皮下注射密盖息加局部神经阻滞治疗Sudeck骨萎缩二例报道

目的介绍一种治疗Sudeck骨萎缩的有效方法。方法用利多卡因在患病关节作局部环行阻滞封闭,并同时皮下注射密盖息治疗。结果本组2例均于上述治疗后1d后疼痛有所减轻,3～4d后肿胀明显消退,4w1疗程后复查,X线光摄片检查示骨质疏松较前有所改善。一定疗程后治愈率为100%。结论用此方法治疗Sudeck骨萎缩效果良好,是临床上治疗的理想方法,值得推广应用。

郑氏伤科针灸结合中药治疗桡骨远端骨折并发Sudeck骨萎缩的临床疗效观察

目的:观察郑氏伤科针灸结合中药治疗桡骨远端骨折并发Sudeck骨萎缩的临床疗效。方法:选取68例桡骨远端骨折并发Sudeck骨萎缩患肢,随机分为对照组和观察组,对照组患者采用单纯针刺治疗,观察组采用郑氏伤科针灸结合中药治疗,2组患者均治疗8周。比较2组患者治疗前后临床症状体征、Cooney腕关节功能评分及临床疗效评价,并进行统计分析。结果:治疗后2组患者的疼痛程度、关节活动度、压痛、皮肤颜色改变评分及肿胀度均较治疗前明显降低(P<0. 05);并且观察组治疗后除压痛评分外的各项评分显著较对照组低(P<0. 05);治疗后2组患者的Cooney腕关节功能各项评分均较治疗前明显升高(P<0. 05);并且观察组治疗后改善幅度较对照组大(P<0. 05);观察组总有效率93. 9%,对照组总有效率79. 4%,差异显著(P<0. 05)。结论:郑氏伤科针灸结合中药治疗桡骨远端骨折并发Sudeck骨萎缩的疗效确切,为临床治疗提供有益参考。

External anal sphincter electromyography in multiple system atrophy:implications for diagnosis,clinical correlations,and novel insights into prognosis

Multiple system atrophy is a sporadic,progressive,adult-onset,neurodegenerative disorder characte rized by autonomic dysfunction symptoms,parkinsonian features,and cerebellar signs in va rious combinations.An early diagnosis of multiple system atrophy is of utmost impo rtance for the proper prevention and management of its potentially fatal complications leading to the poor prognosis of these patients.The current diagnostic criteria incorporate several clinical red flags and magnetic resonance imaging marke rs supporting diagnosis of multiple system atrophy.Nonetheless,especially in the early disease stage,it can be challenging to differentiate multiple system atrophy from mimic disorders,in particular Parkinson’s disease.Electromyography of the external anal sphincter represents a useful neurophysiological tool for diffe rential diagnosis since it can provide indirect evidence of Onuf’s nucleus degeneration,which is a pathological hallmark of multiple system atrophy.However,the diagnostic value of external anal sphincter electromyography has been a matter of debate for three decades due to controve rsial reports in the literature.In this review,after a brief ove rview of the electrophysiological methodology,we first aimed to critically analyze the available knowledge on the diagnostic role of external anal sphincter electromyography.We discussed the conflicting evidence on the clinical correlations of neurogenic abnormalities found at external anal sphincter electro myography.Finally,we repo rted recent prognostic findings of a novel classification of electromyography patterns of the external anal sphincter that could pave the way toward the implementation of this neurophysiological technique for survival prediction in patients with multiple system atrophy.

Screening for celiac disease in Down's syndrome patients revealed cases of subtotal villous atrophy without typical for celiac disease HLA-DQ and tissue transglutaminase antibodies

