Diabetes insípida y virus del Zika / Diabetes insipid us and Zika virus

La diabetes insípida es el resultado de una secreción o acción reducidas de la hormona vasopresina, expresada clínicamente por un cuadro de poliuria-polidipsia. Los arbovirus pueden tener afinidad por el sistema nervioso y se ha demostrado que el Zika desencadena un trastorno autoinmune que ataca a las células nerviosas, lo que puede traer como consecuencia una diabetes insípida central. En la literatura médica nacional e internacional revisada no se reportan casos anteriores donde se vincule la diabetes insípida con el virus del Zika. Se presenta un caso a propósito de esta asociación: paciente femenina de 53 años, diagnosticada con infección por el virus del Zika dos semanas antes de comenzar con los síntomas sugestivos de diabetes insípida. El potencial neurotrópico del virus, así como los resultados en la resonancia magnética nuclear y la determinación de marcadores de autoinmunidad anti-ADNdc positivos, son elementos que apoyan la hipótesis de que la paciente presentó una posible hipofisitis autoinmune, como respuesta inflamatoria post-infección, desarrollando diabetes insípida central transitoria(AU)

Diabetes insipidus is the result of reduced secretion or action of the vasopressin hormone, which is clinically expressed by a polyuria-polydipsia picture. Arboviruses can have a nervous system affinity and Zika has been shown to trigger an autoimmune disorder that attacks nerve cells, which can result in central diabetes insipidus. The reviewed national and international medical literatures does not report previous cases linking diabetes insipidus with Zika virus. It is presented a case about this association: 53-year-old female patient diagnosed with Zika virus infection two weeks before starting symptoms suggestive of diabetes insipidus. The neurotropic potential of the virus, as well as the results in nuclear MRI and the determination of positive anti-ADNdc autoimmunity markers are elements that support the hypothesis that the patient had a possible autoimmune hypophysis, as a post-infection inflammatory response, developing transient central diabetes insipidus(AU)

Efectos endocrinológicos tardíos del tratamiento oncológico en supervivientes de meduloblastoma / Endocrinological late effects of oncologic treatment on survivors of medulloblastoma

INTRODUCCIÓN: La radioterapia, quimioterapia y la cirugía empleada en el tratamiento de los tumores cerebrales tienen efectos en el eje hipotálamo-hipofisario y pueden resultar en disfunción endocrina hasta en el 96% de los casos. PACIENTES Y MÉTODO: Estudio retrospectivo y descriptivo en pacientes diagnos ticados de meduloblastoma sometidos a tratamiento con quimio y radioterapia en los últimos 20 años en un hospital terciario. Se analizan variables edad, sexo, peso, talla, índice de masa corporal (IMC) al final del seguimiento, estadio de maduración sexual, niveles séricos de TSH y T4 libre, ACTH/cortisol e IGF-1, FSH, LH, estradiol, testosterona, perfil lipídico (colesterol total) y prueba de función dinámica de hormona de crecimiento. RESULTADOS: Muestra total de 23 pacientes. El déficit de hormona de crecimiento es la secuela más frecuente (82 %) seguido de disfunción ti roidea (44,8%) y disfunción puberal (24,1%). Solo se diagnosticó un caso de diabetes insípida y 2 casos de déficit de corticotrofina. CONCLUSIONES: El seguimiento a largo plazo de los supervivientes de meduloblastoma tratados con quimio y radioterapia revela una prevalencia muy alta de disfun ción endocrina, particularmente de deficiencia de hormona del crecimiento y de hipotiroidismo. Creemos oportuna la monitorización y el seguimiento a largo plazo de estos pacientes con el fin de garantizar un manejo terapéutico adecuado de aquellas disfunciones tratables.

