Objective To analyze the therapeutic effect of olfactory ensheathing cells (OECs) transplantation for central nervous system diseases. Methods Between November 2001 and January 2008, 1 255 participants with central nervous system diseases were enrolled in this cl inical study for fetal OECs transplantation. There were 928 males and 327 femalesaged 1.2-87 (mean 40) years. The course of disease was (4.52 ± 4.67) years. Among them, 656 participants suffered from chronic spinal cord injury (SCI), 457 amyotrophic lateral sclerosis (ALS), 68 cerebral palsy (CP), 20 multiple sclerosis (MS), 11 the sequelae of stoke, 10 ataxia, and 33 residual diseases. The participants came from 71 countries or regions. Accidentally abortional fetal olfactory bulbs were donated voluntarily and were cultured for 2 weeks, then were transplanted. Results One thousand one hundred and twenty-eight cases were followed up for 2-8 weeks (mean 4 weeks) to obtain integrated data. Among them, the neurological functional amel ioration was noticed in 994 participants with the overall short-term improvement rate of 88.12%. Seventy-six patients experienced the various perioperative compl ications with the incidence rate of 6.74%. One hundred and twenty patients with SCI received over 1 year follow-up. And according to ASIA assessment, motor scores increased from (39.82 ± 20.25) to (44.55 ± 18.99) points, l ight touch scores from (51.56 ± 25.89) to (59.81 ± 27.72) points, pain scores from (50.36 ± 27.44) to (57.09 ± 28.51) points for foreign patients (P lt; 0.05); motor scores increased from (40.52 ± 20.80) to (46.45 ± 20.35) points, l ight touch scores from (55.64 ± 26.32) to (68.64 ± 25.89) points, pain scores from (57.05 ± 26.00) to (66.13 ± 24.29) points for good rehabil itation Chinese patients (overall P lt; 0.05); motor scores from (37.03 ± 18.52) to (38.03 ± 18.50 points (P lt;0.05), l ight touch scores from (45.88 ± 22.56) to (46.63 ± 23.09) points (P gt; 0.05), pain scores from (45.25 ± 23.68) to (45.28 ± 23.63) points (P gt; 0.05) for poor rehabil itation Chinese patients. Compared foreign patients and good rehabil itation Chinese patients with poor rehabil itation Chinese patients, difference in score change was remarkable (P lt; 0.05). One hundred and six cases of ALS, 32 CP, 8 MS, 7 ataxia, and 2 stroke sequelae were followed up for 3-48, 3-36, 2-20, 7-17, 6 and 24 months, One hundred and six cases of respectively. Majority of them (113/155, 72.9%）were benefited from OECs transplantation. Conclusion OECs transplantation into brain and spinal cord is feasible and safe . The therapeutic strategy is valuable treatment for such central nervous system diseases such as chronic SCI, ALS, CP and stroke sequelae and can improve the patients’ neurological functions and/or decrease the progressive deterioration.
Objective To investigate the clinical features, diagnosis and treatment of symptomatic epilepsy complicated with central nervous system leukemia (CNSL) recurrence after acute lymphoblastic leukemia (ALL) treatment in children. MethodsThe clinical data of a child with secondary recurrence of CNSL complicated with symptomatic epilepsy after ALL treatment admitted to the Department of Pediatrics of the Second Affiliated Hospital of Auhui Medical University from December 2020 to February 2023 were retrospectively analyzed, and the relevant literature was reviewed and discussed. ResultsPatient was ALL for nealy two years after treatment in the central nervous system leukemia relapse of concurrent symptomatic epilepsy, two of the central nervous system leukemia relapse when starting symptoms are seizure, the first recurrence was status epilepticus, second recurrence of concurrent limb hemiplegia symptoms, cerebrospinal fluid, cranial magnetic resonance (MRI) and abnormal changes of electroencephalogram and clinical features, the abnormal changes of brain MRI lesions and electroencephalogram did not disappear. Chemotherapy, intrathecal injection and radiotherapy were given for the primary treatment, follow up CAR-T immunotherapy, and the treatment was successively combined with nalproate and levetiracetam. Currently, the seizures were controlled. ConclusionFor children with ALL, the recurrence of CNSL should be warned after the end of treatment. Cerebrospinal fluid, cranial imaging and electroencephalogram examination should be completed in time to confirm the diagnosis. If the crania imaging lesions persist after treatment and abnormal electroencephalogram discharge does not disappear, the possibility of CNSL recurrence should be warned when the epileptic seizures are repeated. On the basis of primary disease active treatment, combination of antiseizure medications is preferable.
