Fragile X syndrome (FXS) is a neurodevelopmental synaptopathy caused by loss of fragile X mental retardation protein (FMRP); abnormal synaptic plasticity is the leading pathological cause of cognitive impairment, fear and anxiety, hyperactivity and stereotyped behavior in FXS patients. In recent years, breakthroughs have been made in functional study of synaptic plasticity in FXS, providing a new theoretical basis for FXS. This article mainly summarizes the dysregulation and influencing factors of synaptic plasticity in FXS, as well as the strategy of targeted synaptic plasticity in FXS, so as to deepen the understanding of medical workers.

Traumatic supraorbital fissure syndrome (TSOFS) is a symptom complex caused by nerve entrapment in the supraorbital fissure after skull base trauma. If the compressed cranial nerve in the supraorbital fissure is not decompressed surgically, ptosis, diplopia and eye movement disorder may exist for a long time and seriously affect the patients′ quality of life. Since its overall incidence is not high, it is not familiarized with the majority of neurosurgeons and some TSOFS may be complicated with skull base vascular injury. If the supraorbital fissure surgery is performed without treatment of vascular injury, it may cause massive hemorrhage, and disability and even life-threatening in severe cases. At present, there is no consensus or guideline on the diagnosis and treatment of TSOFS that can be referred to both domestically and internationally. To improve the understanding of TSOFS among clinical physicians and establish standardized diagnosis and treatment plans, the Skull Base Trauma Group of the Neurorepair Professional Committee of the Chinese Medical Doctor Association, Neurotrauma Group of the Neurosurgery Branch of the Chinese Medical Association, Neurotrauma Group of the Traumatology Branch of the Chinese Medical Association, and Editorial Committee of Chinese Journal of Trauma organized relevant experts to formulate Chinese expert consensus on the diagnosis and treatment of traumatic supraorbital fissure syndrome ( version 2024) based on evidence of evidence-based medicine and clinical experience of diagnosis and treatment. This consensus puts forward 12 recommendations on the diagnosis, classification, treatment, efficacy evaluation and follow-up of TSOFS, aiming to provide references for neurosurgeons from hospitals of all levels to standardize the diagnosis and treatment of TSOFS.

ObjectiveTo comprehensively elucidate the potential mechanisms of Xiao Xumingtang （XXMT） combined with electroacupuncture （EA） collaboratively in alleviating cerebral ischemia/reperfusion （I/R） injury. MethodThe rat model of cerebral I/R injury was established using the modified suture-occluded method. Seven days after modeling， rats in the XXMT+EA groups were administered XXMT at low （15 g·kg^-1）， medium （30 g·kg^-1）， and high （60 g·kg^-1） doses， alongside daily 20-min EA treatment （stimulating acupoints GV14 and GV20）. Cerebral infarction and neuronal damage were evaluated using the Zea Longa test score， TTC staining， and TUNEL staining. Real-time fluorescence quantitative PCR and Western blot were used to detect the expression of NOD-like receptor hot protein domain related protein 3 （NLRP3）， Gasdermin D （GSDMD）， cysteinyl aspartate specific proteinase-1 （Caspase-1）， interleukin-1β （IL-1β）， and interleukin-18 （IL-18） in the ischemic area of the cerebral cortex. ResultCompared with the sham group， the I/R group showed a significant increase in neurological deficit scores and infarct volume （P<0.01）， along with a higher apoptosis rate of cortical neurons and elevated mRNA and protein expression levels of NLRP3， GSDMD， Caspase-1， IL-1β， and IL-18 （P<0.05）. In contrast， the medium- and high-dose XXMT combined with EA treatment significantly reduced neurological deficit scores and infarct volume （P<0.01）， and decreased the apoptosis rate of cortical neurons as well as the mRNA and protein expression levels of NLRP3， GSDMD， Caspase-1， IL-1β， and IL-18 （P<0.05）. The improvement showed a dose-dependent relationship with XXMT. ConclusionThe combined use of XXMT and EA can exert neuroprotective effects by modulating the NLRP3/GSDMD/Caspase-1 signaling pathway， thereby reducing neurological deficits， minimizing brain infarct size， and improving cortical neuronal damage.