INTRODUCTION: Radiation therapy, chemotherapy, and surgery used to treat brain tumors have effects on the hy pothalamic-pituitary-adrenal axis and can result in endocrine dysfunction in up to 96% of cases. PATIENTS Y METHOD: Retrospective and descriptive study in patients diagnosed with medulloblasto ma who underwent treatment with chemo and radiotherapy in the last 20 years in a tertiary hospital. The variables analyzed were age, sex, weight, height, body mass index (BMI) at the end of follow-up, sexual maturity stage, serum levels of TSH and free T4, ACTH/cortisol and IGF-1, FSH, LH, estradiol, testosterone, lipid profile (total cholesterol), and growth hormone dynamic function test. RESULTS: Total sample of 23 patients. Growth hormone deficiency is the most frequent sequelae (82%) fo llowed by thyroid dysfunction (44.8%), and disorders of puberty (24.1%). Only one case of diabetes insipidus and two cases of corticotropin deficiency were diagnosed. CONCLUSIONS: Long-term follow- up of medulloblastoma survivors treated with chemo and radiotherapy reveals a very high prevalence of endocrine dysfunction, especially growth hormone deficiency and hypothyroidism. We believe that monitoring and long-term follow-up of these patients is necessary in order to ensure adequate therapeutic management of those treatable dysfunctions.

Diabetes insipidus secondary to tuberculous meningoencephalitis with hypothalamic involvement extending to the hypophysis: a case report

Abstract The involvement of Mycobacterium tuberculosis in the central nervous system (CNS) is an uncommon and devastating manifestation of tuberculosis. We report a case of disseminated tuberculosis presenting as meningoencephalitis, hypothalamic involvement with extension to the hypophysis, and secondary insipidus diabetes diagnosed at autopsy.

Infundibuloneurohipofisitis: caso clínico y revisión de la literatura / Infundibuloneurohypophysitis: clinical report and literature review

Infundibuloneurohypophysitis is a rare condition, which is part of the group of hypophysitis, of relatively recent description (1993). The main clinical manifestation is diabetes insipidus, whose natural evolution is towards chronicity. The differential diagnosis with other thickening of the hypophysial stem is very important, where the clinic, imaging, laboratory and eventually biopsy are a main support for a correct diagnosis. We present a clinical case that shows the usual picture of infundibuloneurohypophysitis, and illustrates the imaging evolution in a female patient, with diabetes insipidus as the main clinical manifestation

Surgical outcomes of the endoscopic endonasal transsphenoidal approach for large and giant pituitary adenomas: institutional experience with special attention to approach-related complications / Resultados cirúrgicos do acesso endonasal endoscópico transesfenoidal para adenomas hipofisários grandes e gigantes: experiência institucional com ênfase às complicações relacionadas ao acesso cirúrgico

ABSTRACT Objective In this study, we investigate our institutional experience of patients who underwent endoscopic endonasal transsphenoidal approach for treatment of large and giant pituitary adenomas emphasizing the surgical results and approach-related complications. Method The authors reviewed 28 consecutive patients who underwent surgery between March, 2010 and March, 2014. Results The mean preoperative tumor diameter was 4.6 cm. Gross-total resection was achieved in 14.3%, near-total in 10.7%, subtotal in 39.3%, and partial in 35.7%. Nine patients experienced improvement in visual acuity, while one patient worsened. The most common complications were transient diabetes insipidus (53%), new pituitary deficit (35.7%), endonasal adhesions (21.4%), and cerebrospinal fluid leak (17.8%). Surgical mortality was 7.1%. Conclusions Endoscopic endonasal transsphenoidal surgery is a valuable treatment option for large or giant pituitary adenomas, which results in high rates of surgical decompression of cerebrovascular structures.

RESUMO Objetivo Neste manuscrito investigamos a experiência institucional com o acesso endonasal endoscópico transesfenoidal no tratamento de adenomas hipofisários grandes e gigantes com ênfase às complicações relacionadas ao acesso cirúrgico. Método Foram incluídos neste estudo 28 pacientes consecutivos submetidos à cirurgia entre Março de 2010 e Março de 2014. Resultados O diâmetro médio pré-operatório dos tumores era 4,6 cm. Uma ressecção total foi obtida em 14,3%; quase total, em 10,7%; subtotal, em 39,3% e parcial, em 35,7%. Nove pacientes evoluíram com melhora na acuidade visual, enquanto um paciente apresentou piora da função visual. As complicações mais comuns foram diabetes insipidus transitório (53%), novo défice hipofisário (35,7%), sinéquias endonasais (21,4%) e fistula liquórica (17,8%). A mortalidade cirúrgica foi 7,1%. Conclusões A cirurgia por via endonasal endoscópica transesfenoidal é uma opção terapêutica extremamente útil para adenomas hipofisários grandes e gigantes, a resultar numa significativa descompressão das estruturas cerebrovasculares.

