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OBJECTIVE: The aim of this study was to noninvasively explore the glymphatic system (GS) in glioma and its association with glioma characteristics and prognosis by using diffusion tensor image analysis along the perivascular space (ALPS). METHODS: In the period from April 2015 to November 2021, all patients with pathologically confirmed unihemispheric glioma who had not undergone surgery, chemotherapy, radiotherapy, or stereotactic biopsy; who did not have severe brain deformation; who had undergone preoperative conventional and advanced whole-brain diffusion-weighted imaging; and whose data were available and uncompromised were included in this study. Age- and sex-matched healthy controls (HCs) who had undergone diffusion-weighted imaging were also included. The ALPS index was calculated based on diffusivity maps, allowing noninvasive analysis of the GS. The contralateral ALPS index was measured in all glioma patients, and the ipsilateral ALPS index was measured in glioma patients without severe deformation of the ipsilateral hemisphere. The ALPS index was compared between glioma patients and HCs according to tumor grade, IDH genotype, tumor and edema volume, and tumor location. The association between the bilateral ALPS index of gliomas and tumor characteristics was further analyzed. Survival analysis was conducted using Kaplan-Meier survival curves with the log-rank test and univariable and multivariable Cox regressions. RESULTS: Ninety-one patients with unihemispheric glioma (33 female, mean age 46 ± 13 years) and 59 age- and sex-matched HCs were included in this study. The ipsilateral ALPS index decreased in the glioma group versus the HC group, regardless of tumor grade, IDH genotype, tumor and edema volume, or tumor location (p ≤ 0.048), whereas the contralateral ALPS index decreased in gliomas with a high grade, IDH wildtype, larger edema volume, different tumor volumes and locations (p ≤ 0.009). The ipsilateral versus contralateral ALPS index was lower regardless of tumor grade, IDH genotype, tumor and edema volume, or tumor location (p ≤ 0.044). Univariable linear regression revealed age (ß = -0.004, p = 0.026), tumor grade (ß = -0.114, p = 0.011), and IDH genotype (ß = 0.120, p = 0.008) were associated with the ipsilateral ALPS index in glioma. Age (ß = -0.005, p < 0.001), tumor grade (ß = -0.144, p < 0.001), IDH genotype (ß = 0.154, p < 0.001), tumor volume (ß = -0.002, p = 0.001), and peritumoral edema volume (ß = -0.002, p < 0.001) were correlated with the contralateral ALPS index in glioma. Multivariable linear regression revealed that tumor grade (ß = -0.125, p = 0.005) was independently associated with the ipsilateral ALPS index. Age (ß = -0.003, p = 0.022), IDH status (ß = 0.132, p = 0.001), and tumor volume (ß = -0.002, p < 0.001) were independently associated with the contralateral ALPS index. Kaplan-Meier analysis showed different survival times between low and high contralateral ALPS groups (log-rank = 10.574, p = 0.001). Univariable Cox regression analysis demonstrated that the lower contralateral ALPS index was related to a shorter survival time (HR 0.095, p = 0.005). Multivariable Cox regression analysis revealed IDH status as the only independent factor for survival (HR 0.138, p < 0.001). CONCLUSIONS: GS function was impaired in glioma and correlated with tumor characteristics, and worse contralateral GS function was associated with a shorter survival time.
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BACKGROUND: The aim of the study was to investigate the diagnostic value of imaging necrosis (Imnecrosis) in grading, predict the genotype and prognosis of gliomas, and further assess tumor necrosis by dynamic contrast-enhanced MR perfusion imaging (DCE-MRI). PATIENTS AND METHODS: We retrospectively included 150 patients (104 males, mean age: 46 years old) pathologically proved as adult diffuse gliomas and all diagnosis was based on the 2021 WHO central nervous system (CNS) classification. The pathological necrosis (Panecrosis) and gene mutation information were collected. All patients underwent conventional and DCE-MRI examinations and had been followed until May 31, 2021. The Imnecrosis was determined by two experienced neuroradiologists. DCE-MRI derived metric maps have been post-processed, and the mean value of each metric in the tumor parenchyma, peritumoral and contralateral area were recorded. RESULTS: There was a strong degree of inter-observer agreement in defining Imnecrosis (Kappa = 0.668, p < 0.001) and a strong degree of agreement between Imnecrosis and Panecrosis (Kappa = 0.767, p < 0.001). Compared to low-grade gliomas, high-grade gliomas had more Imnecrosis (85.37%, p < 0.001), and Imnecrosis significantly increased with the grade of gliomas increasing. And Imnecrosis was significantly more identified in IDH-wildtype, 1p19q-non-codeletion, and CDKN2A/B-homozygous-deletion gliomas. Using multivariate Cox regression analysis, Imnecrosis was an independent and unfavorable prognosis factor (Hazard Ratio = 2.113, p = 0.046) in gliomas. Additionally, extravascular extracellular volume fraction (ve) in tumor parenchyma derived from DCE-MRI demonstrated the highest diagnostic efficiency in identifying Panecrosis and Imnecrosis with high specificity (83.3% and 91.9%, respectively). CONCLUSIONS: Imnecrosis can provide supplementary evidence beyond Panecrosis in grading, predicting the genotype and prognosis of gliomas, and ve in tumor parenchyma can help to predict tumor necrosis with high specificity.
