Objective:To improve the understanding of the clinical manifestation of immunoglobulin G 4-related disease (IgG 4-RD) with neurological involvement. Methods:Patients presenting with neurological symptoms and biopsy-confirmed IgG 4-RD were enrolled between March 2014 and March 2024 from the Department of Neurology of Xuanwu Hospital of Capital Medical University. Medical record data of all patients were retrospectively reviewed, including clinical manifestations, laboratory findings, radiological results, pathology, treatments, and outcomes. RD were enrolled between March Results:Nine patients (five males, four females; median age at onset: 63 years) were included in the study. Neurological manifestations consisted of pachymeningitis in six cases, peripheral nerve involvement in two, and brain parenchyma involvement in one. Four patients displayed isolated neurologic symptoms. The most common clinical manifestations were headache and cranial nerve palsy, each involving five cases. Magnetic resonance imaging showed thickening and enhancement of the dura mater in the six patients with pachymeningitis. Four of these cases involved the posterior cranial fossa, 3 presented with an inflammatory pseudotumor, and 2 involved the spinal cord. Four patients with pachymeningitis had normal serum IgG 4 concentrations. Eight patients exhibited elevated serum C-reactive protein or erythrocyte sedimentation rates, with some also showing decreased complement C3 and C4 levels. Cerebrospinal fluid immunoglobulin was elevated in all nine cases. In all cases, histopathologic biopsy results showed extensive infiltration of lymphocytes and plasma cells, with the latter displaying IgG 4+abnormalities. All patients received glucocorticoid therapy, with six also receiving immunosuppressants. All patients were followed up for a median of 30 months, with outcomes including two complete remissions, five partial remissions, one unchanged condition, and one death. Six patients experienced a relapse. Conclusions:Isolated hypertrophic pachymeningitis is the most common manifestation of IgG 4-RD, often occurring in the absence of elevated serum IgG 4 levels. Peripheral nerve and brain parenchyma involvement can also be seen. Clinical manifestations are non-specific, and histopathologic biopsy is frequently required for diagnosis. Although the disease responds well to hormone treatment, recurrence is common. Early combined immunosuppressive therapy can improve prognosis.

Objective:To investigate the clinical, imaging and neuropathological characteristics of neuronal intranuclear inclusion disease (NIID) with symptoms of the central nervous system, and to improve the diagnosis and treatments of NIID.Methods:The clinical data of 7 patients with NIID diagnosed by brain biopsy in Xuanwu Hospital, Capital Medical University, Beijing, China from February 2009 to December 2024 were collected. The characteristics of clinical manifestations, imaging, and histology on brain biopsy were retrospectively analyzed.Results:Among the 7 patients, 5 were male and 2 were female. Their ages ranged from 44 to 70 years, median 56 (52, 65) years. Patients were classified into three types of tumor, stroke and encephalitis according to the onset symptoms, imaging manifestations and pathological changes. The chief complaint of the 5 patients was headache, while 4 patients had paroxysmal convulsions, 3 had speech disorders, 2 had abnormal mental behaviors, 2 had memory decline, and 1 had fever accompanied by consciousness disorders. Diffusion-weighted magnetic resonance imaging of the head showed the "ribbon sign" at the junction of the cortex and medulla in 2 cases. Most of the patients had white matter lesions, gyrus swelling and cerebral atrophy. Occasionally gyrus-like enhancement was observed. Brain biopsy reveals the histological changes that matched those on images and initial symptoms. There were proliferation of oligodendrocytes and astrocytes in the white matter, leukoaraiosis and edema, cortical disintegration and lamellar necrosis, as well as infiltration of lymphocytes and microglia, etc. However, the characteristic changes were eosinophilic hyaline inclusions in the nuclei of neurons and astrocytes. Immunohistochemical staining of p62 and ubiquitin showed homogeneous staining in round or ring-shaped nuclei.Conclusions:The clinical manifestations of NIID are highly variable, and a correct diagnosis of NIID requires careful integration of clinical, imaging and histopathologic data. For patients with a high suspicion of NIID, immunohistochemical staining of p62 and ubiquitin is diagnostically valuable.