Spontaneous remission of acromegaly: apoplexy mimicking meningitis or meningitis as a cause of apoplexy? / Remissão espontânea da acromegalia: meningite simulando apoplexia ou meningite como causa da apoplexia?

Pituitary apoplexy is a rare but potentially life-threatening clinical syndrome characterized by ischemic infarction or hemorrhage into a pituitary tumor. The diagnosis of pituitary tumor apoplexy is frequently complicated because of the nonspecific nature of its signs and symptoms, which can mimic different neurological processes, including meningitis. Several factors have been associated with apoplexy, such as dopamine agonists, radiotherapy, or head trauma, but meningitis is a rarely reported cause. We describe the case of a 51-year-old woman with acromegaly due to a pituitary macroadenoma. Before surgical treatment, she arrived at Emergency with fever, nausea, vomiting and meningismus. Symptoms and laboratory tests suggested bacterial meningitis, and antibiotic therapy was initiated, with quick improvement. A computerized tomography (CT) scan at admission did not reveal any change in pituitary adenoma, but a few weeks later, magnetic resonance imaging (MRI) showed data of pituitary apoplexy with complete disappearance of the adenoma. Currently, her acromegaly is cured, but she developed hypopituitarism and diabetes insipidus following apoplexy. We question whether she really experienced meningitis leading to apoplexy or whether apoplexy was misinterpreted as meningitis. In conclusion, the relationship between meningitis and pituitary apoplexy may be bidirectional. Apoplexy can mimic viral or bacterial meningitis, but meningitis might cause apoplexy, as well. This fact highlights the importance of differential diagnosis when evaluating patients with pituitary adenomas and acute neurological symptoms.

A apoplexia é uma síndrome clínica rara, mas potencialmente fatal, caracterizada por infarto isquêmico ou hemorragia em um tumor pituitário. O diagnóstico de apoplexia de tumor pituitário é frequentemente complicado pela natureza inespecífica dos seus sinais e sintomas, que podem simular diferentes processos neurológicos, incluindo a meningite. Vários fatores estão associados com a apoplexia, como o uso de agonistas dopaminérgicos, radioterapia ou trauma da cabeça, mas a meningite foi raramente relatada. Descrevemos o caso de uma mulher de 51 anos de idade com acromegalia por um macroadenoma pituitário. Antes do tratamento cirúrgico, ela foi trazida ao pronto-socorro com febre, náusea, vômitos e meningismo. Os sintomas e análises laboratoriais sugeriram meningite bacteriana e o tratamento com antibióticos foi iniciado, com melhora rápida dos sintomas. Uma tomografia computadorizada (CT) na admissão ao hospital não revelou nenhuma alteração no adenoma pituitário, mas algumas semanas depois uma ressonância magnética (MRI) mostrou informações de apoplexia pituitária, com desaparecimento completo do adenoma. Atualmente, a acromegalia está curada, mas ela desenvolveu hipopituitarismo e diabetes insipidus depois da apoplexia. Questionamo-nos se a paciente realmente apresentou meningite que levou à apoplexia ou se a apoplexia foi mal interpretada como sendo meningite. A relação entre a meningite e a apoplexia pode ser bidirecional. A apoplexia pode simular a meningite viral ou bacteriana, mas a meningite também pode causar apoplexia. Esse fato enfatiza a importância do diagnóstico diferencial ao se avaliar pacientes com adenomas pituitários e sintomas neurológicos.

