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Frontiers in Endocrinology 2023To investigate the prevalence of low blood testosterone level (LTL) and its determinant factors among active male acromegaly patients, as well as the effect of surgery...
PURPOSE
To investigate the prevalence of low blood testosterone level (LTL) and its determinant factors among active male acromegaly patients, as well as the effect of surgery on LTL in male acromegaly patients.
METHODS
A retrospective, single-center study focused on 252 male acromegaly patients aged 18 years-60 years diagnosed in the Peking Union Medical College Hospital from January 2015 to December 2018 was carried out. The measurements of preoperative and postoperative testosterone levels, serum growth hormone (GH), insulin-like growth factor 1 (IGF-1), and other clinical data were analyzed.
RESULTS
Forty per cent of subjects included were diagnosed with LTL pre surgery. Patients were divided into normal testosterone level (NTL) and LTL groups based on their testosterone level. There were significant differences ( < 0.01) between groups in the presence of macroadenomas, invasion of the cavernous sinus, compression of the optic chiasm, and serum GH and prolactin levels pre surgery. Invasion of the cavernous sinus [odds ratio (OR) = 4.299; = 0.000] and serum prolactin level (OR = 1.023, = 0.001) were independent predictors of LTLs in male patients before surgical intervention. A total of 67.9% of LTL patients recovered during the follow-up, with a new-onset rate of 3.4%. Body mass index, invasion of the cavernous sinus, GH, IGF-1, and prolactin levels, the presence of a prolactin-secreting tumor, and recovery from acromegaly were significantly different ( < 0.05) in the NTL group and in the LTL group during the follow-up. The presence of a prolactin-secreting tumor (OR = 0.224; = 0.001) and recovery from acromegaly (OR = 0.168; = 0.006) were independent predictors of LTLs in male acromegaly patients during the follow-up.
CONCLUSION
The invasiveness of tumor and levels of blood prolactin are independent factors for LTLs before surgery, whereas GH and IGF-1 levels are not. Most male patients can recover from LTL after tumor restriction surgery: those who recover from acromegaly have a better chance of recovering from LTL.
Topics: Humans; Male; Acromegaly; Insulin-Like Growth Factor I; Prospective Studies; Prolactin; Retrospective Studies; Pituitary Neoplasms; Testosterone
PubMed: 37886642
DOI: 10.3389/fendo.2023.1259529 -
Journal of Medical Cases Jul 2023Wolfram syndrome (WS) is a rare neurodegenerative and genetic disorder, also known by the synonym DIDMOAD, which stands for diabetes insipidus (DI), childhood-onset...
Wolfram syndrome (WS) is a rare neurodegenerative and genetic disorder, also known by the synonym DIDMOAD, which stands for diabetes insipidus (DI), childhood-onset diabetes mellitus (DM), optic atrophy (OA), and deafness (D). We present a case of a 25-year-old diabetic patient, using insulin for 15 years, who had increasing polyuria and polydipsia, along with progressive hearing and vision loss. Laboratory tests revealed elevated hemoglobin A1c (HbA1c) and blood sugar levels. Optic nerve, optic chiasm, pons, and brain stem atrophy was seen on magnetic resonance imaging (MRI) of brain. After workup, a diagnosis of DI was made. Once the diagnosis was reached, treatment with subcutaneous insulin and nasal desmopressin improved patient's symptoms. In juvenile diabetic patients presenting with new onset or worsening polyuria and polydipsia, the possibility of WS should be considered. Early diagnosis and initiation of appropriate management leads to improved outcomes and the quality of life.
PubMed: 37560547
DOI: 10.14740/jmc4128 -
Multiple Sclerosis (Houndmills,... May 2024Optic neuritis (ON) is a common feature of inflammatory demyelinating diseases (IDDs) such as multiple sclerosis (MS), aquaporin 4-antibody neuromyelitis optica spectrum...
Optic chiasm involvement in multiple sclerosis, aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein-associated disease.