A1M： To investigate the prevalence of celiac disease （CD） as well as CD marker antibodies and susceptibility HLA-DQ haplotypes in 134 karyotyped Down＇s syndrome （DS） patients. METHODS： Immunoglobulin A （IgA） and G （IgG） type anti-gliadin antibodies （AGA）, IgA type anti-tissue transglutaminase （tTG） antibodies （anti-tTG） with antigen of guinea pig and human source were determined by enzyme-linked immunosorbent assay and endomysium antibodies （EHA） by indirect immunofiuoresence test. HLA-DQA1＊0501/DQB1＊0201 （DQ2） was revealed by polymerase chain reaction. Celiac disease was diagnosed by revised ESPGHAN criteria. RESULTS： 41% of DS patients had AGA, 6.0% IgA anti-tTG with guinea pig antigen, and 3.0 % [gA EMA （all positive for anti-tTG with human tTG）. Subtotal villous atrophy was found in 5 out of 9 DS patients who had agreed to small bowel biopsy. One of them had DQA1＊0S01/DQB1＊0201 and anti-tTG and EMA i.e. typical for CD markers （this case also fulfilled the ESPGHAN diagnostic criteria）, but other four lacked these markers. Three non-biopsied DS patients had also most probably CD because DQA1＊0S01/DQB1＊0201 and IgA anti-tTG （EMA） were detected. Thus, the prevalence of CD among our DS patients population is 3.0 % （95 % of confidence interval [CI]： 0.1-5.9 %）. CONCLUSION： We confirm the increased frequency of CD among DS patients. In addition, we have revealed a subgroup of patients with subtotal villous atrophy but without characteristic for CD immunological and genetic markers. Whether these cases represent CD （with atypical immunopathogenesis） or some other immune enteropathy, requires further investigations.

Elevated serum growth differentiation factor 15 in multiple system atrophy patients:A case control study

BACKGROUND Multiple system atrophy(MSA) is a serious progressive neurodegenerative disease. Early diagnosis of MSA is very difficult, and diagnostic biomarkers are limited. Growth differentiation factor 15(GDF15) is involved in the differentiation and progression of the central nervous system, and is widely distributed in peripheral blood, which may be a novel biomarker for MSA.AIM To determine serum GDF15 levels, related factors and their potential diagnostic value in MSA patients, compared with Parkinson’s disease(PD) patients and healthy controls.METHODS A case-control study was conducted, including 49 MSA patients, 50 PD patients and 50 healthy controls. Serum GDF15 levels were measured by human enzymelinked immunosorbent assay, and the differences between the MSA, PD and control groups were analyzed. Further investigations were performed in different MSA subgroups according to age of onset, sex, clinical subtypes, diagnostic criteria, and disease duration. Receiver-operating characteristic curve analysiswas used to evaluate the diagnostic value of GDF15, especially for the differential diagnosis between MSA and PD.RESULTS Serum GDF15 levels were significantly higher in MSA patients than in PD patients and healthy controls(P = 0.000). Males and those with a disease duration of more than three years showed higher serum GDF15 levels(P = 0.043 and 0.000;respectively). Serum GDF15 levels may be a potential diagnostic biomarker for MSA patients compared with healthy controls and PD patients(cutoff: 470.42 pg/m L, sensitivity: 85.7%, specificity: 88.0%;cutoff: 1075.91 pg/m L, sensitivity:51.0%, specificity: 96.0%;respectively).CONCLUSION Serum GDF15 levels are significantly higher in MSA patients and provide suggestions on the etiology of MSA.

补肾活血汤治疗桡骨远端骨折后期并发Sudeck骨萎缩患者的效果分析

目的:分析补肾活血汤治疗桡骨远端骨折后期并发Sudeck骨萎缩患者的临床效果。方法:选取2018年1月-2020年1月收治的76例桡骨远端骨折后期并发Sudeck骨萎缩患者作为本次研究的对象,应用双色球法随机分为针刺组和试验组,各38例。针刺组应用针刺进行治疗,试验组在针刺组基础上加用补肾活血汤进行治疗。比较分析两组临床疗效,治疗前后的临床症状体征、腕关节功能、骨密度。结果:相比针刺组,试验组的总有效率显著更高(P <0.05)。治疗后,相比针刺组,试验组视觉模拟评分法(Visual Analogue Scale,VAS)、关节活动度、压痛及皮肤颜色评分及肿胀体积显著更低(P <0.05)。治疗后,相比针刺组,试验组疼痛、功能状况、活动度百分比、屈伸活动度、握力评分显著更高(P <0.05)。治疗4、8周后,相比针刺组,试验组骨密度显著更高(P <0.05)。结论:应用补肾活血汤治疗桡骨远端骨折后期并发Sudeck骨萎缩患者效果显著,可明显改善患者的临床症状和腕关节功能,提高骨密度,值得推广。