Tumores del sistema nervioso central de localización selar y supraselar. Alteraciones neuro-oftalmológicas y de la función endocrina al diagnóstico / Sellar and suprasellar central nervous system tumors. Neuro-ophthalmological and endocrine function impairment at diagnosis

Los tumores (Tu) del SNC constituyen la segunda enfermedad oncológica en edad pediátrica, con una incidencia referida aproximada que oscila entre el 10 y 15%. En 309 pacientes con tumores selares y supraselares, seguidos durante 15 años, se evaluó en función de los distintos oncotipos tumorales, síntomas iniciales y alteraciones endocrinológicas previas al inicio del tratamiento. De ellos, 227 pacientes presentaron el tumor a edad prepuberal. Los oncotipos tumorales más frecuentes fueron craneofaringioma (CRA), glioma (GLIA) y tumor de células germinales (GERM). También, se encontró una mayor incidencia de presentación en varones. En edad puberal (n:92), el oncotipo tumoral más frecuente fue adenoma hipofisario (ADENO), seguido de GLIA y CRA. En este ultimo oncotipo tumoral, y, a diferencia del grupo prepuberal, su incidencia fue significativamente mayor en niñas. Aproximadamente 90% de los pacientes tuvieron anormalidades neuro-oftalmológicas (hipertensión craneal, dolores de cabeza, vómitos y pérdida progresiva de la visión) como uno de los signos y/o síntomas iniciales. Alteraciones clínicas endocrinológicas como baja talla, velocidad de crecimiento anormal, diabetes insípida y alteraciones del tempo puberal son frecuentes en estos pacientes y están habitualmente asociadas con las alteraciones clínico-neuro-oftalmológicas como las ya mencionadas. No obstante, la mayoría de los tumores del SNC localizados en la línea media suelen ser diagnosticados por manifestaciones neuro-oftalmológicas. Los resultados del estudio muestran alteración de la función endócrina al diagnóstico del Tu. Se concluye que en todo paciente con crecimiento lento o baja talla, así como también signos clínicos que orienten a un diagnóstico de pubertad precoz y/o retardada, el pediatra debe incluir dentro de los diagnósticos diferenciales, el diagnóstico del tumor selar o supraselar. La morbilidad aumenta frecuentemente luego de la cirugía (AU)

During the last 15 years, 309 patients with tumors of the sellar and suprasellar areas of CNS were followed in our Hospital (Endocrine Service). Tumor oncotype, initial symptoms and endocrine disturbances before any treatment was started are presented. In 227 patients, the tumor was diagnosed at prepubertal age. In this group, the most frequent tumoral oncotypes were craniopharyngioma (CRA), glial tumors (GLIA) and germ cells tumors (GERM). The incidence was higher in boys. At pubertal age (n:92), the most frequent tumoral oncotype was pituitary adenoma (ADENO), followed by GLIA and CRA. In the latter, and different from the prepubertal group, the incidence was significantly higher in girls. Approximately 90% of patients had neuro-ophtalmological abnormalities (cranial hypertension, headaches, vomits, and progressive loss of vision) as one of the initial signs and/or symptoms. Clinical endocrine disorders, such as short stature, low growth velocity, diabetes insipidus, and alterations in pubertal "tempo" are frequent in these patients and are often associated with the neuro-ophtalmological abnormalities mentioned above. This clinical symptomatology has to alert the medical team to discard the presence of a CNS tumor at the sellar and/or suprasellar level. We conclude that tumors of the SNC localized in the midline, have potential capacity to provoke abnormalities in endocrine function. Morbidity is often increased after surgery (AU)

Panhipopituitarismo y diabetes insípida secundarios a hipofisitis linfocitaria en una paciente con trisomia 12p / Panhypopituitarism and diabetes insipidus secondary to a lymphocytic hypophysitis in one patient with trisomy 12p

Lymphocytic hypophysitis (LH) is an uncommon inflammatory disease of the hypophysis. It's female to male ratio of appearance is 9:1. Pregnant women are more affected during the third trimester of pregnancy or postpartum. Clinical and radiological presentation can simulate a hypophyseal adenoma. We report a nonpregnant 13 years old adolescent, with a trisomy 12p, with panhypopituitarism, diabetes insipidus and a selar tumor. It was necessary to differentiate between a germinoma and a LH. The latter was confirmed with the hypophyseal biopsy.

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. DASE 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.