Objective:To establish a 14-day prognosis model for emergency patients with acute ischemic cerebral stroke and evaluate its predictive efficacy.Methods:A prospective cohort study was conducted. Patients with acute ischemic stroke admitted to the emergency department of Beijing Bo’ai Hospital within 72 hours of onset from October 2018 to December 2020 were enrolled. Univariate and multivariate logistic regression analysis were used to screen the risk factors of poor prognosis. The ROC curve was drawn to determine the cut-off value of continuous variables and discretise data with reference to clinical practice. The corresponding scores were set up according to the β regression coefficient of each variable, and the clinical scale prediction model of short-term prognosis of acute cerebral infarction was established. Patients with ischemic stroke in the hospital from January to December 2021 were selected as the internal validation, to verify the constructed predictive model.Results:A total of 321 patients were included in the study, including 223 in the training set and 98 in the internal validation set. Multivariate logistic regression analysis showed that age, hypersensitive C-reactive protein, prealbumin (PA), infarct volume, Frailty Screening Questionnaire (FSQ) and National Institute of Health Stroke Scale (NIHSS) were independent risk factors for poor short-term prognosis of acute cerebral infarction. The total score of the clinical prediction scoring system for short-term prognosis of acute cerebral infarction in the emergency department was 15 points, including age ≥74 years (1 point), PA ≤373 mg/L (2 points), large artery atherosclerosis (1 point), cardiogenic embolism (2 points), infarct volume ≥ 2.18 cm 3 (2 points), FSQ ≥3 points (1 point), NIHSS ≥4 points (6 points); The area under the ROC curve (AUC) of the scoring system for predicting short-term poor prognosis of acute cerebral infarction was 0.927 (95% CI: 0.894-0.960). The optimal cut-off value was ≥5 points, and the sensitivity and specificity were 0.770 and 0.976, respectively. In the internal validation set, the scoring system had similar predictive value for poor outcomes (AUC=0.892, 95% CI:0.827-0.957). Conclusion:The scoring system for short-term prognosis prediction of acute ischemic cerebral infarction has good diagnostic efficacy, and could guide clinicians to judge the prognosis of emergency patients in the early stage.

Objective·To assess the accuracy and time of the two-in-one registration technique by comparing it with the U-shaped tube registration in dynamic navigation implantation.Methods·Thirty standardized 3D-printed models with mandibular posterior sites missing a single tooth were randomly divided into three groups:two-in-one registration group,U-shaped tube registration group and free-hand implantation group,and the implant surgical plan was designed by the"YIZHIMEI"DCARER oral implant surgery navigation system.Cone beam CT before and after operation was taken.The implant platform deviation,implant apex deviation and angular deviation of the actual implant positions and the designed implant positions were measured.The operating time for using two-in-one registration technique and the U-shaped tube registration technique was recorded to evaluate the complexity of the two registration techniques.The one-way ANOVA and SNK(Student-Newman-Keuls)test were used to analyze the implant platform deviation,implant apex deviation and angular deviation of each group.Results·There were no statistically significant differences in implant platform deviation,implant apex deviation and angular deviation between the two-in-one registration group and the U-shaped tube registration group(P>0.05).However,the implant platform deviation,implant apex deviation and angular deviation of the two-in-one registration group and the U-shaped tube group were lower than those in the free-hand implantation group,and the differences were statistically significant(P<0.001).The operating time required for the two-in-one registration was shorter than that for the U-shaped tube registration process,and the difference was statistically significant(P<0.001).Conclusion·The accuracy of the two-in-one dynamic navigation registration technique used in implanting on a model of mandibular posterior sites missing a single tooth is similar to that of the U-shaped tube dynamic navigation registration technique.But the two-in-one registration takes less time for registration procedure than the U-shaped tube registration,and is easier to operate.

Primary familial brain calcification (PFBC) is an inherited neurodegenerative disorder mainly characterized by progressive calcium deposition bilaterally in the brain, accompanied by various symptoms, such as dystonia, ataxia, parkinsonism, dementia, depression, headaches, and epilepsy. Currently, the etiology of PFBC is largely unknown, and no specific prevention or treatment is available. During the past 10 years, six causative genes (SLC20A2, PDGFRB, PDGFB, XPR1, MYORG, and JAM2) have been identified in PFBC. In this review, considering mechanistic studies of these genes at the cellular level and in animals, we summarize the pathogenesis and potential preventive and therapeutic strategies for PFBC patients. Our systematic analysis suggests a classification for PFBC genetic etiology based on several characteristics, provides a summary of the known composition of brain calcification, and identifies some potential therapeutic targets for PFBC.
Animals ; Brain Diseases/therapy* ; Xenotropic and Polytropic Retrovirus Receptor ; Brain/pathology*

