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Cureus Aug 2023Sarcoidosis is an inflammatory condition that can impact multiple organs in the body such as the lungs, skin, eyes, and, occasionally, the central nervous system. When...
Sarcoidosis is an inflammatory condition that can impact multiple organs in the body such as the lungs, skin, eyes, and, occasionally, the central nervous system. When sarcoidosis affects the nervous system, it is referred to as neurosarcoidosis and is estimated to occur in approximately 5%-15% of sarcoid patients. When neurosarcoidosis affects the pituitary gland, it can result in panhypopituitarism, which can be life-threatening. A 35-year-old male with a known diagnosis of sarcoidosis by skin biopsies presented to the hospital with altered mental status, hypernatremia, hypotension, and hypothermia. He reported symptoms of polyuria and polydipsia for several weeks before admission. Laboratory workup revealed elevated serum sodium at 167 mmol/L, high serum osmolality at 381 mOsm/kg, and low urine osmolality at 381 mOsm/kg, consistent with diabetes insipidus. Anterior pituitary hormone profile workup revealed low 8 am serum cortisol (1.9 mcg/dL) and inappropriately normal adrenocorticotropic hormone (ACTH) (34 pg/ml), low serum free testosterone (<2.5 ng/dL), low luteinizing hormone (0.7 mIU/ml), low follicular stimulating hormone (< 2.6 mIU/ml), low free T4 at 0.4 ng/dL. and inappropriately normal thyroid-stimulating hormone (TSH) at 2.77 uIU/mL. Serum prolactin was mildly elevated at 86.8 ng/mL. Angiotensin-converting enzyme level was within the normal range at 33 U/L. A diagnosis of panhypopituitarism was made. Brain MRI revealed a 3 cm mass in the suprasellar region involving the hypothalamus and bilateral optic tracts with a mass effect on the anterior third ventricle. No discrete pituitary or stalk lesion was identified. A ventriculostomy tube was placed for developing hydrocephalus. A biopsy of the suprasellar mass revealed non-caseating granuloma, confirming neurosarcoidosis. Treatment was initiated with high-dose IV corticosteroids to manage secondary adrenal insufficiency and neurosarcoidosis. He was also started on IV desmopressin and IV levothyroxine to manage his diabetes insipidus and central hypothyroidism. He was transitioned to oral therapy upon discharge. Panhypopituitarism secondary to neurosarcoidosis is a rare presentation that can occur due to the infiltration of the pituitary gland or the infiltration of the hypothalamus affecting the hypothalamic-pituitary axis. Neurosarcoidosis should be considered a differential when evaluating patients with symptoms consistent with panhypopituitarism. Prompt diagnosis and initiation of corticosteroids and deficient hormones can be lifesaving.
PubMed: 37692696
DOI: 10.7759/cureus.43169 -
JAAD Case Reports Sep 2023
PubMed: 37680570
DOI: 10.1016/j.jdcr.2023.07.003 -
The Journal of the Royal College of... Dec 2023Infection is a rare cause of panhypopituitarism and has not been reported in the context of Lemierre's syndrome. We present the case of a previously well 19-year-old...
Infection is a rare cause of panhypopituitarism and has not been reported in the context of Lemierre's syndrome. We present the case of a previously well 19-year-old man, who presented acutely unwell with meningitis and sepsis. was isolated from peripheral blood cultures and identified on cerebrospinal fluid with 16S rDNA Polymerase Chain Reaction (PCR). Imaging demonstrated internal jugular vein thrombosis with subsequent cavernous venous sinus thrombosis. Pituitary function tests were suggestive of panhypopituitarism. The patient was diagnosed with Lemierre's syndrome complicated by meningitis, cavernous sinus thrombosis, base of skull osteomyelitis, ischaemic stroke and panhypopituitarism. He was treated with 13 weeks of intravenous antibiotics followed by 3 weeks of oral amoxicillin, and anticoagulated with dalteparin then apixaban. His panhypopituitarism was managed with hydrocortisone, levothyroxine and desmopressin.
