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Infundíbulo-neuro-hipofisite
ORPHA:238305CID-10 · E23.6CID-11 · 5A61.5DOENÇA RARA
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Introdução

O que você precisa saber de cara

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Diabetes insípido central, também conhecido como deficiência de arginina vasopressina (AVP-D), é uma forma de diabetes insípido que decorre da falta de produção de vasopressina (ADH) no cérebro. A vasopressina atua aumentando o volume de sangue (intravascular) e diminuindo o volume de urina produzida. Portanto, a sua falta causa aumento na produção de urina e depleção de volume.

Publicações científicas
48 artigos
Último publicado: 2026 Mar 24
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CID-10: E23.6
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Anos de pesquisa1desde 2026
Total histórico48PubMed
Últimos 10 anos29publicações
Pico20174 papers
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2026Hoje · 2026📈 2017Ano de pico
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Publicações mais relevantes

Timeline de publicações
30 papers (10 anos)
#1

Percheron Artery Implicance in Bi-Thalamic Stroke Following Endoscopic Endonasal Approach for Infundibulo-Neurohypophysitis: A Combination of Two Rare Entities.

Turkish neurosurgery2026

Bi-thalamic stroke is rarely reported in the literature as a complication of an endonasal endoscopic procedure. It has been associated with presence of a Percheron artery variant, as well as with top of the basilar syndrome, both of which significantly increase patient surgical morbidity. Infundibulo-neurohypophysitis in turn, is an unusual inflammatory disorder affecting the infundibulum, the pituitary stalk, and the neurohypophysis. We present the case of a patient with visual impairment and an abnormal hormone profile compatible with infundibulo-neurohypophysitis, in whom tumor resection was conducted through an endoscopic endonasal approach (EEA). Patient developed postoperative bi-thalamic stroke due to Percheron artery infarct. A review of both conditions is included. This is the fourth case reported in the literature of a Percheron artery infarct, and to the best of our knowledge, the first linking it to endoscopic treatment of neurohypophysitis, itself an infrequent condition.

#2

A case of Epstein-Barr virus- associated lymphocytic infundibulo-neurohypophysitis.

BMC infectious diseases2025 Nov 19

The reported cases of viral infection-related lymphocytic hypophysitis increased in recent years. However, there is no report on Epstein-Barr Virus (EBV) - associated lymphocytic hypophysitis. Here we present the first case of central diabetes insipidus resulting from EBV-associated lymphocytic infundibulo-neurohypophysitis (LINH) accompanied by acute pancreatitis and acute kidney injury. A 47-year-old male patient presented to the emergency department with a chief complaint of fever, bloated stomach and vomiting for two days. Subsequently, he developed oliguria and tachypnea. With continuous renal replacement treatment and mechanical ventilation, 24-hour urine output gradually increased to a maximum of 10,100 mL accompanied by hypernatremia. After a short-term desmopressin acetate treatment, 24-hour urine output came down within the normal range. The test showed that the patient was having replicative EBV infection. Magnetic resonance imaging of pituitary revealed an absence of posterior pituitary bright spot. The level of ESR was higher than that at admission, even when the patient has no fever and the urine output was normal. Considering all the above and the fact that polyuric phase of recovery from acute kidney injury usually show hyponatremia, we made a diagnosis of EBV- associated LINH. With the lack of mass-like effect symptoms and anterior pituitary dysfunction, glucocorticoids treatment was discontinued after two weeks, even though erythrocyte sedimentation rate (ESR) had not returned to normal range at that time. Fortunately, ESR became normal after 2.5 months and there were bright spots in the posterior pituitary 6 months after discharge. EBV infection could result in severe consequences, presenting a challenge in diagnoses. The inflammatory process of EBV-associated LINH is self-limited and recovers spontaneously.

#3

Arginine-vasopressin deficiency due to long COVID-associated infundibulo-neurohypophysitis.

Archives of endocrinology and metabolism2025 Apr 11

Long COVID is defined by the occurrence of signs, symptoms, and conditions that develop after COVID-19 and may affect several organs and systems. Arginine-vasopressin deficiency (AVP-D; central diabetes insipidus) is a very rare complication of COVID-19 and SARS-CoV-2 immunization. Case reports, original studies, and reviews on AVP-D and long COVID published until February 2024 were retrieved from PubMed. A 47-year-old man presented with polydipsia, polyuria, memory loss, and mental fog 8 weeks after an episode of mild COVID-19. His past personal and family medical history were unremarkable. Biochemical evaluation was relevant for low urine osmolality and a 24-hour urine volume of 10,350 mL. Basal anterior pituitary evaluation was normal. A water deprivation test was started and interrupted after 2 hours due to the development of hypernatremia, high serum osmolality, and low urine osmolality. Urine osmolality significantly increased after intranasal desmopressin 20 μg. Contrast-enhanced pituitary MRI was suggestive of infundibulo-neurohypophysitis. Further biochemical, genetic, and imaging tests excluded secondary AVP-D causes.The patient was subsequently started on oral desmopressin, showing prompt response. After a follow-up of 20 months, he remained well-controlled with isolated AVP-D. Although molecular and histologic confirmation of SARS-CoV-2 infundibulo-neurohypophysitis could not be investigated, a strong temporal relationship and the absence of an alternative diagnosis rendered plausible the inclusion of AVP-D in the myriad of long COVID manifestations. Further studies with patients recovered from COVID-19 are necessary for a better understanding of the epidemiology, pathophysiology, and clinical course of this very rare endocrine condition.