皮下注射密盖息加局部神经阻滞治疗8例Sudeck骨萎缩

目的探索Sudeck骨萎缩治疗的有效方法。方法用利多卡因在患病关节作局部环行阻滞封闭,并同时皮下注射密盖息。结果本组8例均于上述治疗后1d后疼痛有所减轻,3～4d后肿胀明显消退,4周1疗程后复查,X线光摄片检查示骨质疏松较前有所改善。一定疗程后治愈为100%。结论用此方法治疗Sudeck骨萎缩效果良好,是临床上治疗的理想方法,值得推广应用。

Non-pharmacological intervention for posterior cortical atrophy

Posterior cortical atrophy(PCA) is a rare neurodegene-rative condition characterized by progressive visual-perceptual deficits. Although the neurocognitive profile of PCA is a growing and relatively well-established field, non-pharmacological care remains understudied and to be widely established in clinical practice. In the present work we review the available literature on non-pharmacological approaches for PCA, such as cognitive rehabilitation including individual cognitive exercises and compensatory techniques to improve autonomy in daily life, and psycho-education aiming to inform people with PCA about the nature of their visual deficits and limits of cognitive rehabilitation. The reviewed studies represented a total of 7 patients. There is a scarcity of the number of studies, and mostly consisting of case studies. Results suggest non-pharmacological intervention to be a potentially beneficial approach for the partial compensation of deficits, improvement of daily functionality and improvement of quality of life. Clinical implications and future directions are also highlighted for the advancement of the field, in order to clarify the possible role of non-pharmacological interventions, and its extent, in PCA.

Muscular atrophy and weakness in the lower extremities in Behçet’s disease: A case report and review of literature

BACKGROUND In Behçet’s disease(BD),very few cases of muscular involvement have been reported previously.The natural history and therapeutic protocol for muscular involvement in BD are obscure due to the low incidence of peripheral neuropathy or myopathy in BD.The purpose of our study was to report a rare case of BD with chronic,focal forms of neuromyopathy and review the relevant literature.CASE SUMMARY We herein report the case of a 54-year-old man who presented with progressive muscular atrophy and weakness of both thighs 2 years after the presentation of the cardinal symptoms of BD.The past medical history,electrophysiological study,neurological examination,blood tests,magnetic resonance imaging study,and histological exam were performed for the differential diagnosis.Relevant literature on muscular involvement in BD was reviewed.Neurological examination revealed that muscular involvement was predominantly localized in the proximal parts of the lower extremities.Heterogeneous enhancement of several thigh muscles was observed on magnetic resonance imaging,which corresponded with the clinical manifestations.Histological study of one of the enhanced muscles showed denervation atrophy of the muscle with superimposed myopathic changes,while electrophysiological studies only suggested denervation.CONCLUSION To our knowledge,this is the first case of neurogenic muscular atrophy with a specific set of clinical,radiological,electrophysiological,and histological findings reported in BD.

Mavridis' atrophy in Parkinson's disease-five years later: Future perspectives

Mavridis' atrophy(MA) is called the human nucleus accumbens(NA) atrophy in Parkinson's disease(PD).MA begins in early-stage PD patients and is correlated with psychiatric symptoms that occur in PD, mainly apathy and impulsive behavior. It is also associated with cognitive PD symptoms. Purpose of this editorial was to discuss the future perspectives of MA as apathological and imaging finding. MA is obviously part of the degeneration of the dopaminergic nigrostriatal system that occurs in PD and this also explains the fact that MA precedes clinical phenotype. But does the human NA follow the same pattern of degeneration? It would be quite interesting to have a post-mortem pathological study focused on the NA of parkinsonic individuals. Further questions that remain to be answered are whether all parkinsonics suffer MA and whether this phenomenon is also associated with motor PD symptoms. MA as an imaging finding could be a risk factor for the expression and/or severity of specific PD symptoms. It has therefore to be tested whether the presence of MA is related, for example, with the expression and/or severity of motor PD symptoms and whether the severity of MA affects the severity of specific psychiatric symptoms(apathy, compulsive behavior) of parkinsonic individuals. Such clinical studies, that could provide answers to these vital questions, can be easily preformed given the high frequency of PD in modern populations. Future research efforts are mandatory to enrich our knowledge of MA, namely its underlying mechanisms, its pathological features and its clinical consequences.