Topics: Male; Humans; Young Adult; Adult; Lemierre Syndrome; Cavernous Sinus Thrombosis; Brain Ischemia; Stroke; Pulmonary Embolism; Ischemic Stroke; Meningitis
PubMed: 37675957
DOI: 10.1177/14782715231198180 -
Neurosurgical Review Sep 2023The incidence of unplanned reoperation after surgery during the same hospitalization is considered one of most important evaluation indicators for health care quality....
The incidence of unplanned reoperation after surgery during the same hospitalization is considered one of most important evaluation indicators for health care quality. The purpose of this study was to determine the incidence and risk factors related to unplanned reoperation after an endoscopic endonasal approach (EEA). All patients who underwent elective endoscopic endonasal surgery from January 2016 to December 2021 in the Department of Neurosurgery, Tangdu Hospital, Air Force Military Medical University, were included. We identified the patients who underwent an unplanned reoperation and those who did not and divided them into two groups. The demographic data and risk factors were compared between the groups by univariate and multivariate logistic regression analyses. Of the 1783 patients undergoing EEA for various lesions of the skull base, the incidence of unplanned reoperation was 2.3%. The most common unplanned reoperations were repair of cerebrospinal fluid (CSF) leakage (39%), sellar hematoma evacuation (34.1%), hemostasis of epistaxis (14.6%) and external ventricular drainage for obstructive hydrocephalus (9.8%). The maximum diameter of tumor ≥ 3 cm (OR 2.654, CI 1.236-5.698; p = 0.012), meningioma (OR 4.198, CI 1.169-15.072; p = 0.028), craniopharyngioma (OR 5.020, CI 2.020-12.476; p = 0.001) and other sellar lesions (OR 4.336, CI 1.390-13.527; p = 0.012) and an operation time ≥ 240 min (OR 2.299, CI 1.170-4.518; p = 0.016) were the independent risk factors for unplanned reoperations in multivariate regression analysis. Of the 41 patients undergoing unplanned reoperation, 16 patients died, twenty-one patients had panhypopituitarism, 13 patients had transient and 6 had permanent diabetes insipidus, and 11 patients presented with intracranial infection and 6 of these patients were cured. By reviewing our department's data, we stated the incidence and risk factors for unplanned reoperation. It is important for the hospital administration and neurosurgeons to place more emphasis on these indicators. Furthermore, we suggest some effective quality improvement initiatives to reduce the incidence of unplanned reoperation.
Topics: Humans; Reoperation; Incidence; Endoscopy; Cerebrospinal Fluid Leak; Pituitary Neoplasms; Meningeal Neoplasms
PubMed: 37665381
DOI: 10.1007/s10143-023-02134-w -
Annals of Medicine and Surgery (2012) Aug 2023Munchausen syndrome is known as a factitious disorder imposed on the self. Factitious Cushing's syndrome (CS) is a very rare form of Munchausen syndrome, presenting with...
UNLABELLED
Munchausen syndrome is known as a factitious disorder imposed on the self. Factitious Cushing's syndrome (CS) is a very rare form of Munchausen syndrome, presenting with varied clinical and biochemical features, making diagnosis challenging.
CASE PRESENTATION
A 40-year-old female patient who worked as a registered nurse presented with clinical features of CS but denied any exogenous corticosteroid use. The endocrine workup revealed that the patient had a high 24 h urinary-free cortisol collection before admission. Subsequent evaluations showed low levels of morning cortisol and plasma adrenocorticotropic hormone along with a suppressed overnight low-dose dexamethasone suppression test, leading to an investigation of hypercortisolism. Unexpectedly, subsequent testing showed a normal 24 h urinary-free cortisol level. Additionally, the patient was diagnosed with panhypopituitarism, the radiological investigations showed normal pituitary and adrenal glands. Despite consistently denying the use of corticosteroids, it was finally discovered that the patient had been surreptitiously taking prednisone and receiving multiple dexamethasone injections over the past few months. The patient received treatment through a gradual prednisone tapering regimen, accompanied by comprehensive psychiatric evaluation and management.
