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【Objective】 The involvement of upper motor neuron (UMN) degeneration is crucial to the diagnosis of amyotrophic lateral sclerosis (ALS). This study aimed to determine objective and sensitive UMN degeneration markers for an accurate and early diagnosis. 【Methods】 A total of 108 ALS patients and 90 age- and gender-matched control subjects were recruited from ALS Clinic of The First Affiliated Hospital of Xi’an Jiaotong University. The motor homunculus cortex thickness data in MRI were collected from all the participants. The clinical characteristics and UMN clinical examination of bulbar, cervical, thoracic and lumbosacral regions were collected from the ALS patients. 【Results】 Cortical thickness was significantly thinner in the ALS group than in the control group in bilateral head-face-bulbar and upper-limb areas (P<0.05). The cortical thickness of the global UMN positive group was significantly thinner than that of control groups in bilateral head-face-bulbar and upper-limb areas (P<0.05). The cortical thickness of the UMN positive group in the corresponding region was significantly thinner than that of control groups in bilateral head-face-bulbar and upper-limb areas (P<0.05). 【Conclusion】 The thinning of the motor homunculus cortex can be used as an objective marker of UMN involvement in ALS patients in clinical practice.
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【Objective】 To investigate changes in thalamus structure and function and their associations with cognitive impairment in patients with amyotrophic lateral sclerosis (ALS). 【Methods】 3D high-resolution structural imaging and resting-state fMRI were applied in 78 ALS patients and 49 healthy volunteers. The shape of the thalamus was assessed using a vortex analysis and functional connectivity between the thalamus subregion and cortex was evaluated by a seed-based correlation analysis. Partial correlation analysis was used to evaluate the correlation between focal thalamus alterations and clinical assessments. 【Results】 Compared with the control group, vertex analysis showed significant atrophy in the prefrontal and temporal subregions of bilateral thalamus in the ALS group. Patients exhibited increased functional connectivity of motor-sensory ROI with the right motor cortex, of temporal ROI with the bilateral lateral occipital cortex, precuneus and right temporal subregion. In contrast, decreased function connectivity was found mainly between temporal ROI and paracingulate gyrus, left dorsomedial prefrontal lobe and left caudate. Partial correlation analysis showed that the functional connectivity of the precuneus, paracingulate cortex, left caudate nucleus and left prefrontal lobe was correlated with multiple cognitive performances. 【Conclusion】 Selective damage of thalamic structure and function in ALS plays an important role in cognitive and behavioral disorders.
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【Objective】 To investigate cortical thickness changes in the face-head region of the primary motor cortex (PMC) and its effect on survival in amyotrophy lateral sclerosis (ALS) patients. 【Methods】 A retrospective analysis was performed on 105 ALS patients who underwent head MRI scan at the same time. The A4hf (face-head) region of PMC was used as the region of interest (ROI). According to clinical symptoms, patients were divided into two groups: bulbar involvement and non-bulbar involvement. The differences of clinical features and cortical thickness in ROI were analyzed. According to the symptoms of bulbar palsy, physical examination of nervous system and EMG of tongue muscle, the patients with bulbar palsy were divided into lower motor neuron (LMN), upper motor neuron (UMN) and LMN+UMN groups. The differences of bulbar subgroup score and ROI of cortical thickness were analyzed. Age at onset, body mass index, delayed time of diagnosis, bulbar subgroup score, and ROI cortical thickness were included in survival analysis. 【Results】 ① The ROI cortical thickness was significantly lower in bulbar involvement group than non-bulbar involvement group (-0.198±0.87 vs. 0.235±0.95, P=0.017). ② There were no significant differences in the bulbar subgroup scores or cortical thickness of ROI between LMN, UMN and LMN+UMN groups (P>0.05). ③ Survival analysis showed age of onset (HR=3.296, 95% CI:1.63-6.664, P=0.001), delayed time of diagnosis (HR=0.361, 95% CI:0.184-0.705, P=0.003), bulbar subgroup score (HR 0.389, 95% CI:0.174-0.868, P=0.021), and ZRE_ROI cortical thickness (HR=2.309, 95% CI:1.046-5.096, P=0.038) were independent influencing factors of ALS survival. 【Conclusion】 Cortical thickness in A4hf (face-head) region can more objectively reflect UMN signs of region bulbar. In addition to age of onset and delayed time of diagnosis, bulbar subgroup score and cortical thickness of face-head region are also independent influencing factors, and cortical thinning in face-head region is a protective factor for survival of ALS patients.