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Neoplasias Encefálicas , Glioma , Adulto , Masculino , Humanos , Persona de Mediana Edad , Neoplasias Encefálicas/patología , Pronóstico , Estudios Retrospectivos , Clasificación del Tumor , Glioma/diagnóstico por imagen , Glioma/genética , Glioma/patología , NecrosisRESUMEN
OBJECTIVES: Whether the alternation of the glymphatic system exists in neurodevelopmental disease still remains unclear. In this study, we investigated structural and functional changes in the glymphatic system in the treatment-naïve attention-deficit/hyperactivity disorder (ADHD) children by quantitatively measuring the Virchow-Robin spaces (VRS) volume and diffusion tensor image-analysis along the perivascular space (DTI-ALPS). METHODS: Forty-seven pediatric ADHD patients and 52 age- and gender-matched typically developing (TD) children were recruited in this prospective study. The VRS volume was calculated using a semi-automated approach in axial T2-weighted images. Diffusivities along the x-, y-, and z-axes in the projection, association, and subcortical neural fiber areas were measured. The ALPS index, a ratio that accentuated water diffusion along the perivascular space, was calculated. The Mann-Whitney U test was used to compare the quantitative parameters; Pearson's correlation was used to analyze the correlation with clinical symptoms. RESULTS: The cerebral VRS volume (mean, 15.514 mL vs. 11.702 mL) and the VRS volume ratio in the ADHD group were larger than those in the TD group (all p < 0.001). The diffusivity along the x-axis in association fiber area and ALPS index were significantly smaller in the ADHD group vs. TD group (mean, 1.40 vs.1.59, p < 0.05 after false discovery rate adjustment). Besides, the ALPS index was related to inattention symptoms of ADHD (r = - 0.323, p < 0.05). CONCLUSIONS: Our study suggests that the glymphatic system alternation may participate in the pathogenesis of ADHD, which may be a new research direction for exploring the mechanisms of psycho-behavioral developmental disorders. Moreover, the VRS volume and ALPS index could be used as the metrics for diagnosing ADHD. CLINICAL RELEVANCE STATEMENT: Considering the potential relevance of the glymphatic system for exploring the mechanisms of attention deficit/hyperactivity, the Virchow-Robin spaces volume and the analysis along the perivascular space index could be used as additional metrics for diagnosing the disorder. KEY POINTS: ⢠Increased Virchow-Robin space volume and decreased analysis along the perivascular space index were found in the treatment-naïve attention-deficit/hyperactivity disorder children. ⢠The results of this study indicate that the glymphatic system alternation may have a valuable role in the pathogenesis of attention-deficit/hyperactivity disorder. ⢠The analysis along the perivascular space index is correlated with inattention symptoms of attention-deficit/hyperactivity disorder children.
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Trastorno por Déficit de Atención con Hiperactividad , Humanos , Niño , Trastorno por Déficit de Atención con Hiperactividad/diagnóstico por imagen , Estudios Prospectivos , Benchmarking , Difusión , Procesamiento de Imagen Asistido por ComputadorRESUMEN
BACKGROUND: Glioma classification affects treatment and prognosis. Reliable imaging methods for preoperatively evaluating gliomas are essential. PURPOSE: To evaluate tumor multiregional mean apparent propagator (MAP) features in glioma diagnosis and to compare those with diffusion-kurtosis imaging (DKI). STUDY TYPE: Retrospective study. SUBJECTS: 70 untreated glioma patients (31 LGGs (low-grade gliomas), 34 women; mean age, 47 ± 12 years, training (60%, n = 42) and testing cohorts (40%, n = 28)). FIELD STRENGTH/SEQUENCE: 3-T, diffusion-MRI using q-space Cartesian grid sampling with 11 different b-values. ASSESSMENT: Tumor multiregional MAP (mean squared displacement (MSD); q-space inverse variance (QIV); non-Gaussianity (NG); axial/radial non-Gaussianity (NGAx, NGRad); return-to-origin/axis/plane probability (RTOP, RTAP, and RTPP)); and DKI metrics (axial/mean/radial kurtosis (AK, MK, and RK)) on tumor parenchyma (TP) and peritumoral areas (PT) in histopathologically gliomas grading and genotyping were assessed. STATISTICAL TESTS: Mann-Whitney U; Kruskal-Wallis; Benjamini-Hochberg; Bonferroni-correction; receiver operating curve (ROC) and area under curve (AUC); DeLong's test; Random Forest (RF). P value<0.05 was considered statistically significant after multiple comparisons correction. RESULTS: Compared with LGGs, MSD, and QIV were significantly lower in TP, whereas NG, NGAx, NGRad, RTOP, RTAP, RTPP, and DKI metrics were significantly higher in HGGs (high-grade gliomas) (P ≤ 0.007), as well as in isocitrate-dehydrogenase (IDH)-mutated than IDH-wildtype gliomas (P ≤ 0.039). These trends were reversed for PT (tumor grades, P ≤ 0.011; IDH-mutation status, P ≤ 0.012). ROC analysis showed that, in TP, DKI metrics performed best in TP (AUC 0.83), whereas in PT, RTPP performed best (AUC 0.77) in glioma grading. AK performed best in TP (AUC 0.77), whereas MSD and RTPP performed best in PT (AUC 0.73) in IDH genotyping. Further RF analysis with DKI and MAP demonstrated good performance in grading (AUC 0.91, Accuracy 82%) and IDH genotyping (AUC 0.87, Accuracy 79%). DATA CONCLUSION: Tumor multiregional MAP features could effectively evaluate gliomas. The performance of MAP may be similar to DKI in TP, while in PT, MAP may outperform DKI. LEVEL OF EVIDENCE: 4 TECHNICAL EFFICACY: Stage 2.
