Severe rhabdomyolysis due to adipsic hypernatremia after craniopharyngioma surgery

The association of diabetes insipidus and adipsia after craniopharyngioma surgery has high morbidity. Hypernatremia can be caused by adipsia and be aggravated by diabetes insipidus. Rhabdomyolysis rarely occurs. CASE REPORT: This is the first report of a diabetic patient with craniopharyngioma who developed diabetes insipidus and adipsia after surgery, evolving with severe hypernatremia that caused considerable rhabdomyolysis. CONCLUSION: The importance of the evaluation of muscle integrity when under hypernatremic states is pointed out. Although adipsia may have a simple solution through volunteer water ingestion, serious consequences such as repeated severe hypernatremia episodes and intense rhabdomyolysis with high morbidity could occur, if adipsia is not diagnosed.     

Baroregulation of vasopressin release in adipsic diabetes insipidus

Adipsic diabetes insipidus (ADI) occurs in association with a heterogeneous group of conditions. We report vasopressin (AVP) responses to hypotension in nine patients with ADI and nine controls. Hypertonic saline infusion produced absent thirst (1.7 +/- 1.7 to 1.5 +/- 1.7 cm, P = 0.99) and AVP responses (0.3 +/- 0.1 to 0.4 +/- 0.1 pmol/liter, P = 0.99) in the ADI group, who also drank less than the control group (258 +/- 200 ml vs. 1544 +/- 306 ml, P < 0.001). Intravenous infusion of trimetaphan camsylate produced a fall in mean arterial pressure of 31.6% +/- 8.9% in patients and 29.4% +/- 6.1% in controls. Plasma AVP concentrations rose from 1.4 +/- 0.8 to 340.3 +/- 497.4 pmol/liter (P < 0.001) in the control group. In three patients with craniopharyngioma, there was no rise in plasma AVP concentrations (0.3 +/- 0.1 to 0.3 +/- 0.1 pmol/liter, P = 0.96), but plasma AVP rose significantly in response to hypotension in the other six patients (0.4 +/- 0.2 to 204.5 +/- 223.2 pmol/liter, P < 0.001). We concluded that the AVP responses to hypotension in ADI are heterogeneous and reflect the site of the lesion causing the diabetes insipidus.     

Diabetes insipidus and adrenal insufficiency in a patient with metastatic breast cancer

A patient previously treated for bilateral breast cancer with mastectomy, radiation therapy and in remission on hormonal therapy for more than five years presented with abdominal symptoms from breast cancer relapse. She developed inappropriate polyuria and hypernatraemia, which responded to desmopressin. In combination with the absence of a high signal from the posterior lobe of the pituitary on MRI , these data indicated the presence of partial central diabetes insipidus. The anterior pituitary showed partial failure (low follicle-stimulating hormone, luteinising hormone and insulin-like growth factor-1 levels). Furthermore, primary adrenal insufficiency had developed, ascribed to bilateral tumour invasion of the adrenals. This rare combination of endocrinological failures in a patient with metastatic breast cancer is discussed.     

Objective assessment of thirst recovery in patients with adipsic diabetes insipidus

Adipsic diabetes insipidus (ADI) is characterised by impaired thirst and defective AVP secretion. We have assessed the thirst response to graded osmotic stimulation using a visual analog scale (VAS) in patients with a history of ADI following surgery for a craniopharyngioma. The patients were thought to be regaining their thirst response but we wanted to confirm that this was the case objectively before relaxing their strict fluid balance regimen. Three patients with adipisa in the presence of hypernatremia following surgery for a craniopharyngioma are described. Their median age at surgery was 13 years (range 11–15 years). All patients had previously demonstrated no desire to drink despite a serum osmolality in excess of 300 mOsmol/kg. Fluid balance was maintained postoperatively with a regimen involving a fixed daily fluid intake and DDAVP dose together with daily weights and regular assessment of capillary sodium concentrations. Patients were thought to be regaining thirst sensation and so were assessed by hypertonic saline infusion (HSI) with thirst measured using a VAS. Patients underwent a HSI test 4, 6 and 9 months post surgery. All had abnormally low AVP production at raised plasma osmolalities but the visual analogue scale confirmed partial or complete thirst recovery. The intensive regimen used to maintain stable serum sodium concentrations was relaxed without the patients subsequently developing a significant hyperosmolar state. We have shown objective recovery of thirst perception in patients with adipsia within 9 months of surgery, despite persistence of cranial diabetes insipidus. These observations indicate that both osmoreceptors regulating thirst and their efferent pathways demonstrate more plasticity than those regulating AVP production. The HSI and thirst VAS are an objective way of assessing patients known to have ADI who are thought to be recovering thirst perception.     