ObjectiveTo explore the mechanism of Renshen Baidusan in repairing intestinal mucosa in ulcerative colitis （UC） by regulating autophagy to scavenge peroxides. MethodThe mouse model of UC was induced by free drinking of 3.0% dextran sulphate sodium （DSS） solution. Sixty male C57BL/6J mice were randomized into normal， model， mesalazine （0.3 g·kg^-1）， and high-， medium-， and low-dose （12.35， 8.22， 4.11 g·kg^-1， respectively） Renshen Baidusan groups （n=10）. The mice were administrated with corresponding drugs by gavage for 7 consecutive days. The colon tissue was stained with hematoxylin-eosin （HE） to reveal the pathological changes， and Alcian blue-Periodic acid Scheff （PAS/AB） staining was employed to observe the goblet cell changes. The fluorescence expression of reactive oxygen species （ROS） in the colon tissue was detected by the immunofluorescence assay. The activity of superoxide dismutase （SOD） and the content of malondialdehyde （MDA） were measured by the biochemical methods. Western blot was employed to determine the expression levels of proliferating cell nuclear antigen （PCNA）， microtubule-associated protein 1 light chain 3 （LC3）， leucine-rich repeat-containing G protein-coupled receptor 5 （LGR5）， and p62. ResultDestroyed mucosal epithelial structure， intestinal gland destruction， loss of goblet cells， and massive infiltration of inflammatory cells appeared in the model group. Compared with the normal group， the model group showed increased tissue damage injury （TDI） score of the colon tissue， decreased SOD activity and LC3Ⅱ/Ⅰ， PCNA value， and elevated levels of p62， MDA， ROS， and LGR5 （P<0.05）. Compared with the model group， different doses of Renshen Baidusan decreased the TDI score， promoted the generation of new goblet cells， elevated the levels of PCNA， LGR5， SOD， and LC3Ⅱ/Ⅰ， and lowered the levels of p62， MDA， and ROS （P<0.05）. Moreover， the low dose group showed the best performance （P<0.05）. ConclusionRenshen Baidusan can promote intestinal epithelial repair by activating intestinal autophagy， alleviating oxidative stress， and promoting intestinal stem cell proliferation and differentiation.

ObjectiveTo summarize the history and modern clinical application of Renshen Baidusan. MethodThe bibliometric method was used to retrieve the relevant publications of Renshen Baidusan from the ancient book database and China National Knowledge Infrastructure （CNKI）. The publications were screened according to the inclusion and exclusion criteria. The information of dynasty， book title， function， dosage and so on was extracted， on the basis of which the history， composition， dosage， decocting method， original medicinal plants， processing， and modern clinical application of this prescription were analyzed. ResultRenshen Baidusan was first recorded in the Formulary of the Bureau of Taiping People's Welfare Pharmacy， consisting of Bupleuri Radix， Glycyrrhizae Radix et Rhizoma， Platycodonis Radix， Ginseng Radix et Rhizoma， Chuanxiong Rhizoma， Poria， Aurantii Fructus， Peucedani Radix， Notopterygii Rhizoma et Radix， Angelicae Pubescentis Radix， Zingiberis Rhizoma Recens， and Menthae Haplocalycis Herba and with the effect of dispersing cold， removing dampness， reinforcing Qi， and relieving exterior. Later generations of physicians used this prescription on the basis of the record in Formulary of the Bureau of Taiping People's Welfare Pharmacy to treat cold （frequency of 112， 22.63%） and seasonal cold （frequency of 83， 16.77%）. Renshen Baidusan is widely used in modern clinical practice to treat respiratory diseases （frequency of 42， 17.65%）， skin diseases （frequency of 34， 14.29%）， and infectious diseases （frequency of 33， 13.87%）. This prescription is often modified to treat the syndrome of internal deficiency and external contraction， or external contraction of wind， cold and damp pathogens without deficiency of healthy Qi， which fully embodies the concept of treating different diseases with the same method in traditional Chinese medicine. ConclusionThe textual research reveals the key information of the classical prescription Renshen Baidusan， providing a basis for the subsequent development and application of compound preparations.