Objective:To investigate the predictive value of computed tomography(CT) based radiomics model for the prognosis of patients with pancreatic ductal adenocarcinoma(PDAC).Methods:The retrospective cohort study was conducted. The clinicopathological data of 206 PDAC patients who were admitted to Zhongshan Hospital of Fudan University from August 2018 to December 2020 were collected. There were 115 males and 91 females, aged (64±9)years. All 206 pati-ents underwent enhanced CT examination. Based on radom number table, the 206 patients were randomly divided into a training set of 165 cases and a validation set of 41 cases with a ratio of 4∶1. The training set was used to construct the prediction model, and the test set was used to validate the performance of the prediction model. Observation indicators: (1) follow-up; (2) analysis of prognostic factors of PDAC patients in the training set; (3) construction and evaluation of prediction model for prognosis of PDAC patients. Comparison of measurement data with normal distribution between groups was conducted using the t test. Comparison of measurement data with skewed distribution between groups was conducted using the Wilcoxon W test. Comparison of count data between groups was conducted using the chi-square test or corrected chi-square test. The Kaplan-Meier method was used to calculate the survival rate and Log-rank test was used for survival analysis. Univariate and multivariate analyses were conducted using the COX regression model. The PyCharm software was used for the least absolute shrinkage and selection operator method (LASSO)-COX regression analysis. The receiver operating characteristic curve was plotted to evaluate the performance of radiomics model. Results:(1)Follow-up. Of the 206 patients,205 cases were followed up for 17.1(range, 12.0?40.1)months. The postoperative 1-, 2-, 3-year survival rates were 80.10%, 29.61% and 4.85%. (2) Analysis of prognostic factors for PDAC patients in the training dataset. Results of multivariate analysis showed that pathological N stage was an independent influencing factor for prognosis of PDAC patients in the training set ( hazard ratio=1.476, 95% confidence interval as 1.054?2.067, P<0.05). (3) Construction and evaluation of prediction model for prognosis of PDAC patients. A total of 1 595 radiomics features were finally extracted from the 206 patients. By intra-group feature selection and dimensionality reduction using LASSO-COX regression model, 10 radiomics features were obtained. Combined with 10 radiomics features and 11 clinical features, using the LASSO-COX regression analysis, 15 features were finally extracted to construct the CT based radiomics model for predicting prognosis of PDAC. The areas under receiver operating characteristic curve of the prediction model in predicting 2-year and 3-year overall survival rates of PDAC patients in the training set were 0.834 (95% confidence interval as 0.777?0.891) and 0.883 (95% confidence interval as 0.834?0.932), respectively. The area under curve of the prediction model for patients in the validation set was 0.606 (95% confidence interval as 0.456?0.756) and 0.625 (95% confidence interval as 0.477?0.773). Conclusion:The prediction model constructed on CT based radiomics features and clinical features for predicting the prognosis of PDAC patients shows a promising prediction efficiency.

Objective To observe the value of 2D SECara-Net and 3D U2-Net models constructed based on 2D maximal intensity projection(MIP)and 3D time-of-flight MR angiography(3D TOF-MRA)images,respectively,also of their combination for MRA detecting unruptured saccular intracranial aneurysms(USIA).Methods Totally 973 patients with single USIA and 300 subjects who underwent healthy physical examination were retrospectively collected and divided into training set(n=923,containing 723 cases of USIA and 200 healthy subjects)and test set(n=350,containing 250 cases of USIA and 100 healthy subjects)at the ratio of 7:3.Pre-processed 3D TOF-MRA and the obtained 2D-MIP images in training set were imported into 3D U2-Net and 2D SECara-Net models for training and adjusting parameters,respectively.The efficiency of 2 models and their combination for detecting USIA were evaluated.Results The sensitivity,specificity and accuracy of 2D SECara-Net model for detecting USIA in test set was 78.80％(197/250),95.00％(95/100)and 83.43％(292/350),of 3D U2-Net model was 82.80％(207/250),86.00％(86/100)and 83.71％(293/350),respectively.The specificity of 2D SECara-Net model was higher than that of 3D U2-Net model(P=0.030),while no significant difference of sensitivity nor accuracy was found between 2 models(both P＞0.05).The specificity of the combination of the 2 models was 99.00％(99/100),higher than that of 3D U2-Net model(P＜0.05),and the sensitivity and accuracy of the combination was 91.20％(228/250)and 93.43％(327/350),respectivelty,both higher than those of 2 single models(all P＜0.05).Conclusion 2D SECara-Net and 3D U2-Net models had similar,sensitivity and accuracy for MRA detecting USIA.Combination of them could improve the detecting efficacy.