#4

A Toluene-induced Infundibulo-neuro-hypophysitis: Presentation of a New Cause of Hypophysitis Secondary to Toxic Exposure and Review on Toluene Inhalation Endocrine Effects.

Endocrine, metabolic & immune disorders drug targets2025

Hypophysitis is a rare inflammatory disorder of the pituitary gland. Symptoms and signs of hypophysitis can be various, progressing insidiously, and its recognition may be challenging. We report the clinical history and therapeutic management of a 59-year-old man diagnosed with arginine vasopressin deficiency (AVP-D) due to an infundibulo-neurohypophysitis (INH) that occurred after the patient had inhaled spray film containing toluene. In consideration of the clinical signs and radiological imaging suggestive of INH, therapy with desmopressin and corticosteroids was instituted, with gradual improvement of polyuria and resolution of the radiological features of INH. To our knowledge, we described the first case of INH, manifested with AVP-D, secondary to toluene exposure. In addition, the endocrine effects of toluene inhalation were discussed. Finally, given the scarcity of data available, an overview of all the known toxic substances inducing AVP-D was also provided.

#5

A case of central diabetes insipidus after COVID-19 as a probable diagnosis of lymphocytic infundibulo-neurohypophysitis with positive anti-rabphilin-3A antibodies with review of literature.

Endocrine journal2024 Dec 02

Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2, and various complications have been reported. Furthermore, there have been increasing reports of endocrinopathy related to COVID-19 following the pandemic. We report a 49-year-old healthy woman who developed rapid onset of polydipsia and polyuria three weeks after COVID-19. Laboratory tests indicated low urine osmolarity and increased serum osmolarity, and antidiuretic hormone (ADH) was undetectable. Urine osmolality remained low with water deprivation. Similarly, plasma ADH responses to hypertonic-saline infusion were blunted and urine osmolality increased in response to desmopressin. There was no clear evidence of anterior pituitary dysfunction. T1-weighted magnetic resonance imaging (MRI) showed pituitary stalk thickening and absence of posterior pituitary bright signal spots, suggesting the presence of hypophysitis. Based on these results, we made a probable diagnosis of lymphocytic infundibulo-neurohypophysitis (LINH) which have caused central diabetes insipidus. Positive findings for serum anti-rabphilin-3A antibodies, reported as a potential diagnostic marker for LINH, were also noted. Following oral desmopressin administration, polydipsia and polyuria were quickly improved, though treatment with desmopressin was still required over four months. This is the first report of a patient with a probable diagnosis of LINH after COVID-19 who tested positive for anti-rabphilin-3A antibodies. Positive findings for those antibodies suggest that pituitary dysfunction associated with COVID-19 is hypophysitis involving an abnormal immune mechanism. The presence of anti-rabphilin-3A antibodies may be useful as a non-invasive diagnostic marker of LINH and potentially serve as a valuable diagnostic aid in cases of LINH associated with COVID-19.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC24 artigos no totalmostrando 29

2026

Percheron Artery Implicance in Bi-Thalamic Stroke Following Endoscopic Endonasal Approach for Infundibulo-Neurohypophysitis: A Combination of Two Rare Entities.

Turkish neurosurgery
2025

A case of Epstein-Barr virus- associated lymphocytic infundibulo-neurohypophysitis.

BMC infectious diseases
2025

Arginine-vasopressin deficiency due to long COVID-associated infundibulo-neurohypophysitis.

Archives of endocrinology and metabolism
2025

A Toluene-induced Infundibulo-neuro-hypophysitis: Presentation of a New Cause of Hypophysitis Secondary to Toxic Exposure and Review on Toluene Inhalation Endocrine Effects.

Endocrine, metabolic & immune disorders drug targets
2024

A case of central diabetes insipidus after COVID-19 as a probable diagnosis of lymphocytic infundibulo-neurohypophysitis with positive anti-rabphilin-3A antibodies with review of literature.

Endocrine journal
2024

Arginine vasopressin deficiency onset after COVID-19 vaccination with positive anti-rabphilin-3A antibodies: a case report and literature review.

BMC endocrine disorders
2024

A Toluene-induced Infundibulo-neurohypophysitis: A New Cause of Hypophysitis Secondary to Toxic Exposure.