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【Objective】 To explore the characteristics of white matter degeneration in amyotrophic lateral sclerosis (ALS) patients with different onset and spreading patterns by using diffusion tensor imaging (DTI). 【Methods】 We enrolled 86 ALS patients and 44 healthy controls. The patients were divided into bulbar- and spinal-onset subgroups according to their onset site, as well as horizon, vertical, interpose/skip, and caudal-rostral subgroups based on the spreading direction of the involved regions. The white matter fiber tracts corresponding to the motor network were set as the region of interest. We used tract-based spatial statistics to evaluate differences between the above groups and the normal controls, with family-wise error (FWE) correction and P<0.05 as statistical significance. 【Results】 The white matter degeneration of ALS patients with bulbar onset was mainly limited to the corona radiation part of the corticospinal tract, while those with spinal onset showed extensive degeneration of corticospinal tract and corpus callosum Ⅲ area (FWE correction, P<0.05). In patients with horizontal and vertical dissemination, decreased integrity of the entire corticospinal tract was found, with patients in the latter group showed extra degeneration in the Ⅲ part of the corpus callosum. Restricted degeneration of the corticospinal tract within bilateral corona radiata was detected in patients with caudal-rostral and interposed/skip spreading pattens (FWE correction, P<0.05). 【Conclusion】 Different onset and disease spread patterns of ALS patients correspond to divergent brain degeneration patterns. The diagnosis, treatment, and management of ALS should fully consider the heterogeneity of the disease.
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Amyotrophic lateral sclerosis (ALS) is a multi-system neurodegenerative disease characterized with degeneration of both motor and non-motor areas. Complicated clinical manifestations and lack of objective biomarkers for upper motor neuron deficits challenged the early diagnosis of ALS. Meanwhile, heterogeneous non-motor symptoms and conflicted treatment effects exacerbated the management and therapy of the disease. The multiparametric functional MRI has the potential to address all the needs for diagnosis, management, and disease modified therapy in ALS. The present paper summarizes the research progress in both motor and non-motor impairment in ALS, as well as their potential value in visualizing disease stages and drug effect evaluation. Focusing on the heterogeneity of the disease and combining with brain and spinal cord imaging may provide direct evidence for disease diagnosis and treatment and be the priority in the future for ALS.
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Objective:To evaluate changes of large-scale motor and cognition related networks′ function in patients with amyotrophic lateral sclerosis (ALS) and their relationship with corresponding clinical symptoms using independent component analysis combined with dual regression.Methods:Forty-six ALS patients (ALS group) who were treated in the First Affiliated Hospital of Xi′an Jiaotong University from January 2014 to June 2016 were prospectively collected, and 40 gender- and age-matched normal controls (control group) were recruited. All the participants completed the motor and multi-dimensional cognitive function evaluation[including Mini-mental State Examination (MMSE), Montreal Cognitive Assessment (MoCa), Semantic Fluency (SVF), Phonological Fluency (PVF), Digital Span Forward (DS_F), Digital Span backward (DS_B), frontal assessment battery (FAB), Wisconsin Card Sorting Test (WCST) for classification accuracy, classification error, persistent response classification, persistent error response classification, non-persistent error classification and Hamilton Depression Scale (HAMD), Hamilton Anxiety Scale (HAMA)]. The resting-state MRI data of all subjects were collected, and independent component analysis was carried out with multivariate interpretation linear optimization independent component decomposition. Dual regression analysis was performed to compare network differences between groups based on voxel level in sensorimotor network (SMN), default mode network (DMN) and frontal-parietal control network (FPCN). Multivariate covariance analysis was used to evaluate the differences of different cognitive function indexes between ALS group and normal control group, the comparison of brain network differences between the two groups was performed by nonparametric permutation test, corrected by family-wise error (FWE), P<0.008 as the statistical threshold; partial correlation and multiple linear regression were used to evaluate the relationship between changes in functional connectivity of different brain regions and cognitive functions. Results:The scores of MMSE, MoCa, SVF, PVF, DS_B, and classification