Regional patterns of atrophy on MRI in Alzheimer’s disease: Neuropsychological features and progression rates in the ADNI cohort

Background: Discrete clinical and pathological subtypes of Alzheimer’s disease (AD) with variable presentations and rates of progression are well known. These subtypes may have specific patterns of regional brain atrophy, which are identifiable on MRI scans. Methods: To examine distinct regions which had distinct underlying patterns of cortical atrophy, factor analytic techniques applied to structural MRI volumetric data from cognitively normal (CN) (n = 202), amnestic mild cognitive impairment (aMCI) (n = 333) or mild AD (n = 146) subjects, in the Alzheimer’s Disease Neuroimaging Initiative (ADNI) database was applied. This revealed the existence of two neocortical (NeoC-1 and NeoC-2), and a limbic cluster of atrophic brain regions. The frequency and clinical correlates of these regional patterns of atrophy were evaluated among the three diagnostic groups, and the rates of progression from aMCI to AD, over 24 months were evaluated. Results: Discernable patterns of regional atrophy were observed in about 29% of CN, 55% of aMCI and 83% of AD subjects. Heterogeneity in clinical presentation and APOE ε4 frequency were associated with regional patterns of atrophy on MRI scans. The most rapid progression rates to dementia among aMCI subjects (n = 224), over a 24-month period, were in those with NeoC-1 regional impairment (68.2%), followed by the Limbic regional impairment (48.8%). The same pattern of results was observed when only aMCI amyloid positive subjects were examined. Conclusions: The neuroimaging results closely parallel findings described recently among AD patients with the hippocampal sparing and limbic subtypes of AD neuropathology at autopsy. We conclude that NeoC-1, Limbic and other patterns of MRI atrophy may be useful markers for predicting the rate of progression of aMCI to AD and could have utility selecting individuals at higher risk for progression in clinical trials.

Hippocampus Gray Matter Atrophy Happens More Seriously in AD Female Patients

Hippocampus, an area of cortex that plays an important role in thinking, planning and remembering. In Alzheimer’s disease (AD), the hippocampus is one of the first areas of the brain to become shriveled and this leads to the memory less, damage in learning and declaration of emotional behaviors. In this paper, we investigate the effects of sex on hippocampus gray matter (HGM) atrophy in four groups of participants, namely, males with AD (M-AD, n = 34), age-matched normal male controls (M-NC, n = 34), females with AD (F-AD, n = 34), and age matched normal female controls (F-NC, n = 34) from ADNI dataset. In this regard, Analysis of variance (ANOVA) is employed to compare means of HGM differences among groups. The statistical results obtained by ANOVA show that the distribution of HGM atrophy is effected by sex. Also there was a significant diagnosis with higher severity in the F-AD compared to M-AD. The AD studies based on the sex may help to figure out the root of AD mechanisms and poten-tially can be used as an imaging marker for the studies of AD in the future.

Pathogenic roles of alpha-synuclein in Parkinson’s disease and multiple system atrophy

Alpha-synucleinopathies(α-synucleinopathies)are a diverse group of neurodegenerative diseases comprising Parkinson’s disease(PD),dementia with Lewy bodies(DLB),and multiple system atrophy(MSA).Although in all these diseases there exist abnormal accumulation of alpha-synuclein(α-syn)aggregates in nerve tissues,the pathological lesions formed byα-syn aggregates and their cellular locations are quite different.In PD and DLB,the hallmark pathological lesions are Lewy bodies(LBs)and Lewy neurites(LNs),which are localized in the neuronal somata and processes.In MSA,the characteristic pathologic structures are glial cytoplasmic inclusions,which are deposited in the cytoplasm of oligodendrocytes.The fact that PD and MSA have distinct pathologicalα-syn lesions suggest that different mechanisms play a role in the pathogenesis of the two diseases.In this review article,we compare the clinical manifestations and pathological features of PD and MSA,the two common synucleinopathies,and discuss the potential mechanisms for the formation ofα-syn aggregates and their pathologic roles in PD and MSA.