Objective To observe the value of 2D SECara-Net and 3D U2-Net models constructed based on 2D maximal intensity projection(MIP)and 3D time-of-flight MR angiography(3D TOF-MRA)images,respectively,also of their combination for MRA detecting unruptured saccular intracranial aneurysms(USIA).Methods Totally 973 patients with single USIA and 300 subjects who underwent healthy physical examination were retrospectively collected and divided into training set(n=923,containing 723 cases of USIA and 200 healthy subjects)and test set(n=350,containing 250 cases of USIA and 100 healthy subjects)at the ratio of 7:3.Pre-processed 3D TOF-MRA and the obtained 2D-MIP images in training set were imported into 3D U2-Net and 2D SECara-Net models for training and adjusting parameters,respectively.The efficiency of 2 models and their combination for detecting USIA were evaluated.Results The sensitivity,specificity and accuracy of 2D SECara-Net model for detecting USIA in test set was 78.80％(197/250),95.00％(95/100)and 83.43％(292/350),of 3D U2-Net model was 82.80％(207/250),86.00％(86/100)and 83.71％(293/350),respectively.The specificity of 2D SECara-Net model was higher than that of 3D U2-Net model(P=0.030),while no significant difference of sensitivity nor accuracy was found between 2 models(both P＞0.05).The specificity of the combination of the 2 models was 99.00％(99/100),higher than that of 3D U2-Net model(P＜0.05),and the sensitivity and accuracy of the combination was 91.20％(228/250)and 93.43％(327/350),respectivelty,both higher than those of 2 single models(all P＜0.05).Conclusion 2D SECara-Net and 3D U2-Net models had similar,sensitivity and accuracy for MRA detecting USIA.Combination of them could improve the detecting efficacy.

Objective:To investigate the predictive value of computed tomography(CT) based radiomics model for the prognosis of patients with pancreatic ductal adenocarcinoma(PDAC).Methods:The retrospective cohort study was conducted. The clinicopathological data of 206 PDAC patients who were admitted to Zhongshan Hospital of Fudan University from August 2018 to December 2020 were collected. There were 115 males and 91 females, aged (64±9)years. All 206 pati-ents underwent enhanced CT examination. Based on radom number table, the 206 patients were randomly divided into a training set of 165 cases and a validation set of 41 cases with a ratio of 4∶1. The training set was used to construct the prediction model, and the test set was used to validate the performance of the prediction model. Observation indicators: (1) follow-up; (2) analysis of prognostic factors of PDAC patients in the training set; (3) construction and evaluation of prediction model for prognosis of PDAC patients. Comparison of measurement data with normal distribution between groups was conducted using the t test. Comparison of measurement data with skewed distribution between groups was conducted using the Wilcoxon W test. Comparison of count data between groups was conducted using the chi-square test or corrected chi-square test. The Kaplan-Meier method was used to calculate the survival rate and Log-rank test was used for survival analysis. Univariate and multivariate analyses were conducted using the COX regression model. The PyCharm software was used for the least absolute shrinkage and selection operator method (LASSO)-COX regression analysis. The receiver operating characteristic curve was plotted to evaluate the performance of radiomics model. Results:(1)Follow-up. Of the 206 patients,205 cases were followed up for 17.1(range, 12.0?40.1)months. The postoperative 1-, 2-, 3-year survival rates were 80.10%, 29.61% and 4.85%. (2) Analysis of prognostic factors for PDAC patients in the training dataset. Results of multivariate analysis showed that pathological N stage was an independent influencing factor for prognosis of PDAC patients in the training set ( hazard ratio=1.476, 95% confidence interval as 1.054?2.067, P<0.05). (3) Construction and evaluation of prediction model for prognosis of PDAC patients. A total of 1 595 radiomics features were finally extracted from the 206 patients. By intra-group feature selection and dimensionality reduction using LASSO-COX regression model, 10 radiomics features were obtained. Combined with 10 radiomics features and 11 clinical features, using the LASSO-COX regression analysis, 15 features were finally extracted to construct the CT based radiomics model for predicting prognosis of PDAC. The areas under receiver operating characteristic curve of the prediction model in predicting 2-year and 3-year overall survival rates of PDAC patients in the training set were 0.834 (95% confidence interval as 0.777?0.891) and 0.883 (95% confidence interval as 0.834?0.932), respectively. The area under curve of the prediction model for patients in the validation set was 0.606 (95% confidence interval as 0.456?0.756) and 0.625 (95% confidence interval as 0.477?0.773). Conclusion:The prediction model constructed on CT based radiomics features and clinical features for predicting the prognosis of PDAC patients shows a promising prediction efficiency.