accuracy were lower, while the number of error classifications, the non-persistent error classifications, HAMD and HAMA scores were higher in patients with ALS group than those in control group ( P<0.05). After adjusting for gender and age, there was no significant difference in the SMN between ALS group and control group (FWE correction, P>0.008). Compared with control group, patients with ALS showed increased functional connectivity in the left ventromedial prefrontal cortex (vmPFC) of the DMN, and decreased functional connectivity in the right anterior cingulate gyrus (ACC), the right posterior cingulate gyrus, the left inferior parietal lobule and the left inferior temporal gyrus of the FPCN (FWE correction, P<0.008). Increased functional connectivity of the vmPFC in ALS patients was negatively correlated with MoCa score ( r=-0.565, P<0.001), FAB score ( r=-0.373, P=0.015) and the classification accuracy of WCST ( r=-0.478, P=0.002), SVF ( r=-0.458, P=0.002) scores, and was positively correlated with the number of error classifications and HAMA scores ( r=0.416, P=0.007; r=0.388, P=0.011). Decreased functional connectivity were detected in multiple brain regions of FPCN, and the functional connectivity of the ACC was positively correlated with the DS_F ( r=0.341, P=0.027) and MMSE ( r=0.351, P=0.023). The effect of increased vmPFC functional connectivity accounted for 49.6% changes on MoCa score; 35.2% and 34.2% for FAB and HAMA respectively. While the impact of increased functional connectivity in the vmPFC was less than 30% on classification accuracy, classification error of WCST and SVF. The reduced functional connectivity in the ACC accounted for 37.7% impact on the DS_F score. Conclusions:Large-scale brain network changes are dominated by the frontotemporal core brain regions in ALS patients. DMN and FPCN network changes are closely related to the clinical cognitive performance of ALS patients.
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Objective To observe the effect of dexmedetomidine in cerebral aneurysm embolization in pa-tients with hypertension. Methods Sixty patients with hypertension undergoing emergency cerebral aneurysm em-bolization were randomly divided into two groups:research group(dexmedetomidine given group) and control group. SBP,DBP,HR,plasma norepinephrine(NE):baseline value(T0),before intubation(T1),after intubation (T2),after extubation(T3). Extubation time and anesthetic-related complications were also recorded. Results The hemodynamic parameters and plasma norepinephrine concentration in the research group were more stable than those in the control group at different time point during perioperative period. There was no difference between two groups about extubation time. The incidence of anesthetic-related complications in research group was lower than control group(P < 0.05). Conclusions Dexmedetomidine can reduce the stress response of intubation ,extuba-tion and perioperative hemodynamic fluctuations in cerebral aneurysm embolization in patients with hypertension. It can also reduce the consumption of prorofol and the incidences of adverse effects.
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PURPOSE: Secondary hyperparathyroidism (SHPT) develops in patients with chronic renal failure. This study aimed to compare analgesic efficacy in SHPT patients who undergo subtotal parathyroidectomy after superficial versus deep cervical plexus block. METHODS: Sixty chronic renal failure patients with SPHT scheduled for subtotal parathyroidectomy were randomized to receive general anesthesia (group GA), general anesthesia plus bilateral superficial and deep cervical plexus block (group BD), or general anesthesia plus bilateral superficial cervical plexus block (group BS) (n = 20). Bilateral superficial cervical plexus block or combined superficial and deep cervical plexus block with 0.5% ropivacaine was administered. Postoperative pain was assessed using visual analogue scale (VAS). RESULTS: VAS score at 1 hour, 4 hours, and 8 hours after operation was 3.71 +/- 0.60, 2.72 +/- 0.54, 2.17 +/- 0.75 in BS group; 4.00 +/- 0.28, 2.89 +/- 0.21, and 2.46 +/- 1.01 in BD group, significantly lower than in GA group (6.50 +/- 0.50, 5.02 +/- 0.54, and 4.86 +/- 0.51, respectively). The dosage of tramadol was 109.0 +/- 35.2 mg in BS group and 93.0 +/- 24.52 mg in BD group, significantly lower than in GA group (300.0 +/- 27.13 mg). The incidence of complications in GA group (90%) was significantly higher than in BS group (30%) and BD group (15%). Serum glucose and norepinephrine levels were significantly higher at 1 hour, 4 hours, and 8 hours after operation, but returned to baseline levels at 24 hours after operation. CONCLUSION: Superficial cervical plexus block or combined superficial and deep cervical plexus block effectively reduces postoperative pain, stress response, and complications in SHPT patients who undergo subtotal parathyroidectomy.