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BACKGROUND: Accumulating evidences indicate regional gray matter (GM) morphology atrophy in spinocerebellar ataxia type 3 (SCA3); however, whether large-scale morphological brain networks (MBNs) undergo widespread reorganization in these patients remains unclear. OBJECTIVE: To investigate the topological organization of large-scale individual-based MBNs in SCA3 patients. METHODS: The individual-based MBNs were constructed based on the inter-regional morphological similarity of GM regions. Graph theoretical analysis was taken to assess GM structural connectivity in 76 symptomatic SCA3, 24 pre-symptomatic SCA3, and 54 healthy normal controls (NCs). Topological parameters of the resulting graphs and network-based statistics analysis were compared among symptomatic SCA3, pre-symptomatic SCA3, and NCs groups. The inner association between network properties and clinical variables was further analyzed. RESULTS: Compared to NCs and pre-symptomatic SCA3 patients, symptomatic SCA3 indicated significantly decreased integration and segregation, a shift to "weaker small-worldness", characterized by decreased Cp , lower Eloc, and Eglob (all p < 0.005). Regarding nodal properties, symptomatic SCA3 exhibited significantly decreased nodal profiles in the central executive network (CEN)-related left inferior frontal gyrus, limbic regions involving the bilateral amygdala, left hippocampus, and bilateral pallidum, thalamus; and increased nodal degree, efficiency in bilateral caudate (all pFDR <0.05). Meanwhile, clinical variables were correlated with altered nodal profiles (pFDR ≤0.029). SCA3-related subnetwork was closely interrelated with dorsolateral cortico-striatal circuitry extending to orbitofrontal-striatal circuits and dorsal visual systems (lingual gyrus-striatal). CONCLUSION: Symptomatic SCA3 patients undergo an extensive and significant reorganization in large-scale individual-based MBNs, probably due to disrupted prefrontal cortico-striato-thalamo-cortical loops, limbic-striatum circuitry, and enhanced connectivity in the neostriatum. This study highlights the crucial role of abnormal morphological connectivity alterations beyond the pattern of brain atrophy, which might pave the way for therapeutic development in the future.
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Enfermedad de Machado-Joseph , Humanos , Enfermedad de Machado-Joseph/diagnóstico por imagen , Enfermedad de Machado-Joseph/genética , Enfermedad de Machado-Joseph/patología , Imagen por Resonancia Magnética/métodos , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Sustancia Gris/diagnóstico por imagen , Sustancia Gris/patología , Atrofia/patologíaRESUMEN
Regional atrophy and metal deposition are typical manifestations in Wilson's disease, but their relationship has not been systematically investigated. We aim to investigate the association of regional brain atrophy and metal deposition in the deep gray matter nucleus at MRI in Wilson's disease. We acquired the structural and susceptibility mapping and performed a cross-sectional comparison of volume and susceptibility in deep gray matter nucleus. The most extensive and severe atrophy was detected in brain regions in neuro-Wilson's disease, as well as the most widespread and heaviest metal deposits. Metal deposits were significantly negatively correlated with volume in the bilateral thalamus, caudate, and putamen. None of correlation was found between the clinical score with volume or susceptibility in the focused regions. In the 1-year follow-up analysis, the volume of right thalamus, globus pallidus, and brainstem and the susceptibility of the left caudate have decreased significantly as the symptom improvement. In Wilson's disease, phenotypes have varied scope and extend of volumetric atrophy and metal deposits. This study is expected to take the lead in revealing that in neuro-Wilson's disease, greater regional atrophy associated with heavier metal deposits in Wilson's disease. Moreover, after 1-year treatment, the imaging data have changed as the patient's condition improvement.
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Degeneración Hepatolenticular , Humanos , Degeneración Hepatolenticular/diagnóstico por imagen , Degeneración Hepatolenticular/patología , Sustancia Gris/diagnóstico por imagen , Sustancia Gris/patología , Estudios Transversales , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Atrofia/patología , Imagen por Resonancia Magnética/métodosRESUMEN
BACKGROUND: Clinical decision-making in spinocerebellar ataxia spectrum diseases (SCAs) has mainly been based on genetic tests, not considering the SCAs' imaging and clinical heterogenicity. OBJECTIVE: To identify SCAs phenogroups by analysis and hierarchical clustering of infratentorial morphological MRI for unveiling pathophysiological differences among common SCA subtypes. METHODS: We prospectively enrolled 119 (62 women; mean age 37 years) genetically diagnosed SCAs (SCA1 n = 21, SCA2 n = 10, symptomatic SCA3 n = 59, presymptomatic SCA3 n = 22, SCA6 n = 7) and 35 healthy controls (HCs). All patients underwent MRI and detailed neurological and neuropsychology examinations. The width of each cerebellar peduncle (CP) and anteroposterior diameter of the spinal cord and pontine were measured. Twenty-five SCAs patients (15 women; mean age 35 years) were followed for at least a year (17 (15, 24) months), whose MRI and the Scale for the Assessment and Rating of Ataxia (SARA) were collected. RESULTS: Infratentorial morphological MRI measurements could significantly discriminate SCAs from HCs, even among SCA subtypes. Two mutually exclusive and clinically distinct phenogroups were identified. Despite similar (CAG)n, phenogroup 1 (n = 66, 55.5%) presented more atrophied infratentorial brain structures and more severe clinical symptoms with older age and earlier age of onset when compared with phenogroup 2. More importantly, all SCA2, most of SCA1 (76%), and symptomatic SCA3 (68%) were classified into phenogroup 1, whereas all SCA6 and all presymptomatic SCA3 were in phenogroup 2. The right middle CP had the highest diagnostic value in predicting phenogroup 2 (AUC = 0.99; P < 0.01) with high specificity (95%). Consistent with the significantly increased SARA (7.5 vs 10, P = 0.021), the bilateral inferior CP, spinal cord, and pontine tegmentum were more atrophy during the follow-up (P < 0.05). CONCLUSION: SCAs were with significant infratentorial brain atrophy than HCs. We identified two different SCAs phenogroups associated with substantial differences in infratentorial brain atrophy, clinical presentation, and may reflect the underlying molecular profiles to some extent, paving the way for a more personalized diagnostic and treatment approach.