A associação de diabetes insipidus e adipsia após cirurgia de craniofaringioma implica em alta morbidade. Hipernatremia pode desenvolver-se devido a adipsia e ser agravada por diabetes insipidus. Rabdomiólise raramente ocorre. DESCRIÇÃO DO CASO: Esta é a primeira descrição de paciente diabético com craniofaringioma que desenvolveu diabetes insipidus e adipsia após a cirurgia, evoluindo com hipernatremia grave e conseqüente rabdomiólise maciça. CONCLUSÃO: Ressalta-se a necessidade de avaliar a integridade muscular na vigência de estados hipernatrêmicos. Apesar de apresentar solução simples, como ingestão voluntária de água, pode haver sérias conseqüências se o diagnóstico de adipsia não é realizado, como episódios repetidos de hipernatremia grave com rabdomiólise intensa e elevada morbidade.

Syndromes related to sodium and arginine vasopressin alterations in post-operative neurosurgery

BACKGROUND: Cerebral salt wasting syndrome (CSWS), syndrome of inappropriate antidiuretic hormone secretion (SIADH) and diabetes insipidus (DI) are frequently found in postoperative neurosurgery. PURPOSE: To identify these syndromes following neurosurgery. METHOD: The study included 30 patients who had been submitted to tumor resection and cerebral aneurysm clipping. Sodium levels in serum and urine and urine volume were measured daily up to the 5th day following surgery. Plasma arginine vasopressin (AVP) was measured on the first, third and fifth days post-surgery. RESULTS: CSWS was found in 27/30 patients (90 percent), in 14 (46.7 percent) of whom it was associated with a reduction in the levels of plasma AVP (mix syndrome). SIADH was found in 3/30 patients (10 percent). There was no difference between the two groups of patients. CONCLUSION: CSWS was the most common syndrome found, and in half the cases it was associated with DI. SIADH was the least frequent syndrome found.

INTRODUÇÃO: A síndrome perdedora de sal (SPS), síndrome da secreção inapropriada do hormônio antidiurético (SIADH) e diabetes insipidus (DI) são freqüentemente encontradas no pós-operatório de neurocirurgia. OBJETIVO: Identificar essas síndromes relacionadas à neurocirurgia. MÉTODO: Foram estudados 30 pacientes submetidos à ressecção de tumor (n=19) e clipagem de aneurisma (n=11) cerebral durante os primeiros cinco dias do pós-operatório. Os pacientes foram submetidos a dosagens diárias de sódio sérico e urinário até o 5° dia pós-operatório, com controle de volume urinário neste período e dosagem de arginina-vasopressina (AVP) plasmática no 1°, 3° e 5° dias pós-operatórios. RESULTADOS: A SPS foi encontrada em 27/30 pacientes (90 por cento), em 14/27 (46,7 por cento) associada à diminuição dos níveis de AVP plasmática (síndrome mista). A SIADH foi encontrada em 3/30 pacientes (10 por cento). Não houve diferença entre os dois grupos de pacientes. CONCLUSÃO: A SPS foi a síndrome mais freqüente, em metade de casos associada ao DI. A SIADH foi a menos freqüente.

Diabetes insípido pós-traumático: relato de caso / Post-traumatic diabetes insipidus: case report and review of the literature

Diabetes insípido é uma síndrome caracterizada por poliúria e polidipsia. Existem várias causas para o seu desenvolvimento. O traumatismo craniencefálico corresponde à cerca de 3 por cento das causas de diabetes insípido central. Os autores relatam o caso de um paciente com 27 anos de idade, politraumatizado, que desenvolveu diabetes insípido dois dias após o trauma. Discutem a fisiopatologia, diagnóstico, tratamento e fazem uma revisão de leiteratura médica.

A Case of Gastric Cancer Initially Presenting with Polydipsia

Metastatic brain tumors from gastric cancer are extremely rare. A 61-year-old Korean woman, initially presenting with polydipsia and polyuria, was found to have metastatic lesions in the brain by MRI. We performed several diagnostic procedures to determine the origin of the brain metastases. She was revealed to have a soft tissue mass of the right adrenal gland and fungating ulcers in the stomach. Histologic studies of both the adrenal gland mass and gastric tissues revealed malignant tumors composed of anaplastic cells. Based on the electron microscopy study, the malignant tumor of the right adrenal gland was a metastatic lesion from the anaplastic carcinoma of stomach. Therefore, the malignant tumors of the brain were assumed to have originated from the gastric cancer. This case report is presented to make clinicians aware of the possibility that diabetes insipidus (polydipsia) may present as an initial manifestation of brain metastases.