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Humans , Anesthesia, General , Blood Glucose , Cervical Plexus , Hyperparathyroidism, Secondary , Incidence , Kidney Failure, Chronic , Norepinephrine , Pain, Postoperative , Parathyroidectomy , TramadolABSTRACT
Objective To analyze the characteristics of the common first attack of headache in patients with cerebral venous sinus thrombosis (CVST). Methods The clinical data of 51 patients who were diagnosed as CVST with MR venography (MRV)or DSA were collected retrospectively. The patients were divided into either a acute group (≤3 week,n= 32)or a chronic group (>3 weeks, n=19). Results (1)The age of onset of symptoms in these patients was 20 to 40 years,and most of them were females. Of all the first symptoms,headache ranked first,accounting for 84. 3%(43/51 );headache only accounted for 52. 9%(27/51 ),headache with other symptoms (ophthalmic symptoms, hemiplegia,and aphasia,etc. )accounted for 31. 4%(16/51 ),and other symptoms such as epilepsy, paralysis,and ophthalmic symptoms accounted for 15. 7%(8/51). (2)The proportion of headache only as the first symptom in the acute group was higher than that in the chronic group (65. 6% vs. 31. 6%,P0. 05 ). Conclusion Young patients without previous history of migraine,especially women of childbearing age with sudden onset and progressive worsening headache,and the patients with idiopathic intracranial hypertension,CVST should be considered as an important possibility.
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Objective To evaluate the gray matter alterations in amyotrophic lateral sclerosis (ALS)by voxel-based morphometry (VBM),and further analyze the correlation between volume changes of gray matter and clinical characteristics.Methods Twenty-seven non-demented patients with ALS and 27 age and gender matched healthy controls were recruited.FSL-VBM was used to detect whole brain gray matter differences between the two groups.Seven prior ROIs were set to be analyzed,including bilateral precentral gyrus,postcentral gyrus,superior,medial,inferior,middle frontal gyrus,and insular cortex.The mean gray matter density of the ROIs was extracted in order to explore the correlation with several clinical measurements such as disease durations and disease severity scores,by using partial correlation analysis with age as covariates.Results Whole brain analysis showed significant gray matter loss in the left precentral gyrus,superior frontal gyrus and postcentral gyrus (numbers of voxel in clusters were 338,112,127,Z =4.83,4.09,6.42,P ＜0.05,FWE corrected).A prior seven ROIs analysis detected gray matter loss in the left precental gyrus,right precentral gyrus,left postcentral gyrus,superior frontal gyrus and left insular cortex (numbers of voxel in clusters were 1104,34,114,91,107,Z =5.87,3.71,4.26,6.29 and 3.51,P ＜0.05,FWE corrected).No statistical significant correlation between regional gray matter loss and clinical measurements were found.Conclusions Motor and extra-motor gray matter loss are present among patients with ALS,which demonstrates ALS as a multi-system disorder.In contrast to whole brain gray matter analysis,ROI analysis is more sensitive to detect extensive cortical changes.The heterogeneity of disease and sensitivity of method may contribute to the lack of correlation between gray matter volume decrease revealed by VBM and clinical characteristics.
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Objective To investigate the effects of single and combined exposure to ozone and nitrogen dioxide on airway injury and inflammatory levels in mice. Methods Eighty BALB/C mice were randomly divided into eight groups:four were sensitized model groups(the control group,the O3 group,the NO2 group and the O3+NO2 group based on the way of exposure) ,the other four were non-sensitized groups. The concentration of O3 was 0.16 mg/m3,NO2 was 0.30 mg/m3. Mice were exposed 2 hours every day for 7 consecutive days. Mice were sacrificed in the exposure end and cell counting and cytokine measurement from bronchoalveolar lavage fluid(BALF) were performed. Results In the sensitized groups,neutrophil proportion and IL-6 were significantly increased except the control group and IL-2 and MDA were also significantly increased in the O3 group and the O3+NO2 group(P