BACKGROUND
Optic neuritis (ON) is a common feature of inflammatory demyelinating diseases (IDDs) such as multiple sclerosis (MS), aquaporin 4-antibody neuromyelitis optica spectrum disorder (AQP4 + NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). However, the involvement of the optic chiasm (OC) in IDD has not been fully investigated.
AIMS
To examine OC differences in non-acute IDD patients with (ON+) and without ON (ON-) using magnetisation transfer ratio (MTR), to compare differences between MS, AQP4 + NMOSD and MOGAD and understand their associations with other neuro-ophthalmological markers.
METHODS
Twenty-eight relapsing-remitting multiple sclerosis (RRMS), 24 AQP4 + NMOSD, 28 MOGAD patients and 32 healthy controls (HCs) underwent clinical evaluation, MRI and optical coherence tomography (OCT) scan. Multivariable linear regression models were applied.
RESULTS
ON + IDD patients showed lower OC MTR than HCs (28.87 ± 4.58 vs 31.65 ± 4.93; = 0.004). When compared with HCs, lower OC MTR was found in ON + AQP4 + NMOSD (28.55 ± 4.18 vs 31.65 ± 4.93; = 0.020) and MOGAD (28.73 ± 4.99 vs 31.65 ± 4.93; = 0.007) and in ON- AQP4 + NMOSD (28.37 ± 7.27 vs 31.65 ± 4.93; = 0.035). ON+ RRMS had lower MTR than ON- RRMS (28.87 ± 4.58 vs 30.99 ± 4.76; = 0.038). Lower OC MTR was associated with higher number of ON (regression coefficient (RC) = -1.15, 95% confidence interval (CI) = -1.819 to -0.490, = 0.001), worse visual acuity (RC = -0.026, 95% CI = -0.041 to -0.011, = 0.001) and lower peripapillary retinal nerve fibre layer (pRNFL) thickness (RC = 1.129, 95% CI = 0.199 to 2.059, = 0.018) when considering the whole IDD group.
CONCLUSION
OC microstructural damage indicates prior ON in IDD and is linked to reduced vision and thinner pRNFL.
Topics: Adult; Female; Humans; Male; Middle Aged; Aquaporin 4; Autoantibodies; Magnetic Resonance Imaging; Multiple Sclerosis, Relapsing-Remitting; Myelin-Oligodendrocyte Glycoprotein; Neuromyelitis Optica; Optic Chiasm; Optic Neuritis; Tomography, Optical Coherence; Young Adult
PubMed: 38646958
DOI: 10.1177/13524585241240420 -
Frontiers in Neuroscience 2023Xanthogranulomas are considered rare tumors, with their sellar and non-sellar frequency ranging from 1.6 to 7% among intracranial lesions, and described as a separate...
Xanthogranulomas are considered rare tumors, with their sellar and non-sellar frequency ranging from 1.6 to 7% among intracranial lesions, and described as a separate entity by the World Health Organization in 2000. The diagnosis of sellar xanthogranulomas is challenging, given their uncertain origin and clinical course. In addition, the limited reporting of sellar xanthogranuloma cases and the absence of characteristic images make these entities difficult to distinguish from other cystic lesions of the sellar region, such as adamantinomatous craniopharyngiomas, Rathke's cleft cysts, pituitary tumors, arachnoid cysts, epidermoid cysts, and dermoid cysts. Here, we describe the clinical presentation, radiological findings, immunohistochemical/histopathological analysis, and the ultrastructural examination by transmission electron microscopy of five sellar xanthogranulomas cases reported in two care centers in Cordoba, Argentina. Two males and three females between 37 and 73 years of age (average 51.8 years) presented with persistent headaches, generalized endocrine defects, and visual problems. MRI revealed cystic formations in the sellar region, which usually projected into adjacent tissues such as the suprasellar region or cavernous sinuses, and compressed other structures such as the optic chiasm, pituitary gland, and cranial nerves. All patients underwent surgical intervention to remove the tumor tissue. The histopathological analysis of the samples showed cellular tissue with a xanthogranulomatous appearance, inflammatory cellular infiltrate (mainly lymphocytes and macrophages), fibroblasts, abundant collagen fibers, and hemorrhages. An ultrastructural analysis helped to identify cellular infiltrates and granules resulting from tumor cell activity. The data support the hypothesis that sellar xanthogranulomas could occur as an inflammatory reaction secondary to the rupture and hemorrhage of a previous cystic process, thereby generating an expansion of the tumor body toward adjacent tissues. The information obtained from these cases contributes to the current knowledge about this disease's origin and clinical and histological evolution. However, the scarcity of patients and the observed phenotypic heterogeneity make its diagnosis still challenging. Undoubtedly, more investigations are needed to provide additional information in order to be able to achieve a more accurate diagnosis and effective treatment of this rare disease.