Diabetes insipidus in a patient with a highly malignant B-cell lymphoma and stomatitis.

A 37-year-old male patient with a diffuse pleomorphic B-cell-lymphoma, which has been diagnosed two month earlier with the primary site at the pterygopalatine fossa on both sides with infiltration of the clivus and cavernous sinus was referred to our hospital for continuation of the third course of CHOP chemotherapy. At admission he reported about a recent history of painful swallowing and intermittent substernal chest pain. Alleviation of the pain on swallowing and the chest pain was apparently only possible by drinking 10 to 15 l of cold coca cola throughout the day and night, a regimen that resulted in polyuria. Physical examination revealed extensive thrush stomatitis and soor esophagitis. Despite successful treatment with fluconazole, polydipsia continued unabated. The classic osmotic test of dehydration and exogenous vasopressin revealed hypothalamic diabetes insipidus (DI). Basal hormones and stimulated endocrine function tests of the adenohypophysis were found to be normal. MRI-scan revealed lymphoma infiltration of the neurohypophysis. After the third course of CHOP chemotherapy the patient surprisingly recovered completely from his excessive thirst. The present report shows that clinical disorders such as thrush stomatitis can mask diabetes insipidus caused by an early relapsing lymphoma.     

Management of geriatric mania complicated by hyponatremia and psychogenic adipsic hypernatremia

A 70-year-old male presented with first episode mania and hyponatremia, which were later corrected with an infusion of hypertonic saline. His clinical condition deteriorated because of adipsic hypernatremia associated with hypokalemic periodic paralysis. Hypernatremia correction and potassium supplementation were started but could not be achieved because of manic symptoms as the patient's oral intake was poor. He was restarted on sodium valproate and olanzapine. Over the next few days, the patient's manic symptoms improved significantly and oral intake improved, which eventually led to correction of hypernatremia. Clinicians should evaluate the existence of electrolyte imbalance while dealing with a case of geriatric mania.     

Clinical insights into adipsic diabetes insipidus: a large case series

OBJECTIVE: Adipsic diabetes insipidus (DI) causes significant hypernatraemia. Morbidity and mortality data for patients with adipsic DI have been previously published as single case reports, rather than as formal trials or case series from units with established management protocols. Our objective was to describe morbidity and mortality in patients with adipsic DI attending a tertiary referral centre, representing the largest reported series of adipsic DI, and to suggest management protocols for such patients, based on our extensive experience of this condition. DESIGN: Arginine vasopressin (AVP) responses to hypotension were recorded during trimetaphan infusion. Sleep abnormalities were identified using overnight oximetry or polysomnography. Case-note analysis defined other clinical abnormalities including seizures and thrombotic episodes. Important clinical points for the management of these patients are highlighted. PATIENTS: Thirteen patients with adipsic DI defined by thirst and plasma AVP responses to hypertonic saline infusion. RESULTS: All patients had absent AVP and thirst responses to osmotic stimulation, with subnormal water intake. Five patients had absent AVP responses to hypotension; the remainder had normal responses. Eight patients were obese [body mass index (BMI) > 30 kg/m(2)], and three were overweight (BMI > 25 kg/m(2)). Seven patients had sleep apnoea, of whom three died at 36 years or younger. Four patients developed venous thrombosis during episodes of hypernatraemia. Two patients had thermoregulatory dysfunction and seven patients had seizure activity. CONCLUSION: Adipsic DI is associated with significant morbidity and mortality. Physicians should be aware of associated, treatable hypothalamic abnormalities such as obesity, sleep apnoea, seizures and thermoregulatory disorders when managing adipsic DI.     

渴感减退性高钠血症病因及治疗探讨——5例报道及去氨加压素疗效观察

目的 渴感减退性高钠血症,又称特发性高钠血症.临床罕见,国内外报道有限,发病机制不详,尚无有效安全的治疗手段.本研究根据本组先前对渴感减退性高钠血症发病机制和治疗的探索,对5例渴感减退性高钠血症患者进行了醋酸去氨加压素的实验性临床治疗,观察对该病的临床缓解效果.方法 5例高钠血症伴有渴感缺失或减退的患者根据Halter等提出的参考标准诊断.对该5例患者进行血尿渗透压、血钠、尿比重、自由水清除率测定和禁水加压实验及水负荷实验,以视觉模拟刻度对渴感等级进行评估,并进行垂体前叶功能评价,所有患者进行强制性每日饮水2 000～2 500 ml,有垂体前叶功能减退者给予相应激素替代补充治疗,1周后在患者高血钠没有缓解的情况下给予醋酸去氨加压素治疗,观察高钠血症和渴感减退的缓解情况.结果 5例患者的血钠水平为160～190 mmol/L,血浆渗透压为330～370 mmol/L,而患者的渴感等级为0～2级.患者均存在部分性中枢性尿崩症,渗透压阈值升高为330～360 mmol/L.强制性每日饮水和垂体前叶激素替代治疗1周后高血钠仍然不能缓解.通过治疗尿崩症的药物醋酸去氨加压素0.05～0.2 mg/d对该5例患者进行试验性治疗5～7天后,发现患者血钠下降,渴感减退也有明显缓解,渴感等级上升为5～7级,且未见明显不良反应.结论 渴感减退性高钠血症存在中枢性部分性尿崩症,醋酸去氨加压素对渴感减退性高钠血症的治疗有效而且安全方便.     