Objective〓〖WTBZ〗To observe the clinical features of autoimmune optic neuropathy (AON). 〖WTHZ〗Methods〓 〖WTBZ〗The clinical data of 58 patients with AON from Jan. 2006 to Dec. 2007 were retrospectively analyzed. The patients had undergone routine ophthalmological, neurological examination, visual field test, all set of autoimmune antibody test, brain MRI. 〖WTHZ〗Results〓〖WTBZ〗In 93 eyes of 58 patients with AON, the lowest best corrected visual acuity (BCVA) was lt;01 in 68 eyes (731%), 10 patients (172%) had other symptoms of nervous system, 14 patients (241%) had lesions of nonneurological system. Positive antinuclear antibody was found in 43 patients (796%); other abnormal antibodies were also found, including antiSSA/SSB, antidsDNA, antihistonic, anticardiolipin, and antihuman leukocyte antigen B27 antibodies. Systematic connective tissue disease presented in 20 patients (345%), such as sicca syndrome, systemic lupus erythematosus, and Behcet disease. 32 patients (552%) had abnormal brain MRI, and the BCVA of 49 eyes (777%) improved significantly after hospitalization. 〖WTHZ〗Conclusion〓〖WTBZ〗Patients with AON always have poor visual function, some of whom associate with other systems, as well as damages to other parts of the nervous system. While some AON patients are secondary to systemic connective tissue disease involving the optic nerve, the majority of these patients are isolated autoimmune optic neuropathy.
ObjectiveTo explore the expression of growth associated protein-43 (GAP-43) in spasm segment and expansion segment of hirschsprung disease (HD), and to explore the pathogenesis of HD. MethodsThe expression of GAP-43 in 30 patients with HD who underwent surgical resection for absence of enteric plexuses from Jan. 2012 to Jun. 2013 in Shen zhen Children's Hospital were analyzed by using immunohistochemistry method and real-time PCR method. Aganglionic tissues of all patients were included as spasm group, and ganglionic tissues of the same patients were served as expansion group. Then comparison of the expression levels of GAP-43 mRNA and its protein between 2 groups was performed. Resultsof real-time RCR showed that the expression level of GAP-43 mRNA in expansion group was higher than that of spasm group (0.119 0 vs. 0.052 8, P<0.05). Immunohistochemistry results showed that GAP-43 protein expressed both in the myenteric plexus and ganglionic plexus of submucos in all patients, but lighter in spasm group. Compared with ganglionic plexus of circular muscle layer and longitudinal muscle layer/ganglionic plexus of submucosa in expansion group, the average optical density values at corresponding sites of intestinal tissues in spasm group were both lower (P<0.05). ConclusionExpression of GAP-43 protein is lower in spastic intestinal tissue of patients with HD, which suggests that down-regulation of GAP-43 protein may be a risk factor for HD.