PubMed: 37811322
DOI: 10.3389/fnins.2023.1227144 -
Radiation Oncology (London, England) Aug 2023Children with brain tumors are at high risk of neurocognitive decline after radiotherapy (RT). However, there is a lack of studies on how RT doses to organs at risk...
BACKGROUND
Children with brain tumors are at high risk of neurocognitive decline after radiotherapy (RT). However, there is a lack of studies on how RT doses to organs at risk (OARs) impacts neurocognition. The aim of this study was to examine dose-risk relationships for mean RT dose to different brain structures important for neurocognitive networks. We explored previously established OARs and potentially new OARs.
METHODS
A sample of 44 pediatric brain tumor survivors who had received proton and/or photon RT were included. Correlations between mean RT doses to OARs and IQ were analyzed. Previously established OARs were cochleae, optic chiasm, optic nerve, pituitary gland, hypothalamus, hippocampus and pons. Potential new OARs for RT-induced neurocognitive decline were cerebellum, vermis and thalamus.
RESULTS
Mean RT dose to different OARs correlated with several IQ subtests. Higher mean RT dose to cochleae, optic nerve, cerebellum, vermis and pons was correlated with lower performance on particularly full-scale IQ (FIQ), Perceptual Reasoning (PRI), Working Memory (WMI) and Processing Speed Index (PSI). Higher mean RT dose to hippocampus correlated with lower performance on processing speed and working memory. For those receiving whole brain RT (WBRT), higher mean RT dose to the pituitary gland correlated with lower performance on working memory.
CONCLUSION
A high dose-risk correlation was found between IQ subtests and mean RT dose in established and potential new OARs. Thus, in the lack of validated dose constraints for vulnerable brain structures, a parsimonious approach in RT planning should be considered to preserve neurocognitive networks.
Topics: Child; Humans; Organs at Risk; Radiotherapy Dosage; Radiotherapy Planning, Computer-Assisted; Brain; Brain Neoplasms; Radiotherapy, Intensity-Modulated
PubMed: 37568180
DOI: 10.1186/s13014-023-02324-2 -
JCEM Case Reports Jul 2023Hypopituitarism due to an internal carotid artery (ICA) aneurysm is rare. We present a case of hypopituitarism and hyperprolactinemia due to a giant right ICA aneurysm....
Hypopituitarism due to an internal carotid artery (ICA) aneurysm is rare. We present a case of hypopituitarism and hyperprolactinemia due to a giant right ICA aneurysm. A 56-year-old woman with a history of primary hypothyroidism presented with fatigue, right-sided headache, and blurred vision. Magnetic resonance (MR) of the brain revealed a sellar mass measuring 3.5 × 2.2 cm involving the right cavernous sinus. Initial neurologic examination was unremarkable, and her biochemical evaluation revealed secondary adrenal insufficiency, central hypogonadism, low serum free thyroxine, and mildly elevated serum prolactin, consistent with stalk effect. Hydrocortisone therapy was started for secondary adrenal insufficiency and her levothyroxine dose was adjusted. The patient was referred to neurosurgery for surgical management of her sellar lesion. Preoperative computed tomography angiography (CTA) of the brain revealed a right ICA aneurysm that contacted the optic chiasm and displaced the pituitary gland. The aneurysm was embolized and diverting stents were placed. Repeat laboratory tests showed resolution of the patient's secondary adrenal insufficiency, normalization of serum prolactin, and an increase in serum gonadotropin concentrations to the postmenopausal range. This case highlights that not all sellar lesions are pituitary adenomas, and CTA should be performed in the evaluation of large cavernous sinus lesions to exclude ICA aneurysm.