Characteristics and advantages of adeno-associated virus vector-mediated gene therapy for neurodegenerative diseases

Common neurodegenerative diseases of the central nervous system are characterized by progressive damage to the function of neurons, even leading to the permanent loss of function. Gene therapy via gene replacement or gene correction provides the potential for transformative therapies to delay or possibly stop further progression of the neurodegenerative disease in affected patients. Adeno-associated virus has been the vector of choice in recent clinical trials of therapies for neurodegenerative diseases due to its safety and efficiency in mediating gene transfer to the central nervous system. This review aims to discuss and summarize the progress and clinical applications of adeno-associated virus in neurodegenerative disease in central nervous system. Results from some clinical trials and successful cases of central neurodegenerative diseases deserve further study and exploration.

Dementia and Cognitive Impairment Reduction after Laser Transcatheter Treatment of Alzheimer’s Disease

Reduced cerebral perfusion and microcirculation are found among AD causes, which should be considered in the development of new treatments for the disease. 165 patients with AD were examined. The examination plan included clinical assessment of dementia severity (CDR), cognitive function assessment (MMSE), laboratory examination, cerebral scintigraphy (SG), rheoencephalography (REG), cerebral CT and MRI, morphometric AD stages assessment (TDR) and cerebral multi-gated angiography (MUGA). 89 patients aged 34 - 79 (average age 67) were selected for the treatment: 31 (34.83%) male, 58 (65.17%) female patients. According to their AD stage, the patients were divided into: TDR-0 (preclinical stage)—10 (11.24%) patients, TDR-1 (early stage with mild dementia, mild cognitive impairment)—28 (31.46%) patients, TDR-2 (medium stage with moderate dementia, cognitive impairment sufficiently persistent)—34 (38.20%) patients, TDR-3 (late stage with sufficiently severe dementia and cognitive impairment)—17 (19.10%) patients. Test Group—46 (51.68%) patients—had transcatheter treatment with low-energy lasers. Control Group—43 (48.31%)—had conservative treatment with Memantin and Rivastigmine. The Test Group had cerebral microcirculation improvement leading to permanent dementia reduction and cognitive recovery which allowed transferring the patients to a lighter TDR group or withdrawing them from the scale. Control Group patients with earlier AD stages (TDR-0, TDR-1, TDR-2) obtained stabilization for a period of 6 months-3 years, with subsequent growth of dementia and cognitive impairment;patients with late AD stage (TDR-3) showed further increase of cognitive impairment and dementia. Transcatheter treatment allows reducing the effects of dyscirculatory angiopathy of Alzheimer’s type (DAAT) improving cerebral microcirculation and metabolism, which leads to permanent dementia regression and cognitive impairment reduction. These data show that AD treatment should be comprehensive and aimed at both the recovery of cerebral microcirculation and blood supply and the normalization of amyloid beta metabolism in the cerebral tissue.

Ursolic acid induces neural regeneration after sciatic nerve injury

In this study, we aimed to explore the role of ursolic acid in the neural regeneration of the injured sciatic nerve. BALB/c mice were used to establish models of sciatic nerve injury through unilateral sciatic nerve complete transection and microscopic anastomosis at 0.5 cm below the ischial tuber-osity. The successful y generated model mice were treated with 10, 5, or 2.5 mg/kg ursolic acid via intraperitoneal injection. Enzyme-linked immunosorbent assay results showed that serum S100 protein expression level gradual y increased at 1-4 weeks after sciatic nerve injury, and significantly decreased at 8 weeks. As such, ursolic acid has the capacity to significantly increase S100 protein expression levels. Real-time quantitative PCR showed that S100 mRNA expression in the L 4-6 segments on the injury side was increased after ursolic acid treatment. In addition, the muscular mass index in the soleus muscle was also increased in mice treated with ursolic acid. Toluidine blue staining revealed that the quantity and average diameter of myelinated nerve fibers in the injured sciatic nerve were significantly increased after treatment with ursolic acid. 10 and 5 mg/kg of ursolic acid produced stronger effects than 2.5 mg/kg of ursolic acid. Our findings indicate that ursolic acid can dose-dependently increase S100 expression and promote neural regeneration in BALB/c mice fol owing sciatic nerve injury.