ObjectiveTo analyze the effect of carotid artery stenosis degree and intervention for carotid artery stenosis on the incidence of central nervous system complications after off-pump coronary artery bypass grafting (OPCABG) and explore the influencing factors. MethodsA total of 1 150 patients undergoing OPCABG in our hospital from June 2018 to June 2021 were selected and divided into two groups according to whether there were central nervous system complications, including a central nervous system complication group [n=61, 43 males and 18 females with a median age of 68.0 (63.0, 74.0) years] and a non-central nervous system complication group [n=1 089, 796 males and 293 females with a median age of 65.5 (59.0, 70.0) years]. The risk factors for central nervous system complications after OPCABG were analyzed. ResultsUnivariate analysis showed that age, smoking, hyperlipidemia, preoperative left ventricular ejection fraction, intra-aortic ballon pump (IABP), postoperative arrhythmia, postoperative thoracotomy and blood transfusion volume were associated with central nervous system complications. The incidence of central nervous system complications in patients with severe carotid artery stenosis or occlusion (11.63%) was higher than that in the non-stenosis and mild stenosis patients (4.80%) and moderate stenosis patients (4.76%) with a statistical difference (P=0.038). The intervention for carotid artery stenosis before or during the operation did not reduce the incidence of central nervous system complications after the operation (42.11% vs. 2.99%, P<0.001). Age, postoperative arrhythmia, severe unilateral or bilateral carotid artery stenosis and occlusion were independent risk factors for postoperative central nervous system complications (P<0.05). Conclusion The age, smoking, hyperlipidemia, preoperative left ventricular ejection fraction, intraoperative use of IABP, postoperative arrhythmia, secondary thoracotomy after surgery, blood transfusion volume and OPCABG are associated with the incidence of postoperative central nervous system complications in patients. Age, postoperative arrhythmia, severe unilateral or bilateral carotid artery stenosis and occlusion are independent risk factors for postoperative central nervous system complications. In patients with severe carotid artery stenosis, preoperative treatment of the carotid artery will not reduce the incidence of central nervous system complications.
Objective To investigate the cl inical features of mal ignant melanoma (MM) in the central nervous system (CNS) and to improve the diagnosis and treatment of this disease. Methods Seven MM-in-CNS patients’ records between September 1996 and April 2007 were analyzed retrospectively, including 6 males and 1 female aged 18-74 years. The 5 cases were located in the supra-tentorial area, 1 in the spinal cord and 1 in the whole brain. CT or MRI scan was appl ied. The lesion was in the right frontal area in 4 cases, in the right temporal are in 1 case, in the left temporal area in 1 case, in the left apex area in 1 case and in the cervical spinal cord of C5-7 in 1 case. Six patients underwent neurosurgical operation and1 patient received the Gamma Knife therapy. The pathological examination revealed that 2 cases were metastatic MM and 5 were primary. Results One patient with primary MM received no follow-up, and the rest 6 patients were followed up for 2 weeks to 2 years with the time of median 8 months. One patient with metastatic MM died 2 months after operation, 1 patient to with metastatic MM died 2 weeks after Gamma-Knife treatment, 1 patient with metastatic MM with primary MM died 2 years after operation, and 3 patients with primary MM were still al ive and self-independent 6, 10 and 24 months after operation, respecti vely. Conclusion Since MM-in-CNS is short of specificity in cl inical symptoms and signs, its diagnosis mainly rel ies on the pathological examination and is assisted by MRI. The combined therapy giving priority to operation is recommended.
Objective To review the progress of the research on therelationship between the central nervous system injury and the heterotopic ossification. Methods The recent articles on the central nervous system injury and the heterotopic ossification were extensively reviewed, and the related clinical signs, symptoms, pathogenesis diagnosis, risk factors, prophylaxis, and treatment of the neurogenic heterotopic ossification were investigated. Results The possible mechanism for the neurogenic heterotopic ossification might involve the roles of thebone morphogenetic protein and the basic fibroblast growth factor, which were suggested as mediators in differentiation of the progenitor cells. Sonographic, serum creatine phosphokinase, and C-reactive protein were recommended as the useful screening tools for heterotopic ossification. Colchicine and rofecoxib could be used for the prophylaxis or treatment of heterotopic ossification. Conclusion The research on the neurogenic heterotopic ossification has achieved a great development but further studies in this field are still required.
