct and exclude hypothalamic–pituitary injury. Treatment with desmopressin and the patient’s clinical response were documented. Patient confidentiality was maintained according to institutional guidelines. Results: Following a right MCA infarct, the patient developed significant polyuria and rising serum sodium. Laboratory studies confirmed CDI. Desmopressin therapy led to rapid normalization of urine output and serum sodium. The CDI was transient, resolving after short-term treatment without recurrence. Conclusion: CDI can occur as a rare complication of MCA infarction. Clinicians should consider CDI in stroke patients with unexplained polyuria, as prompt diagnosis and treatment can prevent metabolic complications and support recovery. The occurrence of CDI following a stroke, along with its complete resolution during neurological recovery, points to a vascular mechanism as the underlying cause of this condition. Take home message: Central diabetes insipidus is a rare but important complication of middle cerebral artery infarction. Early recognition of post-stroke polyuria enables timely diagnosis and desmopressin treatment, preventing hypernatremia and dehydration. Transient CDI following ischemic stroke suggests a reversible vascular mechanism.

Central diabetes insipidus in five cats: clinical presentation, diagnosis and oral desmopressin therapy