Objective:To improve the understanding of the clinical manifestation of immunoglobulin G 4-related disease (IgG 4-RD) with neurological involvement. Methods:Patients presenting with neurological symptoms and biopsy-confirmed IgG 4-RD were enrolled between March 2014 and March 2024 from the Department of Neurology of Xuanwu Hospital of Capital Medical University. Medical record data of all patients were retrospectively reviewed, including clinical manifestations, laboratory findings, radiological results, pathology, treatments, and outcomes. RD were enrolled between March Results:Nine patients (five males, four females; median age at onset: 63 years) were included in the study. Neurological manifestations consisted of pachymeningitis in six cases, peripheral nerve involvement in two, and brain parenchyma involvement in one. Four patients displayed isolated neurologic symptoms. The most common clinical manifestations were headache and cranial nerve palsy, each involving five cases. Magnetic resonance imaging showed thickening and enhancement of the dura mater in the six patients with pachymeningitis. Four of these cases involved the posterior cranial fossa, 3 presented with an inflammatory pseudotumor, and 2 involved the spinal cord. Four patients with pachymeningitis had normal serum IgG 4 concentrations. Eight patients exhibited elevated serum C-reactive protein or erythrocyte sedimentation rates, with some also showing decreased complement C3 and C4 levels. Cerebrospinal fluid immunoglobulin was elevated in all nine cases. In all cases, histopathologic biopsy results showed extensive infiltration of lymphocytes and plasma cells, with the latter displaying IgG 4+abnormalities. All patients received glucocorticoid therapy, with six also receiving immunosuppressants. All patients were followed up for a median of 30 months, with outcomes including two complete remissions, five partial remissions, one unchanged condition, and one death. Six patients experienced a relapse. Conclusions:Isolated hypertrophic pachymeningitis is the most common manifestation of IgG 4-RD, often occurring in the absence of elevated serum IgG 4 levels. Peripheral nerve and brain parenchyma involvement can also be seen. Clinical manifestations are non-specific, and histopathologic biopsy is frequently required for diagnosis. Although the disease responds well to hormone treatment, recurrence is common. Early combined immunosuppressive therapy can improve prognosis.