CONCLUSION
This case underscores the exceptional rarity of factitious CS and emphasizes the importance of considering it as a potential differential diagnosis in hypercortisolism cases, particularly when the patient's medical history contradicts investigative findings. Furthermore, it highlights the criticality of adopting a multidisciplinary approach to investigate patients whose clinical presentation aligns with factitious CS.
PubMed: 37554867
DOI: 10.1097/MS9.0000000000001050 -
Brain Tumor Research and Treatment Jul 2023The World Health Organization (WHO) updated the classification of pituitary tumors in 2022. The new classification presents detailed histological subtyping of a... (Review)
Review
The World Health Organization (WHO) updated the classification of pituitary tumors in 2022. The new classification presents detailed histological subtyping of a pituitary neuroendocrine tumor (PitNET) based on the tumor cell lineage, cell type, and related characteristics. The immunohistochemistry for pituitary transcription factors (PIT1, TPIT, SF1, GATA3, and ERα) is routinely needed in this classification. The controversy regarding the change of behavior code of all PitNET/pituitary adenoma from "0" for benign tumors to "3" for primary malignant tumors is a topic of debate among experts, nowadays. Some authors represent that pituitary adenoma has a tendency for hemorrhage and necrosis and frequent invasion of the cavernous sinus. However, most small PitNET/pituitary adenoma do not need any treatment because of benign biologic behavior or less than 5% recurrence after gross total removal. Pituitary apoplexy is also benign nature but has a tendency of cranial nerve compression or panhypopituitarism. Most of cavernous invasion is compression of the cavernous sinus. Aggressive PitNET/pituitary adenoma with malignant biological behavior is less than 1%.
PubMed: 37550816
DOI: 10.14791/btrt.2023.0015 -
Radiology Case Reports Oct 2023A 38-year-old man who was delivered in a breech position presented with delayed development of secondary sexual characteristics and malaise. He was diagnosed with...
A 38-year-old man who was delivered in a breech position presented with delayed development of secondary sexual characteristics and malaise. He was diagnosed with panhypopituitarism caused by interruption of the pituitary stalk due to perinatal complications. Brain magnetic resonance imaging findings for pituitary stalk interruption syndrome are well-documented; however, reports of the imaging findings of the bones and several organs related to the effects of panhypopituitarism are limited. In this patient with anterior pituitary dysfunction, imaging revealed diverse sequelae, including delayed skeletal maturation, osteopenia, genital atrophy, fatty liver, and adrenal atrophy. Radiologists may find it difficult to discern complex imaging findings unless they are informed of the clinical course of the patient. Therefore, radiologists should coordinate with clinicians to arrive at a diagnosis.
PubMed: 37547790
DOI: 10.1016/j.radcr.2023.07.039 -
SAGE Open Medical Case Reports 2023Hypogonadotropic hypogonadism is a common finding in patients who are diagnosed with a prolactinoma. It can be accompanied by the presence of other pituitary hormone...
Hypogonadotropic hypogonadism is a common finding in patients who are diagnosed with a prolactinoma. It can be accompanied by the presence of other pituitary hormone deficits, including secondary adrenal insufficiency and central hypothyroidism. While the proportion of improvement in endocrine deficits over the short term is well characterized, there is not enough literature about the recovery of pituitary function over the longer term. We present the case of a 23-year-old man with a giant prolactinoma who initially presented with pituitary hemorrhage and panhypopituitarism. He underwent decompression of the pituitary tumor followed by treatment with cabergoline. Over a 9-year follow-up period, we noted that the hypogonadotropic hypogonadism resolved after 4 years and the secondary adrenal insufficiency resolved after 8 years. This case suggests that partial or complete recovery of the pituitary function is possible over the long-term even in patients with a giant prolactinoma.
