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 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 diagnostic performance of MRI Liver Imaging Reporting and Data System version 2018 in high-risk hepatocellular carcinoma (HCC) patients with intrahepatic parenchymal substantial lesions ≤3.0 cm.Methods:A retrospective analysis was conducted in hospitals between September 2014 to April 2020. 131 pathologically confirmed non-HCC cases with lesions ≤3.0 cm in diameter were randomly matched with 131 cases with lesions ≤3.0 cm in diameter and divided into benign (56 cases), other hepatic malignant tumor (OM, 75 cases), and HCC group (131 cases) in a 1:1 ratio. MRI features of the lesions were analyzed and classified according to LI-RADS v2018 criteria (tie-break rule was applied to lesions with both HCC and LR-M features). Taking the pathological results as the gold standard, the sensitivity and specificity of the LI-RADS v2018 classification criteria and the more stringent LR-5 criteria (with three main signs of HCC at the same time) were calculated for HCC, OM or benign lesions diagnosis. Mann -Whitney U test was used to compare the classification results. Results:The number of cases classified as LR-M, LR-1, LR-2, LR-3, LR-4, and LR-5 in HCC group after applying the tie-break rule were 14, 0, 0, 12, 28, and 77, respectively. There were 40, 0, 0, 4, 17, 14 and 8, 5, 1, 26, 13, 3 cases in benign and OM group, respectively. There were 41 (41/77), 4 (4/14) and 1 (1/3) lesion case in the HCC, OM and benign group, respectively, that met the more stringent LR-5 criteria. The sensitivity of LR-4 combined with LR-5 (LR-4/5) criteria, LR-5 criteria and more stringent LR-5 criteria for HCC diagnosis were 80.2% (105/131), 58.8% (77/131) and 31.3% (41/131), respectively, and the specificity were 64.1% (84/131), 87.0% (114/131) and 96.2% (126/131), respectively. The sensitivity and specificity of LR-M were 53.3% (40/75) and 88.2% (165/187), respectively. The sensitivity and specificity using LR-1 combined with LR-2 (LR-1/2) criteria for the diagnosis of benign liver lesions were 10.7% (6/56) and 100% (206/206), respectively.Conclusions:LR-1/2, LR-5, and LR-M criteria have high diagnostic specificity for intrahepatic lesions with a diameter of ≤3.0 cm. Lesions classified as LR-3 are more likely to be benign. The specificity of LR-4/5 criteria is low, while the more stringent LR-5 criteria has a high specificity for HCC diagnosis.

Objective: To observe interhemispheric and interregional resting-state functional connectivity using voxel-mirrored homotopic connectivity (VMHC) in patients with amnestic mild cognitive impairment (aMCI). Methods Totally 47 aMCI patients (aMCI group) and 43 gender, age and education matched normal controls (control group) were enrolled. The cranial structural MRI and resting-state fMRI data were collected. VMHC between both groups were compared to obtain the brain areas with altered VMHC. Using the altered VMHC areas as ROIs, the functional connectivity to the whole brain areas was calculated to observe the brain areas with different functional connection. Correlation analysis was performed between altered functional connection and neurocognitive scores. Results aMCI patients showed decreased inter-hemispheric VMHC primarily at bilateral inferior frontal gyrus, anterior insula and putamen. No area showed increased VMHC was found in aMCI patients. In aMCI patients, the functional connection of anterior insula to default mode network (DMN) regions including the posterior cingulate cortex (PCC) and the medial prefrontal cortex (MPFC) increased, and to attention control network and cerebellum decreased, and the functional connection of the left anterior insula to PCC and MPFC was positively correlated with recognition scores of auditory verbal learning test (r=0.38, 0.33, both P<0.05). Conclusion aMCI patients show deficits in inter-hemispheric VMHC and altered functional connection between different brain networks, suggesting an important new avenue for better understanding of the nature of cognitive changes in patients with aMCI.

OBJECTIVE: To explore the contents of secoiridoid substances (gentiopicroside, swertiamarin, sweroside) in the leaves of Gentiana rigescens from different populations in Dali Bai Autonomous Prefecture (Shorted for Dali prefecture), and to provide reference for the selection of fine varieties, the development and utilization of G.rigescens. METHODS: The contents of secoiridoid substances in the leaves of G. rigescens from different populations in Dali prefecture were determined by HPLC, and the HPLC fingerprint was established. The contents of secoiridoid substances in the leaves of G. rigescens from different populations was studied by using single factor variance analysis, clustering analysis and principal component analysis. RESULTS: There were statistical significance in the contents of gentiamarin, swertiamarin and sweroside and the total contents in the leaves of G. rigescens from different populations in Dali prefecture (P<0. 05 or P<0. 01). Twelve common peaks were found in HPLC fingerprint, and HPLC fingerprint similarities of common peaks except Gantong population were all over 0. 972. Three peaks of them were identified as the characteristic peaks of gentiamarin, swertiamarin and sweroside which were correlated to the activities. The total contents of gentiamarin, swertiamarin and sweroside from different populations obtained by clustering analysis were consistent with that by HPLC fingerprint principal component analysis. CONCLUSIONS: There are great differences in the content of gentiamarin, swertiamarin and sweroside in the leaves of G. rigescens from different populations in Dali prefecture. The similarity of secoiridoid substances in the leaves of G. rigescens from different populations except Gantong population is higher.

Alzheimer disease (AD) is the most common cause of dementia in the aging people.The early diagnosis is very important to slow down the progression and improve the prognosis of AD.At present,most researches focus on the subjective cognitive decline (SCD),which is considered as the preclinical stage of AD.The use of biological markers to predict whether the progress of SCD to AD is necessary.The progresses of imaging studies on SCD were reviewed in this article.