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Ataxias Espinocerebelosas , Degeneraciones Espinocerebelosas , Humanos , Femenino , Adulto , Ataxias Espinocerebelosas/diagnóstico por imagen , Ataxias Espinocerebelosas/genética , Imagen por Resonancia Magnética , Cerebelo , Atrofia , Análisis por ConglomeradosRESUMEN
OBJECTIVES: To comprehensively evaluate the glioma using quantitative susceptibility mapping (QSM). MATERIALS AND METHODS: Forty-two patients (18 women; mean age, 45 years) with pathologically confirmed gliomas were retrospectively included. All the patients underwent conventional and advanced MRI examinations (QSM, DWI, MRS, etc.). Five patients underwent paired QSM (pre- and post-enhancement). Four Visually Accessible Rembrandt Image (VASARI) features and intratumoural susceptibility signal (ITSS) were observed. Three ROIs each were manually drawn separately in the tumour parenchyma with relatively high and low magnetic susceptibility. The association between the tumour's magnetic susceptibility and other MRI parameters was also analysed. RESULTS: Morphologically, gliomas with heterogeneous ITSS were more similar to high-grade gliomas (p = 0.006, AUC: 0.72, sensitivity: 70%, and specificity: 73%). Heterogeneous ITSS was significantly associated with tumour haemorrhage, necrosis, diffusion restriction, and avid enhancement but did not change between pre- and post-enhanced QSM. Quantitatively, tumour parenchyma magnetic susceptibility had limited value in grading gliomas and identifying IDH mutation status, whereas the relatively low magnetic susceptibility of the tumour parenchyma helped identify oligodendrogliomas in IDH mutated gliomas (AUC = 0.78) with high specificity (100%). The relatively high tumour magnetic susceptibility significantly increased after enhancement (p = 0.039). Additionally, we found that the magnetic susceptibility of the tumour parenchyma was significantly correlated with ADC (r = 0.61) and Cho/NAA (r = 0.40). CONCLUSIONS: QSM is a promising candidate for the comprehensive evaluation of gliomas, except for IDH mutation status. The magnetic susceptibility of tumour parenchyma may be affected by tumour cell proliferation. KEY POINTS: ⢠Morphologically, gliomas with a heterogeneous intratumoural susceptibility signal (ITSS) are more similar to high-grade gliomas (p = 0.006; AUC, 0.72; sensitivity, 70%; and specificity, 73%). Heterogeneous ITSS was significantly associated with tumour haemorrhage, necrosis, diffusion restriction, and avid enhancement but did not change between pre- and post-enhanced QSM. ⢠Tumour parenchyma's relatively low magnetic susceptibility helped identify oligodendroglioma with high specificity. ⢠Tumour parenchyma magnetic susceptibility was significantly correlated with ADC (r = 0.61) and Cho/NAA (r = 0.40).