Endovascular Application of Low-Energy Laser in the Treatment of Dyscirculatory Angiopathy of Alzheimer’s Type

Purpose: We propose an analysis of dyscirculatory angiopathy of Alzheimer’s type (DAAT) endovascular treatment method based on transcatheter revascularization and recovery of collateral and microvascular bed of the brain by means of low-energy transluminal laser irradiation as well as its comparison with traditional Alzheimer’s disease (AD) treatment methods. Methods: The research involved 81 patients aged 34 - 79 (average age 67). 46 (46.8%) patients were treated using endovascular method—Test Group. 35 (43.2%) patients were given conventional treatment—Control Group. Patients were subdivided: Group (CDR-0): 9 (11.1%), pre-clinical stage or increased AD risk;Group (CDR-1): 24 (29.6%), mild dementia and cognitive impairment;Group (CDR-2): 31 (38.3%), moderate dementia and persistent cognitive impairment;Group (CDR-3): 17 (21.0%), severe dementia and cognitive impairment. Research plan included CT or MRI with subsequent temporal lobes volume calculation, brain scintigraphy (SG), rheoencephalography (REG), and cerebral MUGA. There were indications and contraindications for treatment in Test Group. In Group CDR-0, endovascular intervention was prophylactic, against the background of increasing memory impairment;in Groups CDR-1, CDR-2, CDR-3, it was conducted in 1 to 12 years period from AD symptoms appear-ance. Conservative treatment with Memantin and Rivastigmine was carried out in Control Group. Results: In Test Group, positive outcome accompanied by prolonged dementia decline, cognitive impairment decrease, and patients’ transition to CDR group of an earlier stage, was obtained in all cases. In Control Group, patients’ temporary stabilization in their own CDR group was achieved. Conclusions: Endovascular treatment of patients with AD different stages can not only reduce DAAT phenomena but can also cause AD regression possibly accompanied by regenerative processes in the cerebral tissue. Conservative treatment only allows stabilizing the patient’s condition for a while.

Antibody-based immunotherapies for Parkinsonian syndromes

What is the rationale for immunotherapies in Parkinsonian syndromes (PS)? PS are neurodegenerative diseases which are clinically characterized by a hypokinetic phenotype in combination with additional motor and non-motor symptoms. One major pathological hallmark of all PS consists of a non-physiological detrimental accumulation of protein aggregates which appear intracellularly in neurons and glial cells but also in the extracellular space (Wong and Krainc, 2017). Depending on the pathogenic protein, PS can be divided into synucleinopathies, characterized by aggregation of the protein alpha-Synuclein (aSyn), and tauopathies, characterized by aggregation of the protein Tau (Levin et al., 2016;Poewe et al., 2017). Clinical syndromes of synucleinopathies include Parkinson’s disease (PD), multiple system atrophy (MSA) and dementia with Lewy bodies, and tauopathies include progressive supranuclear palsy (PSP) and corticobasal degeneration.

Dyscirculatory Angiopathy of Alzheimer's Type

Purpose: We assess the significance of dyscirculatory angiopathy of Alzheimer’s type (DAAT) in identify- ing the predisposition to the development and diagnosis of Alzheimer’s disease (AD) different stages. Meth- ods: 108 patients took part in the research:1) 49 aged 34-79 suffering from AD or running an increased risk of its development (those not diagnosed with AD but having growing memory disorders without any mani- festations of dementia or specific cognitive impairments, and having 2 or more immediate relatives with AD) - Test Group;2) 59 aged 28-78 suffering from different types of brain lesions accompanied by dementia but not suffering from AD or corresponding to their age norm - Control Group. All the patients underwent MRI, CT with subsequent calculation of the temporal lobes atrophy degree, brain scintigraphy (SG), rheoencepha- lography (REG), and MUGA. Results: Characteristic features of patients with an increased risk of AD as well as at its various stages are: 1) Temporal lobes and hippocampus atrophy ranging from 4% among those with an increased risk of AD to 62% among those at its advanced stages;2) DAAT manifestations: reduction of the capillary bed in the temporal and frontoparietal regions with the development of multiple arterioven- ous shunts of the same localization and correspondent early venous discharge accompanied by venous stasis on the border of the frontal and parietal region;3) DAAT phenomena equally develop both among those with an increased risk of developing AD and those at various AD stages. Similar changes are not observed among Control Group patients with other brain lesions, regardless of the severity of dementia, as well as among practically healthy people of the corresponding age group. Conclusion: Timely identification of the above- mentioned changes can reveal a predisposition to AD development long before its initial manifestations, and it allows differentiating AD from other diseases attended by dementia. In both cases, timely diagnosis allows beginning timely treatment and thus achieving more stable results.