Diabetes insipidus in a patient with diabetes mellitus.

The association of Diabetes Mellitus (DM) and Diabetes Insipidus (DI) without any congenital defects is very rare and we report here a case of type 2 diabetes mellitus (NIDDM) whose blood sugar was controlled by insulin, developing central diabetes insipidus 2 years later, which could be successively controlled by synthetic vasopressin.

Diabetes insípida en cáncer de mama. Comunicación de dos casos clínicos. Revisión de la literatura / Diabetes insipidus in breast cancer. Report of 2 cases. Literature review

Las metástasis hipofisarias son poco frecuentes, constituyendo 0,14-2,8 por ciento de las metástasis encefálicas. Las neoplasias que con mayor frecuencia las origina son: el cáncer de mama y el cáncer de pulmón. Afectan principalmente la neurohipófisis siendo la Diabetes Insípida una de sus manifestaciones. Es un sindrome clínico poco frecuente caracterizado por polidipcia, poliuria de inicio brusco con aumento del volumen urinario y disminución de la osmolaridad urinaria. El diagnóstico se centra en la historia clínica, pero se apoya en la TAC/RNM de cráneo, dosificación plasmática de vasopresina y pruebas dinámicas. Su tratamiento se basa en dos pilares como son la administración de análogos de vasopresina y el tratamieto de la enfermedad de fondo. Se revisa la literatura a propósito de dos casos clínicos y se discuten las estrategias terapéuticas

Suprasellar tuberculoma presenting with diabetes insipidus and hypothyroidism--a case report.

Suprasellar tuberculomas are exceptionally rare and are even rarer in children. A case of suprasellar tuberculoma in a five year old male child who presented with diabetes insipidus, hypothyroidism and visual field defect is described. Deranged CSF parameters, imaging findings of a suprasellar rounded, peripherally enhancing lesion with thickening of the pituitary stalk, led to an accurate diagnosis of tuberculosis. The patient responded well to antituberculous treatment and an unnecessary surgery was avoided.

Complicaçöes das cirurgias hipofisárias: análise de 120 cirurgias no HUCFF-UFRJ / Complications of pituitary surgery: analysis of 120 operations at HUCFF-UFRJ

Estudamos uma série de 108 pacientes, submetidos a 120 intervenções cirúrgicas para tratamento de tumores hipofisários realizadas no Hospital Universitário Clementino Fraga Filho (HUCFF) da Universidade Federal do Rio de Janeiro (UFRJ), no período de 1979 a julho de 1998, com o objetivo de analisar a morbidade e mortalidade pós-operatória imediata. A idade dos pacientes variou entre 15 e 70 anos. Os diagnósticos etiológicos foram: adenomas não secretores, 46 (38,34 por cento); acromegalia, 30 (25 por cento); prolactinoma, 29 (24,16 por cento) e doença de Cushing, 15 (12,5 por cento). As principais complicações endocrinológicas foram: pan-hipopituitarismo, 16 (13,34 por cento); diabetes insípidus central (DIC), 15 (12,5 por cento); hiposuparenalismo, 4 (3,34 por cento). Complicações neurocirúrgicas: infecção, 13 (10,84 por cento) e fístula liquórica, 6 (5 por cento). Ocorreram 2 óbitos (1,67 por cento). Nossos resultados se enquadram dentro dos encontrados na literatura internacional. Quando analisamos ano a ano esta casuística identificamos queda progressiva da morbimortalidade, demonstrando a importância não só da habilidade do neurocirgião, como da uniformidade da equipe que acompanha estes pacientes.

Acute spinal cord and head injury: case report and discussion of cardiac, respiratory and endocrine abnormalities

We report a male patient who after a fall suffered high cervical spinal cord and head (cerebral) injuries. These injuries led to spinal shock, marked sinus bradycardia and asystolic cardiac and respiratory arrests, recalcitrant central traumatic diabetes insipidus, and death within approximately seven weeks. Temporary transvenous cardiac pacing proved useful in the management of this patient