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Neoplasias Encefálicas , Glioma , Oligodendroglioma , Humanos , Femenino , Persona de Mediana Edad , Neoplasias Encefálicas/patología , Estudios Retrospectivos , Sensibilidad y Especificidad , Glioma/patología , Imagen por Resonancia Magnética/métodos , Oligodendroglioma/diagnóstico por imagen , Hemorragia , Clasificación del Tumor , Imagen de Difusión por Resonancia Magnética/métodosRESUMEN
OBJECTIVES: To investigate and characterize the structural alterations of the brain in SCA3, and their correlations with the scale for the assessment and rating of ataxia (SARA) and normal brain ATXN3 expression. METHODS: We performed multimodal analyses in 52 SCA3 (15 pre-symptomatic) and healthy controls (HCs) (n = 35) to assess the abnormalities of gray and white matter (WM) of the cerebrum, brainstem, and cerebellum via FreeSurfer, SUIT, and TBSS, and their associations with disease severity. Twenty SCA3 patients (5 pre- and 15 symptomatic) were followed for at least a year. Besides, we uncovered the normal pattern of brain ATXN3 spatial distribution. RESULTS: Pre-symptomatic patients showed only WM damage, mainly in the cerebellar peduncles, compared to HCs. In the advanced stage, the WM damage followed a caudal-rostral pattern. Meanwhile, continuous nonlinear structure damage was characterized by brainstem volumetric reduction and relatively symmetric cerebellar and basal ganglia atrophy but spared the cerebral cortex, partially explained by the ATXN3 overexpression. The bilateral pallidum, brainstem, and cerebellar peduncles demonstrated a very large effect size. Besides, all these alterations were significantly correlated with SARA; the pons (r = -0.65) and superior cerebellar peduncle (r = -0.68) volume demonstrated a higher correlation than the cerebellum with SARA. The longitudinal study further uncovered progressive atrophy of pons in symptomatic SCA3. CONCLUSIONS: Significant WM damage starts before the ataxia onset. The bilateral pallidum, brainstem, and cerebellar peduncles are the most vulnerable targets. The volume of pons appears to be the most promising imaging biomarker for a longitudinal study. TRIAL REGISTRATION: ClinicalTrial ID: ChiCTR2100045857 ( http://www.chictr.org.cn/edit.aspx?pid=55652&htm=4 ) KEY POINTS: ⢠Pre- SCA3 showed WM damage mainly in cerebellar peduncles. Continuous brain damage was characterized by brainstem, widespread, and relatively symmetric cerebellar and basal ganglia atrophy. ⢠Volumetric abnormalities were most evident in the bilateral pallidum, brainstem, and cerebellar peduncles in SCA3. ⢠The volume of pons might identify the disease progression longitudinally.
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Enfermedad de Machado-Joseph , Imagen por Resonancia Magnética , Humanos , Atrofia/diagnóstico por imagen , Atrofia/patología , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Cerebelo/diagnóstico por imagen , Cerebelo/patología , Estudios Longitudinales , Enfermedad de Machado-Joseph/diagnóstico por imagen , Enfermedad de Machado-Joseph/genética , Enfermedad de Machado-Joseph/patología , Imagen por Resonancia Magnética/métodosRESUMEN
BACKGROUND: Pediatric meningioma with YAP1 fusion is a rare subset of meningiomas. Currently, there are lack of integrated clinical, radiological, and pathological features on this subset. Here, we reported a case of pediatric meningioma with a novel MAML2-YAP1 fusion variant and reviewed the relevant literature. CASE PRESENTATION: We presented a case of 12-year-old boy with meningioma adjacent to the superior sagittal sinus and falx. Simpson grade II gross total resection was performed after diagnosis. Pathologically, he was diagnosed as WHO grade I meningothelial meningioma with rhabdoid features. A next-generation sequencing-based gene panel was performed to determine the molecular features for potential treatment, and a novel MAML2-YAP1 fusion break point was identified. CONCLUSION: Pediatric meningioma with the fusion of YAP1 and MAML2 genes is more likely to have pathological features of rhabdiod cells, which needs to be validated in large-scale studies for exploring better treatment under the integrated diagnosis.
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Neoplasias Meníngeas , Meningioma , Masculino , Niño , Humanos , Meningioma/diagnóstico por imagen , Meningioma/genética , Factores de Transcripción/genética , Secuenciación de Nucleótidos de Alto Rendimiento , Neoplasias Meníngeas/diagnóstico por imagen , Neoplasias Meníngeas/genética , Transactivadores/genéticaRESUMEN
Background: After deep brain stimulation (DBS), hiccups as a complication may lead to extreme fatigue, sleep deprivation, or affected prognosis. Currently, the causes and risk factors of postoperative hiccups are unclear. In this study, we investigated the risk factors for hiccups after DBS of the subthalamic nucleus (STN) for Parkinson's disease (PD) under general anesthesia. Methods: We retrospectively included patients who underwent STN DBS in the study, and collected data of demographic characteristics, clinical evaluations, and medications. According to the occurrence of hiccups within seven days after operation, the patients were divided into a hiccups group and non-hiccups group. The potentially involved risk factors for postoperative hiccups were statistically analyzed by logistic regression analysis. Results: A total of 191 patients were included in the study, of which 34 (17.80%) had postoperative transient persistent hiccups. Binary univariate logistic regression analysis showed that male, higher body mass index (BMI), smoker, Hoehn and Yahr stage (off), preoperative use of amantadine, hypnotic, Hamilton anxiety scale and Hamilton depression scale scores, and postoperative limited noninfectious peri-electrode edema in deep white matter were suspected risk factors for postoperative hiccups (p < 0.1). In binary multivariate logistic regression analysis, male (compared to female, OR 14.00; 95% CI, 1.74−112.43), postoperative limited noninfectious peri-electrode edema in deep white matter (OR, 7.63; 95% CI, 1.37−42.37), preoperative use of amantadine (OR, 3.64; 95% CI, 1.08−12.28), and higher BMI (OR, 3.50; 95% CI, 1.46−8.36) were independent risk factors for postoperative hiccups. Conclusions: This study is the first report about the risk factors of hiccups after STN DBS under general anesthesia for PD patients. The study suggests that male, higher BMI, preoperative use of amantadine, and postoperative limited noninfectious peri-electrode edema in deep white matter are independent risk factors for postoperative hiccups of STN-DBS for PD patients. Most hiccups after STN-DBS for PD patients were transient and self-limiting.