Endocrine, metabolic & immune disorders drug targets
2024

A Pregnant Woman with Excess Vasopressinase-Induced Diabetes Insipidus Complicated by Central Diabetes Insipidus like Lymphocytic Infundibulo-Neurohypophysitis.

Case reports in endocrinology
2023

A Case of Lymphocytic Infundibulo-neurohypophysitis Exhibiting Spontaneous Regression.

JCEM case reports
2023

Two children with lymphocytic hypophysitis presenting with positive anti-rabphilin-3A antibody.

Endocrine journal
2023

Neuroimaging of hypophysitis: etiologies and imaging mimics.

Japanese journal of radiology
2022

Infantile-Onset Isolated Neurohypophyseal Langerhans Cell Histiocytosis with Central Diabetes Insipidus: A Case Report.

Children (Basel, Switzerland)
2022

Pituitary stalk thickening in patients under 18 years of age - the most common causes and diagnostic procedures.

Pediatric endocrinology, diabetes, and metabolism
2022

Ketoacidosis, Hypertriglyceridemia and Acute Pancreatitis Induced by Soft Drink Polydipsia in a Patient with Occult Central Diabetes Insipidus.

Internal medicine (Tokyo, Japan)
2021

Diabetes insipidus secondary to nivolumab-induced neurohypophysitis and pituitary metastasis.

Endocrinology, diabetes & metabolism case reports
2020

Anti-pituitary antibodies as a marker of autoimmunity in pituitary glands.

Endocrine journal
2021

A case of isolated hypothalamitis with a literature review and a comparison with autoimmune hypophysitis.

Endocrine journal
2020

Antibodies Against Hypothalamus and Pituitary Gland in Childhood-Onset Brain Tumors and Pituitary Dysfunction.

Frontiers in endocrinology
2020

Pituitary Stalk Enlargement in Adults.

Neuroendocrinology
2018

Manifestation of Central Diabetes Insipidus in a Patient with Thyroid Storm.

Internal medicine (Tokyo, Japan)
2018

Critical role of rabphilin-3A in the pathophysiology of experimental lymphocytic neurohypophysitis.

The Journal of pathology
2018

Cullin-associated NEDD8-dissociated protein 1, a novel interactor of rabphilin-3A, deubiquitylates rabphilin-3A and regulates arginine vasopressin secretion in PC12 cells.

Endocrine journal
2017

Review on Recent Topics in Hypophysitis.

Journal of Nippon Medical School = Nippon Ika Daigaku zasshi
2017

Usefulness of anti-rabphilin-3A antibodies for diagnosing central diabetes insipidus in the third trimester of pregnancy.

Endocrine journal
2016

Central diabetes insipidus.

Nagoya journal of medical science
2017

Anti-pituitary antibodies against corticotrophs in IgG4-related hypophysitis.

Pituitary
2017

Spontaneous intermittent MRI changes of a pituitary stalk lesion causing diabetes insipidus and amenorrhea.

Endocrine
2015

Lymphocytic infundibulo-neurohypophysitis: a clinical overview.

Endocrine
2015

Rabphilin-3A as a Targeted Autoantigen in Lymphocytic Infundibulo-neurohypophysitis.

The Journal of clinical endocrinology and metabolism

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Percheron Artery Implicance in Bi-Thalamic Stroke Following Endoscopic Endonasal Approach for Infundibulo-Neurohypophysitis: A Combination of Two Rare Entities.
    Turkish neurosurgery· 2026· PMID 41553207mais citado
  2. A case of Epstein-Barr virus- associated lymphocytic infundibulo-neurohypophysitis.
    BMC infectious diseases· 2025· PMID 41257589mais citado
  3. Arginine-vasopressin deficiency due to long COVID-associated infundibulo-neurohypophysitis.
    Archives of endocrinology and metabolism· 2025· PMID 40215290mais citado
  4. A Toluene-induced Infundibulo-neuro-hypophysitis: Presentation of a New Cause of Hypophysitis Secondary to Toxic Exposure and Review on Toluene Inhalation Endocrine Effects.
    Endocrine, metabolic & immune disorders drug targets· 2025· PMID 39318010mais citado
  5. A case of central diabetes insipidus after COVID-19 as a probable diagnosis of lymphocytic infundibulo-neurohypophysitis with positive anti-rabphilin-3A antibodies with review of literature.
    Endocrine journal· 2024· PMID 39198191mais citado
  6. A Case of Arginine Vasopressin Deficiency with Anti-rabphilin-3A Antibody Positivity Following an Influenza Infection.
    Intern Med· 2026· PMID 41882896recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:238305(Orphanet)
  2. MONDO:0016534(MONDO)
  3. GARD:20632(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55786290(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

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Infundíbulo-neuro-hipofisite

ORPHA:238305 · MONDO:0016534
CID-10
E23.6 · Outros transtornos da hipófise
CID-11
MedGen
UMLS
C5190834
EuropePMC
Wikidata
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