Objective:To evaluate the feasibility of the screw and plate for clival fixation using a transoral expanded approaches.Methods:The transoral expanded approaches were performed on craniocervical segment specimens obtained from 7 subjects, including transoral approach (TO), transoral with soft (TOP) or hard (TOHP) palate split, mandibulotomy (MO) and mandibuloglossotomy (MLO). The distribution and thickness of soft tissue, the configuration of the vertebral arteries, the distance between the midline and the vertebral arteries, the exposed area of the clivus and cervical spine, and the range of screw angle (the angle between the line from the lower incisor or the central base of the mandible to the exposed area of the clivus and the tangent line of the clivus) were evaluated.Results:The thickness of the soft tissue on the posterior pharyngeal wall above the clival pharyngeal nodules was 3.5±0.6 mm. That on the anterior C 1-C 5 vertebrae was 5.0±0.5 mm. The distances from the bilateral vertebral arteries to the midline was 19.5±1.2 mm at C 1, 2, 14.6±2.7 mm at C 2, 3, 14.0±2.7 mm at C 3, 4, and 13.9±2.7 mm at C 4, 5. For the TO approach, the longitudinal diameter of the exposed clivus was 8.3±3.0 mm. The distance from the lower incisor to the superior margin of the exposed clivus, the lower margin of the exposed clivus, the anterior arch of C 1, the vertebral body of C 2 and C 3 were 104.7±4.3 mm, 99.2±6.8 mm, 81.4±4.3 mm, 75.1±4.0 mm and 68.7±6.5 mm, respectively. Six specimens were exposed to the C 3, while one was exposed to the C 2. For the TOP approach, the longitudinal diameter of the exposed clivus was 18.5±4.8 mm. The distance from the lower incisor to the superior margin of the exposed clivus and the pharyngeal nodules were 107.9±6.7 mm and 104.8±6.7 mm, respectively. For the TOHP approach, the longitudinal diameter of the exposed clivus was 26.3±1.8 mm (the clival length) with distance from the lower incisor to the superior margin of the clivus 112.4±12.6 mm. For the MO/MLO approach, the entire clivus was exposed. The distance from the central base of the mandible to the superior and inferior margin of the exposed clivus and the pharyngeal nodules were 141.8±15.7 mm, 131.0±9.9 mm and 120.5±8.2 mm, respectively. The inferior margin of the exposed cervical vertebra was C 5, 6. The rate of the clival screw placement through anterior occipitocervical fixation using TO, TOP, TOHP, MO and MLO was 0%, 71% (5/7), 86% (6/7), and 100%, respectively. The screw angle was 99.0°±1.8°, 92.6°±7.7°, 92.6°±7.7°, 75.1°±7.7°, and 75.1°±7.7°, respectively. Conclusion:Occipitocervical fixation with clival screw and plate could be conducted in most cases via TOP and TOHP approaches. However, in some cases with small split-mouth or mouth opening limited, smaller clival screw angle caused by basilar impression or basilar invagination, requiring fixation and reconstruction of the lower cervical spine, and the MO/MLO approaches could be still required to achieve the fixation.

Objective:To test the usefulness of PET-range verification (RV) method for proton radiation accuracy verification in poly (methyl methacrylate) (PMMA) phantom using off-line PET/CT scanning.Methods:Proton irradiation dose of 2 Gy and 4 Gy were delivered in PMMA phantom. Given the difference of clinical target volume (CTV), 7 subgroups with different depth (5.0, 7.5, 10.0, 12.5, 15.0, 17.5, 20.0 cm) were set for each dose (14 radiation plans or radiation fields). PET/CT scan was performed 10 min after irradiation of 48-221 MeV proton beam. A co-registration between CT from treatment planning system and PET/CT was performed, as well as the smoothing and normalization of PET/CT data. The region of interest (ROI) and profile lines were drawn with the Raystation PET-RV software. The predictive induced radioactivity and the measured induced radioactivity profile lines were analyzed to evaluate the Δ R50, namely, the error at the position corresponding to 50% of the maximum predictive induced radioactivity at the end of both curves. Results:The size of each ROI was 5.0 cm×5.0 cm×2.5 cm. Profile lines were evenly distributed with the interval of 3 mm, and totally 289 pairs of profile lines were drew. The 2 Gy- and 4 Gy-dose groups yielded similar mean depth errors (Δ R50 between 1 mm and -1 mm with a standard deviation <1 mm). Conclusions:The off-line PET/CT scanning of PMMA phantom reveals a good agreement between predicted and measured PET data, with error of ±1 mm. The PET-RV method can be extended to clinical cases′ verification in human body treatment with further investigation.