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Objective: This study aimed to discuss adjusting the treatment plan for patients with type 2 diabetes mellitus (T2DM) who are positive for exogenous insulin antibody (IA). The outcome of patients who are IA-positive with an adjusted treatment plan was considered. Methods: The treatment plan for patients with IA-positive T2DM was adjusted to oral medication or long-acting insulin + oral medication. Insulin antibody, C-peptide, and insulin were re-examined before treatment and at 1, 3, 6, 12, 18, and 24 months after treatment. The time of IA-negative seroconversion and its indexes, including blood glucose, C-peptide, and insulin, were recorded and analyzed. Results: After adjusting the treatment plan for 2 years, in 73 patients, 57 had IA-negative seroconversion, and 16 had positive IA. The blood glucose, C-peptide, insulin, glycosylated hemoglobin (HbA1c), and the daily dose of insulin in the seroconversion group and the non-seroconversion group decreased compared with before the adjustment of the treatment plan (P < 0.05). The negative seroconversion rate within 2 years was related to the insulin concentration before treatment. Conclusion: Patients with IA-positive T2DM need to adjust their treatment plans in time. Even if IA does not turn negative within 2 years after adjusting the treatment plan, the levels of blood glucose, C-peptide, insulin, and HbA1c along with the insulin dosage would be significantly improved, which can benefit patients. The higher the fasting insulin and 2-hour insulin values before adjusting the treatment plan, the longer the time required for IA to turn negative.
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Objective: To investigate the epidemiology and the effectiveness of resuscitation from cardiopulmonary arrest (CPA) among critically ill children and adolescents during pediatric intensive care unit (PICU) stay across China. Methods: A prospective multicenter study was conducted in 11 PICUs in tertiary hospitals. Consecutively hospitalized critically ill children, from 29-day old to 18-year old, who had suffered from CPA and required cardiopulmonary resuscitation (CPR) in the PICU were enrolled (December 2017-October 2018). Data were collected and analyzed using the "in-hospital Utstein style." Neurological outcome was assessed with the Pediatric Cerebral Performance Category (PCPC) scale among children who had survived. Factors associated with the return of spontaneous circulation (ROSC) and survival at discharge were evaluated using multivariate logistic regression. Results: Among 11,599 admissions to PICU, 372 children (3.2%) had CPA during their stay; 281 (75.5%) received CPR, and 91 (24.5%) did not (due to an order of "Do Not Resuscitate" requested by their guardians). Cardiopulmonary disease was the most common reason for CPA (28.1% respiratory and 19.6% circulatory). The most frequent initial dysrhythmia was bradycardia (79%). In total, 170 (60.3%) of the total children had an ROSC, 91 had (37.4%) survived till hospital discharge, 28 (11.5%) had survived 6 months, and 19 (7.8%) had survived for 1 year after discharge. Among the 91 children who were viable at discharge, 47.2% (43/91) received a good PCPC score (1-3). The regression analysis results revealed that the duration of CPR and the dose of epinephrine were significantly associated with ROSC, while the duration of CPR, number of CPR attempts, ventricular tachycardia/ventricular fibrillation (VT/VF), and the dose of epinephrine were significantly associated with survival at discharge. Conclusion: The prevalence of CPA in critically ill children and adolescents is relatively high in China. The duration of CPR and the dose of epinephrine are associated with ROSC. The long-term prognosis of children who had survived after CPR needs further improvement.
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Homozygous familial hypercholesterolemia (HoFH) is a rare autosomal recessive genetic disorder. It is difficult to diagnose and treat it at early stage. We present a nine-year-old boy with HoFH from China. At the beginning, he was misdiagnosed as xanthomatosis in the dermatology department of the local hospital, but the disease did not alleviate after three laser ablation operations. Later, blood lipid monitoring, ultrasound of heart and carotid artery were further added in our hospital, and finally the boy was diagnosed with HoFH by genetic testing. A biallelic mutations was observed in the fourth exon of low density lipoprotein receptor (LDLR): c.418G>A (p.E140K). Our patient achieved a relatively satisfactory therapeutic results after a series of lipid-lowering therapies including atorvastatin monotherapy, lipoprotein apheresis and double-filtration plasma pheresis. We found that LDL-C levels obtained 57% reduction from baseline after atorvastatin combined with double-filtration plasma pheresis (DFPP). It was observed that regression of carotid intima-media thickness (cIMT), valve regurgitation and xanthoma occurred after a series of Intensive lipid-lowering therapy.