Severe hypernatremia in a patient with psychiatric illness

A 17-year-old schizophrenic developed severe hypernatremia during a period of psychosis. The thirst-deficient abnormality that caused this hypernatremia resolved when his psychosis improved. The primary disorders causing a thirst deficiency leading to hypernatremia fall into three categories: lesions of the central nervous system, mineralocorticoid excess, and drug side effects. None of these disorders was found in our patient. We conclude that psychosis can severely impair the thirst mechanism directly.     

Diabetes insipidus as a complication of cryptococcal meningitis in an HIV-infected patient

We describe an HIV-infected patient with central diabetes insipidus as a complication of cryptococcal meningitis. Nephrogenic diabetes insipidus as a result of amphotericin B treatment is a rare but known complication. Central diabetes insipidus in cryptococcal meningitis has not been reported before. The patient responded well to desmopressin treatment.     

Diabetes insipidus in a pacient with multiple sclerosis

Multiple Sclerosis (ME) is a chronic progressive disease characterized by relapses of demyelination that can occur anywhere in the brain stem, spinal cord and optic nerve. Since central diabetes insipidus (DI) is mainly caused by central nervous system damage (such as trauma, surgery, tumor, infection, sarcoidosis), ME is included among its possible etiologies. However, this association is not commonly described. The clinical suspicion must be made in the presence of polyuria and polydipsia or refractory hypernatremia (in patients without free access to water) during the evolution of ME. We will describe a clinical report in which this association occurred and, after the beginning of desmopressin therapy, the clinical findings were reverted.     

Myelodysplastic syndrome with central diabetes insipidus manifesting hypodipsic hypernatremia and dehydration

Central diabetes insipidus (DI) is a rare but recognized complication of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) that is caused by leukemic infiltration to the hypothalamo-neurohypophyseal system. In rare patients in whom a wide region of the hypothalamus is involved, central DI results in hypodipsic hypernatremia and dehydration. Typical DI symptoms such as polydipsia, polyuria, and marked thirst are concealed in these cases, because the hypothalamic "thirst center" cannot send thirst stimuli to the cerebral cortex. Herein we describe a patient with MDS developing into AML, who presented with hypodipsic hypernatremia and dehydration. A diagnosis of central DI was made on the ground of a low level of serum anti-diuretic hormone (ADH) despite high serum osmolality. A magnetic resonance imaging study revealed attenuation of a physiological "bright spot" of the neurohypophysis. An induction course chemotherapy including regular-dose cytarabine and daunorubicin produced a rapid improvement of hypernatremia. The bone marrow aspirate after two courses of chemotherapy showed complete remission. At that point, ADH release and the "bright spot" were recovered. In order to correctly diagnose central DI in association with hematological malignancies, we should not overlook this atypical type of DI.     

Diabetes insipidus associated with leukaemia

Three cases of leukaemia complicated by pituitary diabetes insipidus are presented. In one patient diabetes insipidus developed five months before acute myelomonocytic leukaemia became apparent. The two other patients suffered from acute myelomonocytic leukaemia and from a blastic transformation of chronic myeloid leukaemia. In one case a transient cure of diabetes insipidus was obtained after intrathecal chemotherapy and irradiation of the skull.     

Diabetes insipidus and pregnancy

Diabetes insipidus (DI) is a rare complication of pregnancy. It is usually transient, being due to increased placental production of vasopressinase that inactivates circulating vasopressin. Gestational, transient DI occurs late in pregnancy and disappears few days after delivery. Acquired central DI can also occur during pregnancy, for example in a patient with hypophysitis or neuroinfundibulitis during late pregnancy or postpartum. Finally, pre-existing central or nephrogenic DI may occasionally be unmasked by pregnancy. Treatment with dDAVP (desmopressin, Minirin^®) is very effective on transient DI of pregnancy and also on pre-existing or acquired central DI. Contrary to vasopressin, dDAVP is not degraded by vasopressinase. Nephrogenic DI is insensitive to dDAVP and is therefore more difficult to treat during pregnancy if fluid intake needs to be restricted.