PubMed: 37908996
DOI: 10.1210/jcemcr/luad076 -
Physics and Imaging in Radiation... Oct 2023Tools for auto-segmentation in radiotherapy are widely available, but guidelines for clinical implementation are missing. The goal was to develop a workflow for...
BACKGROUND AND PURPOSE
Tools for auto-segmentation in radiotherapy are widely available, but guidelines for clinical implementation are missing. The goal was to develop a workflow for performance evaluation of three commercial auto-segmentation tools to select one candidate for clinical implementation.
MATERIALS AND METHODS
One hundred patients with six treatment sites (brain, head-and-neck, thorax, abdomen, and pelvis) were included. Three sets of AI-based contours for organs-at-risk (OAR) generated by three software tools and manually drawn expert contours were blindly rated for contouring accuracy. The dice similarity coefficient (DSC), the Hausdorff distance, and a dose/volume evaluation based on the recalculation of the original treatment plan were assessed. Statistically significant differences were tested using the Kruskal-Wallis test and the post-hoc Dunn Test with Bonferroni correction.
RESULTS
The mean DSC scores compared to expert contours for all OARs combined were 0.80 ± 0.10, 0.75 ± 0.10, and 0.74 ± 0.11 for the three software tools. Physicians' rating identified equivalent or superior performance of some AI-based contours in head (eye, lens, optic nerve, brain, chiasm), thorax (e.g., heart and lungs), and pelvis and abdomen (e.g., kidney, femoral head) compared to manual contours. For some OARs, the AI models provided results requiring only minor corrections. Bowel-bag and stomach were not fit for direct use. During the interdisciplinary discussion, the physicians' rating was considered the most relevant.
CONCLUSION
A comprehensive method for evaluation and clinical implementation of commercially available auto-segmentation software was developed. The in-depth analysis yielded clear instructions for clinical use within the radiotherapy department.
PubMed: 38111502
DOI: 10.1016/j.phro.2023.100515 -
Journal of Neuroscience Methods Jan 2024Blood perfusion of the optic nerve (ON) plays a key role in many optic neuropathies. Microvascular changes precede or accompany neuronal changes, and detecting these...
BACKGROUND
Blood perfusion of the optic nerve (ON) plays a key role in many optic neuropathies. Microvascular changes precede or accompany neuronal changes, and detecting these changes at an early stage may facilitate early treatment to avoid blindness. However, the quantification of ON blood perfusion remains a challenge. This study aimed to evaluate the viability of three-dimensional pseudocontinuous arterial spin labelling (3D-pCASL) MRI for the quantification of ON blood flow (BF).
NEW METHOD
The ON segmentation was performed using nnFormer on a cohort of ten participants (4 males, 6 females, 25-59 years old). Subsequently, the mean BF of each ON segment was calculated using whole brain 3D-pCASL image data.
RESULTS
The average ON-BF values of the left and right intraorbital segments, left and right intracanalicular segments, left and right intracranial segments, optic chiasma, and left and right optic tract were 41.308 mL/100 g/min, 43.281 mL/100 g/min, 53.188 mL/100 g/min, 57.202 mL/100 g/min, 45.089 mL/100 g/min, 49.554 mL/100 g/min, 42. 326 mL/100 g/min, 43.831 mL/100 g/min and 45.176 mL/100 g/min, respectively. The ON-BF correlated with cerebral BF (r = 0.503, p = 0.024).