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BACKGROUND: Monitoring the depth of anesthesia by electroencephalogram (EEG) based on the prefrontal cortex is an important means to achieve accurate regulation of anesthesia for subthalamic nucleus (STN) deep brain stimulation (DBS) under general anesthesia in patients with Parkinson's disease (PD). However, no previous study has conducted an in-depth investigation into this monitoring data. Here, we aimed to analyze the characteristics of prefrontal cortex EEG during DBS with propofol general anesthesia in patients with PD and determine the reference range of parameters derived from the depth of anesthesia monitoring. Additionally, we attempted to explore whether the use of benzodiazepines in the 3 days during hospitalization before surgery impacted the interpretation of the EEG parameters. MATERIALS AND METHODS: We included the data of 43 patients with PD who received STN DBS treatment and SedLine monitoring during the entire course of general anesthesia with propofol in a single center. Eighteen patients (41.86%) took benzodiazepines during hospitalization. We divided the anesthesia process into three stages: awake state before anesthesia, propofol anesthesia state, and shallow anesthesia state during microelectrode recording (MER). We analyzed the power spectral density (PSD) and derived parameters of the patients' prefrontal EEG, including the patient state index (PSI), spectral edge frequency (SEF) of the left and right sides, and the suppression ratio. The baseline characteristics, preoperative medication, preoperative frontal lobe image characteristics, preoperative motor and non-motor evaluation, intraoperative vital signs, internal environment and anesthetic information, and postoperative complications are listed. We also compared the groups according to whether they took benzodiazepines before surgery during hospitalization. RESULTS: The average PSI of the awake state, propofol anesthesia state, and MER state were 89.86 ± 6.89, 48.68 ± 12.65, and 62.46 ± 13.08, respectively. The preoperative administration of benzodiazepines did not significantly affect the PSI or SEF, but did reduce the total time of suppression, maximum suppression ratio, and the PSD of beta and gamma during MER. Regarding the occurrence of postoperative delirium and mini-mental state examination (MMSE) scores, there was no significant difference between the two groups (chi-square test, p = 0.48; Mann-Whitney U test, p = 0.30). CONCLUSION: For the first time, we demonstrate the reference range of the derived parameters of the depth of anesthesia monitoring and the characteristics of the prefrontal EEG of patients with PD in the awake state, propofol anesthesia state, and shallow anesthesia during MER. Taking benzodiazepines in the 3 days during hospitalization before surgery reduces suppression and the PSD of beta and gamma during MER, but does not significantly affect the observation of anesthesiologists on the depth of anesthesia, nor affect the postoperative delirium and MMSE scores.
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Objective: To investigate the profiles of brain volumetric measurements in children with attention deficit hyperactivity disorder (ADHD), and the consistency of these brain volumetric measurements derived from the synthetic and conventional T1 weighted MRI (SyMRI and cT1w MRI). Methods: Brain SyMRI and cT1w images were prospectively collected for 38 pediatric patients with ADHD and 38 healthy children (HC) with an age range of 6-14 years. The gray matter volume (GMV), white matter volume (WMV), cerebrospinal fluid (CSF), non-WM/GM/CSF (NoN), myelin, myelin fraction (MYF), brain parenchyma volume (BPV), and intracranial volume (ICV) were automatically estimated from SyMRI data, and the four matching measurements (GMV, WMV, BPV, ICV) were extracted from cT1w images. The group differences of brain volumetric measurements were performed, respectively, using analysis of covariance. Pearson correlation analysis and interclass correlation coefficient (ICC) were applied to evaluate the association between synthetic and cT1w MRI-derived measurements. Results: As for the brain volumetric measurements extracted from SyMRI, significantly decreased GMV, WMV, BPV, and increased NON volume (p < 0.05) were found in the ADHD group compared with HC; No group differences were found in ICV, CSF, myelin volume and MYF (p > 0.05). With regard to GMV, WMV, BPV, and ICV estimated from cT1w images, the group differences between ADHD and HC were consistent with the results estimated from SyMRI. And these four measurements showed noticeable correlation between the two approaches (r = 0.692, 0.643, 0.898, 0.789, respectively, p < 0.001; ICC values are 0.809, 0.782, 0.946, 0.873, respectively). Conclusion: Our study demonstrated a global brain development disability, but normal whole-brain myelination in children with ADHD. Moreover, our results demonstrated the high consistency of brain volumetric indices between synthetic and cT1w MRI in children, which indicates the high reliability of SyMRI in the child-brain volumetric analysis.
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Glioma is a type of severe brain tumor, and its accurate segmentation is useful in surgery planning and progression evaluation. Based on different biological properties, the glioma can be divided into three partially-overlapping regions of interest, including whole tumor (WT), tumor core (TC), and enhancing tumor (ET). Recently, UNet has identified its effectiveness in automatically segmenting brain tumor from multi-modal magnetic resonance (MR) images. In this work, instead of network architecture, we focus on making use of prior knowledge (brain parcellation), training and testing strategy (joint 3D+2D), ensemble and post-processing to improve the brain tumor segmentation performance. We explore the accuracy of three UNets with different inputs, and then ensemble the corresponding three outputs, followed by post-processing to achieve the final segmentation. Similar to most existing works, the first UNet uses 3D patches of multi-modal MR images as the input. The second UNet uses brain parcellation as an additional input. And the third UNet is inputted by 2D slices of multi-modal MR images, brain parcellation, and probability maps of WT, TC, and ET obtained from the second UNet. Then, we sequentially unify the WT segmentation from the third UNet and the fused TC and ET segmentation from the first and the second UNets as the complete tumor segmentation. Finally, we adopt a post-processing strategy by labeling small ET as non-enhancing tumor to correct some false-positive ET segmentation. On one publicly-available challenge validation dataset (BraTS2018), the proposed segmentation pipeline yielded average Dice scores of 91.03/86.44/80.58% and average 95% Hausdorff distances of 3.76/6.73/2.51 mm for WT/TC/ET, exhibiting superior segmentation performance over other state-of-the-art methods. We then evaluated the proposed method on the BraTS2020 training data through five-fold cross validation, with similar performance having also been observed. The proposed method was finally evaluated on 10 in-house data, the effectiveness of which has been established qualitatively by professional radiologists.