Objective:To investigate the clinical, imaging and neuropathological characteristics of neuronal intranuclear inclusion disease (NIID) with symptoms of the central nervous system, and to improve the diagnosis and treatments of NIID.Methods:The clinical data of 7 patients with NIID diagnosed by brain biopsy in Xuanwu Hospital, Capital Medical University, Beijing, China from February 2009 to December 2024 were collected. The characteristics of clinical manifestations, imaging, and histology on brain biopsy were retrospectively analyzed.Results:Among the 7 patients, 5 were male and 2 were female. Their ages ranged from 44 to 70 years, median 56 (52, 65) years. Patients were classified into three types of tumor, stroke and encephalitis according to the onset symptoms, imaging manifestations and pathological changes. The chief complaint of the 5 patients was headache, while 4 patients had paroxysmal convulsions, 3 had speech disorders, 2 had abnormal mental behaviors, 2 had memory decline, and 1 had fever accompanied by consciousness disorders. Diffusion-weighted magnetic resonance imaging of the head showed the "ribbon sign" at the junction of the cortex and medulla in 2 cases. Most of the patients had white matter lesions, gyrus swelling and cerebral atrophy. Occasionally gyrus-like enhancement was observed. Brain biopsy reveals the histological changes that matched those on images and initial symptoms. There were proliferation of oligodendrocytes and astrocytes in the white matter, leukoaraiosis and edema, cortical disintegration and lamellar necrosis, as well as infiltration of lymphocytes and microglia, etc. However, the characteristic changes were eosinophilic hyaline inclusions in the nuclei of neurons and astrocytes. Immunohistochemical staining of p62 and ubiquitin showed homogeneous staining in round or ring-shaped nuclei.Conclusions:The clinical manifestations of NIID are highly variable, and a correct diagnosis of NIID requires careful integration of clinical, imaging and histopathologic data. For patients with a high suspicion of NIID, immunohistochemical staining of p62 and ubiquitin is diagnostically valuable.

Post-transplant lymphoproliferative disorder (PTLD) is a solid organ or hematopoietic stem cells transplant associated syndrome, and central nervous system PTLD(CNS-PTLD) is extremely rare. A case of CNS-PTLD occurring after 24 years of kidney transplant was reported, and pathological examination proved it to be diffuse large B cell lymphoma. Cerebrospinal fluid next generation sequencing and pathological examination supported that Epstein-Barr virus infection was associated with it.

Objective:To investigate the clinicopathological features, diagnosis and prognosis of diffuse leptomeningeal glioneuronal tumor (DLGNT).Methods:Five cases of DLGNT diagnosed from January 2016 to January 2020 were collected from Xuanwu Hospital, Capital Medical University. The clinical features, histopathologic characteristics, immunohistochemical and molecular genetic findings and prognosis were analyzed and the relevant literature was reviewed.Results:The five patients (two males and three females) were aged 2 to 52 years (median 11 years), and had history of increased intracranial pressure (headache and vomiting) or limb weakness. Three of them were younger than 16 years of age. The imaging studies showed diffuse intracranial and intraspinal nodular leptomeningeal thickening and enhancement, with or without parenchymal involvement. At times there were associated small cyst-like lesions. Imaging interpretations were inflammatory lesions in three cases and space occupying lesions in two. Microscopically, in three cases the tumors showed low to moderate cellularity, consisting of relatively monomorphous oligodendrocyte-like cells arranged in small nests or diffusely distribution. No mitosis and necrosis were observed. In two cases there were increased cellularity with a diffuse honeycomb pattern. The tumor showed mild to moderate polymorphism with hyperchromatic nuclei. Mitosis, endothelial vascular proliferation and glomeruloid vessels were seen. Necrosis was absent. The tumor cells in all five cases were positive for synaptophysin,Olig2 and negative for IDH1 and H3 K27M. GFAP was focally positive in four cases and only one case expressed NeuN partly. The Ki-67 labeling index was 1%-35%. BRAF fusion was detected in four cases. Genetic analysis showed solitary 1p deletion in two cases (2/5), while all cases were negative for 1p/19q co-deletion (0/5). The five patients were followed up for 13 to 28 months (median 15 month). One patient died after 27 months. There was no evidence of tumor progression in the remaining four patients.Conclusions:DLGNT is rare and easily confused with other central nervous system tumors and inflammatory lesions. Therefore, the diagnosis of DLGNT should be made based on comprehensive information including imaging, morphologic and corresponding immunohistochemical examinations and molecular genetics to avoid misdiagnosis and delay in management.