COMPARISON WITH EXISTING METHOD(S)
The 3D-pCASL can measure tissue microvascular blood perfusion in absolute quantitative units with good test-retest repeatability over a wide field of view and without restrictions on depth. The use of the nnFormer makes the measurement easy, objective and reproducible.
CONCLUSIONS
The study showed that, 3D-pCASL may be a promising tool for detecting abnormal ON-BF. In particular, 3D-pCASL coupled with the nnFormer provides an objective, reproducible, and reliable method to quantify BF in ON.
Topics: Male; Female; Humans; Adult; Middle Aged; Magnetic Resonance Angiography; Spin Labels; Magnetic Resonance Imaging; Cerebrovascular Circulation; Optic Nerve
PubMed: 37952831
DOI: 10.1016/j.jneumeth.2023.110007 -
Journal of Neurosurgery. Case Lessons Feb 2024A cavernous malformation of the optic nerve (CMON) is a rare condition that often presents with an abrupt decline in vision. Acute management of ruptured optic nerve...
BACKGROUND
A cavernous malformation of the optic nerve (CMON) is a rare condition that often presents with an abrupt decline in vision. Acute management of ruptured optic nerve cavernous malformations is generally surgical, although the timing of surgery is controversial.
OBSERVATIONS
A 47-year-old female experienced the sudden loss of vision in her left eye. Examination showed that this eye was nearly blind, and her right eye had a temporal field defect. Neuroimaging showed hemorrhage in her left optic nerve and optic chiasm. She was taken to the operating room on an emergent basis where the optic canal was decompressed, the hemorrhage was evacuated, and a vascular malformation with features of a cavernoma was removed from the optic nerve. Over the next 2 days, the vision in her right eye significantly recovered.
LESSONS
CMONs remain rare, and it is unlikely that enough cases can be gathered to form a larger trial to compare the role and timing of surgery. On the basis of our experience with this case, the authors recommend that acute CMON-related hematomas should be treated as a surgical emergency and managed with acute optic nerve decompression, hematoma evacuation, and cavernoma resection to improve chances of vision recovery and prevent further vision loss.
PubMed: 38408352
DOI: 10.3171/CASE23746 -
Cureus Dec 2023Objective This study aims to thoroughly assess the radiation dose distribution to critical organs in patients with nasopharyngeal carcinoma, focusing on the correlation...
Objective This study aims to thoroughly assess the radiation dose distribution to critical organs in patients with nasopharyngeal carcinoma, focusing on the correlation between the radiation dosages for the various organs at risk (OARs) in nasopharyngeal cancer patients. Methods We meticulously analysed a dataset comprising 38 nasopharyngeal carcinoma patients, focusing on radiation dosages measured in Gray (Gy) and volumetric data in cubic centimetres (cc) of critical organs, including the lens, brainstem, spinal cord, optic nerve, optic chiasm, and cochlea. A detailed exploratory data analysis approach encompassed univariate, bivariate, and multivariate techniques. Results Our analysis revealed several key findings. The mean and median values across various dose measurements were closely aligned, indicating symmetrical distributions with minimal skewness. The histograms further corroborated this, showing evenly distributed dose values across different anatomical regions. The correlation matrix highlighted varying degrees of interrelationships between the doses, with some showing strong correlations while others exhibited minimal or no correlation. The 3D scatter plot provided a view of the multi-dimensional dose relationships, with a specific focus on the spinal cord, lens, and brainstem doses. The bivariate scatter plots revealed symmetrical distributions between the right and left lens doses and more complex relationships involving the brainstem and spinal cord, illustrating the intricacies of dose distribution in radiation therapy. Conclusion Our findings reveal distinct radiation exposure patterns to OARs of nasopharyngeal carcinoma. This research emphasises the need for tailored radiation therapy planning to achieve optimal clinical outcomes while safeguarding vital organs.
PubMed: 38053989
DOI: 10.7759/cureus.49882