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Wilson's disease (WD) is an inherited autosomal recessive disorder of copper metabolism, and its neurological and neuropsychiatric manifestations are associated with copper accumulation in brain. A few neuroimaging studies have shown that gray matter atrophy in WD affects both subcortical structures and cortex. This study aims to quantitatively evaluate the morphometric brain abnormalities in patients with WD in terms of whole brain volume and cortical thickness and their associations with clinical severity of WD. Thirty patients clinically diagnosed as WD with neurological manifestations and 25 healthy controls (HC) were recruited. 3D T1-weighted images were segmented into 276 whole-brain regions of interest (ROIs) and 68 cortical ROIs. WD-vs-HC group comparisons were then conducted for each ROI. The associations between those morphometric measurements and the Global Assessment Scale (GAS) score for WD were analyzed. Compared with HC, significant WD-related volumetric decreases were found in the bilateral subcortical nuclei (putamen, globus pallidus, caudate nucleus, substantia nigra, red nucleus and thalamus), diffuse white matter and several gray matter regions. WD patients showed reduced cortical thickness in the left precentral gyrus and the left insula. Further, the volumes of the right globus pallidus, bilateral putamen, right external capsule and left superior longitudinal fasciculus were negatively correlated with GAS. Our results indicated that significant WD-related morphometric abnormalities were quantified in terms of whole-brain volumes and cortical thicknesses, some of which correlated significantly to the clinical severity of WD. Those morphometrics may provide a potentially effective biomarker of WD.
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Degeneración Hepatolenticular , Atrofia/patología , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Correlación de Datos , Degeneración Hepatolenticular/diagnóstico por imagen , Degeneración Hepatolenticular/patología , Humanos , Imagen por Resonancia MagnéticaRESUMEN
OBJECTIVES: Comparing the diagnostic efficacy of diffusion kurtosis imaging (DKI) derived from different region of interest (ROI) methods in tumor parenchyma for grading and predicting IDH-1 mutation and 1p19q co-deletion status of glioma patients and correlating with their survival data. METHODS: Sixty-six patients (29 females; median age, 45 years) with pathologically proved gliomas (low-grade gliomas, 36; high-grade gliomas, 30) were prospectively included, and their clinical data were collected. All patients underwent DKI examination. DKI maps of each metric were derived. Three groups of ROIs (ten spots, ROI-10s; three biggest tumor slices, ROI-3s; and whole-tumor parenchyma, ROI-whole) were manually drawn by two independent radiologists. The interobserver consistency, time spent, diagnostic efficacy, and survival analysis of DKI metrics based on these three ROI methods were analyzed. RESULTS: The intraexaminer reliability for all parameters among these three ROI methods was good, and the time spent on ROI-10s was significantly less than that of the other two methods (p < 0.001). DKI based on ROI-10s demonstrated a slightly better diagnostic value than the other two ROI methods for grading and predicting the IDH-1 mutation status of glioma, whereas DKI metrics derived from ROI-10s performed much better than those of the ROI-3s and ROI-whole in identifying 1p19q co-deletion. In survival analysis, the model based on ROI-10s that included patient age and mean diffusivity showed the highest prediction value (C-index, 0.81). CONCLUSIONS: Among the three ROI methods, the ROI-10s method had the least time spent and the best diagnostic value for a comprehensive evaluation of glioma. It is an effective way to process DKI data and has important application value in the clinical evaluation of glioma. KEY POINTS: ⢠The intraexaminer reliability for all DKI parameters among different ROI methods was good, and the time spent on ROI-10 spots was significantly less than the other two ROI methods. ⢠DKI metrics derived from ROI-10 spots performed the best in ROI selection methods (ROI-10s, ten-spot ROIs; ROI-3s, three biggest tumor slices ROI; and ROI-whole, whole-tumor parenchyma ROI) for a comprehensive evaluation of glioma. ⢠The ROI-10 spots method is an effective way to process DKI data and has important application value in the clinical evaluation of glioma.
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Neoplasias Encefálicas , Glioma , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/genética , Imagen de Difusión por Resonancia Magnética , Imagen de Difusión Tensora , Femenino , Glioma/diagnóstico por imagen , Glioma/genética , Humanos , Persona de Mediana Edad , Clasificación del Tumor , Reproducibilidad de los ResultadosRESUMEN
It is unclear whether the brain activity during phonological processing of second languages (L2) is similar to that of the first language (L1) in trilingual individuals, especially when the L1 is logographic, and the L2s are logographic and alphabetic, respectively. To explore this issue, this study examined brain activity during visual and auditory word rhyming tasks in Cantonese-Mandarin-English trilinguals. Thirty Chinese college students whose L1 was Cantonese and L2s were Mandarin and English were recruited. Functional magnetic resonance imaging (fMRI) was conducted while subjects performed visual and auditory word rhyming tasks in three languages (Cantonese, Mandarin, and English). The results revealed that in Cantonese-Mandarin-English trilinguals, whose L1 is logographic and the orthography of their L2 is the same as L1-i.e., Mandarin and Cantonese, which share the same set of Chinese characters-the brain regions for the phonological processing of L2 are different from those of L1; when the orthography of L2 is quite different from L1, i.e., English and Cantonese who belong to different writing systems, the brain regions for the phonological processing of L2 are similar to those of L1. A significant interaction effect was observed between language and modality in bilateral lingual gyri. Regions of interest (ROI) analysis at lingual gyri revealed greater activation of this region when using English than Cantonese and Mandarin in visual tasks.