PubMed: 37533485
DOI: 10.1177/2050313X231190672 -
Endocrine Connections Oct 2023Hypothalamic dysfunction is a rare condition and can be encountered in patients who have been diagnosed or treated for a suprasellar brain tumor. Due to its rarity, the...
OBJECTIVE
Hypothalamic dysfunction is a rare condition and can be encountered in patients who have been diagnosed or treated for a suprasellar brain tumor. Due to its rarity, the signs and symptoms of hypothalamic dysfunction may be difficult to recognize, leading to delayed diagnosis of the suprasellar brain tumor or to difficulties in finding the health-care expertise for hypothalamic dysfunction after tumor treatment. To improve the care and outcome of patients with acquired hypothalamic dysfunction, professionals are required to understand the patient's needs.
DESIGN
A worldwide online survey was distributed from April 2022 to October 2022 to patients with childhood-onset hypothalamic dysfunction (as reported by the patient) following a brain tumor.
METHODS
Patients were notified about the survey through patient advocacy groups, the SIOPe craniopharyngioma working group and the Endo-ERN platform.
RESULTS
In total, 353 patients with hypothalamic dysfunction following craniopharyngioma (82.2%), low-grade glioma (3.1%) or a pituitary tumor (8.2%) or caregivers responded to the survey. Sixty-two percent had panhypopituitarism. Obesity (50.7%) and fatigue (48.2%) were considered the most important health problems. Unmet needs were reported for help with diet, exercise and psychosocial issues. Patients' suggestions for future research include new treatments for hypothalamic obesity and alternative ways for hormone administration.
CONCLUSIONS
According to the patient's perspective, care for acquired hypothalamic dysfunction can be improved if delivered by experts with a holistic view of the patient in a multidisciplinary setting with a focus on quality of life. Future care and research on hypothalamic dysfunction must integrate the patients' unmet needs.
SIGNIFICANCE STATEMENT
Patients with hypothalamic dysfunction may experience a variety of symptoms, which are not always adequately recognized or addressed. In previous papers, the perspective of caregivers of children with craniopharyngioma has been reported (Klages et al. 2022, Craven et al. 2022). Now we address the patients' perspective on acquired hypothalamic dysfunction using an Endo-ERN global survey. According to the patients' perspective, care can be improved, with needs for improvement in the domains of obesity, fatigue and lifestyle. Research may focus on ways to improve hypothalamic obesity and alternative ways for hormone administration. Ideally, care should be delivered by doctors who have a holistic view of the patient in a multidisciplinary expert team. The results of this study can be used to formulate best practices for clinical care and to design future research proposals.
PubMed: 37531603
DOI: 10.1530/EC-23-0147 -
Journal of Medical Cases Jun 2023Hyperbilirubinemia and transaminitis are rarely associated with a disorder of endocrine function. It mostly manifests as a cholestatic pattern of liver injury. Herein, a...
Hyperbilirubinemia and transaminitis are rarely associated with a disorder of endocrine function. It mostly manifests as a cholestatic pattern of liver injury. Herein, a 25-year-old female patient with a past medical history of congenital hypopituitarism due to pituitary ectopia presented with serum direct bilirubin level of 9.9 mg/dL and aspartate transaminase (AST)/alanine transaminase (ALT) of 60/47 U/L. All tests for chronic liver disease imaging and liver biopsy were normal. She was found to have central hypothyroidism and low cortisol level. She was started on intravenous (IV) levothyroxine 75 µg daily and IV hydrocortisone 10-5 mg AM/PM. She was discharged on oral levothyroxine 88 µg daily and hydrocortisone orally 10 mg twice daily. Follow-up labs 1 month later showed completely normal liver function test. In conclusion, hyperbilirubinemia due to congenital hypopituitarism can occur in adults. Delayed recognition of underlying endocrine disorder as a cause of hyperbilirubinemia and hepatocellular inflammation can result in end-stage liver damage due to prolonged cholestasis.
PubMed: 37435107
DOI: 10.14740/jmc4102