This article investigates the role of AMP-activated protein kinase (AMPK) and its downstream signaling targets in mediating cellular processes such as autophagy, apoptosis, and inflammation, offering insights into how acupuncture may treat common central nervous system (CNS) diseases, including ischemic stroke, spinal cord injury, Parkinson disease, and Alzheimer disease. AMPK and its downstream effectors are pivotal in the signaling pathways that underlie the pathophysiology of CNS diseases. These pathways are implicated in a variety of cellular responses that contribute to the progression of neurological disorders. During CNS injury, AMPK can be activated through phosphorylation, triggering the regulation of downstream molecules and exerting protective effects on neuronal function. Acupuncture has been shown to promote neuroprotection and enhance recovery in CNS diseases through multiple mechanisms, one of which involves the activation of AMPK-related signaling pathways. Nevertheless, numerous unresolved challenges remain in this research field.
ObjectiveTo observe and analyze the clinical characteristics and correlation between the eye and nervous system in children with infantile gangliosideosis.MethodsFrom November 2018 to January 2021, 3 children with infantile ganglion lipidosis diagnosed by genetic examination in the Department of Ophthalmology and Neurology, Beijing Children's Hospital of Capital Medical University, and through China National Knowledge Infrastructure and Wanfang database and The National Library of Medicine of the United States (PubMed) were searched, and 53 cases of Chinese infantile gangliosideosis diagnosed by gene, enzyme activity or pathological examination were selected and a total of 56 cases were included in the study. The searching time was from the establishment of the database to February 2021, and the search keywords are "gangliosideosis", "cherry-spot" macula and "Chinese". The demographic characteristics of 56 cases of children and other system manifestations were analyzed such as eyes, nervous system, skin, bones. According to the presence or absence of cherry-spot (CS) on the fundus examination, the children were divided into a fundus CS group (group A) and a fundus without CS group (group B), with 20 and 27 cases, respectively. The age of onset, gender, different types and neurological manifestations of the two groups of children were compared and analyzed. The non-parametric rank sum test was used for age comparison between groups; the χ2 test or Fisher's exact test were used for the comparison of gender, disease type and incidence between groups.ResultsAmong the 56 children, 27 were males and 29 were females; the median age of onset was 7.0 months. There were 33 and 23 cases of GM1 and GM2, respectively. Among 44 children with visual function examination records, 41 cases (93.2%, 41/44) were unable to follow the visual object. Of 47 children who underwent ocular fundus examination, 20 cases (42.6%, 20/47) had CS on the fundus. The main manifestations of the nervous system are neuromotor development regression or retardation (100%, 56/56), convulsions (58.1%, 25/43), and "startle" phenomena (89.7%, 26/29). Among 42 patients with brain magnetic resonance imaging examination records, 39 cases (92.9%) were abnormal. The incidence of "startle" and seizures in group A was higher than that in group B, and the difference was statistically significant (χ2=5.815, 6.182, P=0.021, 0.013).Conclusios Chinese infantile gangliosideosis is more common in GM1 type. Ocular visual impairment is the visual object as the main manifestation, the incidence of fundus CS is 42.6%, and the symptoms of neurological damage in children with CS are more severe.
ObjectiveTo summarize the current research progress on the changes of enteric glial cells (EGCs) in intestinal motility disorders and its possible molecular mechanisms in regulating intestinal motility.MethodThe literatures related to the EGCs and intestinal dysmotility were collected and analyzed.ResultsThe EGCs were involved in the occurrence and development of intestinal motility disorders, and there were abnormalities in the quantity, receptor, and phenotype in the different dysmotility diseases such as the postoperative ileus, Hirschsprung disease, inflammatory bowel disease, diabetes and so on. It could sense the neuronal signals and communicate with the enteric neurons via Ca2+ response and connexin-43 to affect the intestinal motility.ConclusionStudy of role and mechanism of EGCs in intestinal motor dysfunction is helpful to discovery new targets for treatment of these diseases.