Research progress of diabetes mellitus complicated with chronic inflammatory demyelinating polyneuropathy / 天津医药

In recent years,the results of epidemiological research findings on chronic inflammatory demyelinating polyneuropathy(CIDP)in patients with diabetes mellitus(DM)are quite different,and its correlation is still controversial.Nevertheless,due to the high incidence and treatability of CIDP in DM patients,accurate diagnosis and differentiation of DM-CIDP are of great value for guiding clinical drug use.In terms of clinical manifestations,it can be distinguished by age of onset,disease duration,blood glucose control level and main symptom characteristics.Supplementary diagnostic methods frequently involve evaluating cerebrospinal fluid protein levels,demyelination patterns in electrophysiological tests and neuroimaging studies.Additionally,neuropathological examination and specific serum antibodies can provide more diagnostic support for distinguishing DM-CIDP.Furthermore,a positive response to immunomodulatory and/or immunosuppressive therapies supports the diagnosis of DM-CIDP.By comprehensively evaluating clinical manifestations,electrophysiological characteristics,laboratory tests,neuropathology,imaging,and treatment responses,the accuracy of the differential diagnosis for DM-CIDP can be improved.

Research advance on the pathogenesis of autosomal recessive spastic ataxia of Charlevoix-Saguenay / 中华医学遗传学杂志

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare and early-onset neurodegenerative disease caused by variants of the SACS gene which maps to chromosome 13q11 and encodes sacsin protein. Sacsin is highly expressed in large motor neurons, in particular cerebellar Purkinje cells. This article has provided a review for the structure and function of sacsin protein and the mechanisms underlying abnormalities of sacsin in ARSACS disease.

Drug-induced peripheral neuropathy / 中华神经科杂志

There are many causes of peripheral neuropathy, one of which is that caused by various therapeutic drugs, namely drug-induced peripheral neuropathy (DIPN). With the increasing number of cancer patients, chemotherapy-induced peripheral neuropathy (CIPN) is prominent. Most of DIPN showed chronic, sensory and axonal polyneuropathy, and numbness and neuropathic pain were the main symptoms. The medical history of drug exposure and related risk factors are the key to clinical diagnosis. DIPN caused by non-chemotherapy drugs must be stopped in time to prevent further dysfunction. For CIPN, no agents are recommended for the prevention; the appropriateness of dose delaying and reduction, substitutions or stopping chemotherapy should be evaluated; duloxetine is the only agent that has appropriate evidence to support its use, whereas the amount of benefit is limited. It is urgent to perform the multidisciplinary randomized clinical trial for the treatment of CIPN.

Clinical Features and Differences of Miller Fisher Syndrome in Southern China: Retrospective Analysis of 72 Patients in 13 Provinces of Southern China

Background@#and Purpose We aimed to determine the clinical features of Miller Fisher syndrome (MFS) in southern China and compare them with those presenting in other countries. @*Methods@#We collected the medical records of patients diagnosed with MFS during 2013–2016.We analyzed the age, sex, onset season, precursor events, clinical symptoms and signs, findings of nerve conduction studies (NCS), cerebrospinal fluid (CSF), therapeutic remedies, nadir time, and length of hospital stay of patients with MFS in southern China. We concurrently compared the differences between urban and rural areas and between patients with incomplete ophthalmoplegia (IO) and complete ophthalmoplegia (CO). @*Results@#The study enrolled 72 patients: 36 from rural areas and 36 from urban areas, and 50 males and 22 females. The mean age at onset was 47.72 years, and 30 (41.7%) and 21 (29.2%) patients developed MFS in spring and winter, respectively. The typical triad of ophthalmoplegia, ataxia, and areflexia was observed in 50 (69.4%) patients. A history of upper respiratory tract infection 1 week before onset was found in 52.8% of the patients, while 5.6% experienced gastrointestinal infections and 48 (73.8%) exhibited albuminocytological dissociation in the CSF study. Only 26 (36.1%) patients presented abnormalities in NCS. Moreover, restricted outward eyeball movement presented in 83.5% of the patients with classic MFS and acute ophthalmoplegia, and bilateral symmetrical ophthalmoplegia presented in 64.2%. With the exception of the higher proportion of NCS abnormalities in urban areas (47.2% vs. 25.0%), urban and rural differences were insignificant regarding sex ratio, age at onset, high-incidence season, precursor events, disease characteristics, and albuminocytological dissociation in the CSF. Furthermore, patients with CO were older than those with IO (64.53±7.69 vs. 43.19±14.40 years [mean±standard deviation], p<0.001). @*Conclusions@#The patients with MFS were mostly male and middle-aged, and most presented in winter and (especially) spring. More than half of the patients had clear precursor events, most of which were classic MFS with the typical triad. More than 70% of the patients presented albuminocytological dissociation in the CSF. NCS abnormalities were uncommon in MFS. The age at onset was lower in patients with IO than in patients with CO; bilateral symmetrical extraocular muscle paralysis was the most common symptom, and the external rectus was the most frequently involved muscle.

Clinical characteristics of Guillain-Barré syndrome combined with hyponatremia in Southern China and its risk factors for prognosis: a multi-center retrospective analysis / 中华神经医学杂志

Objective:To investigate the clinical characteristics of Guillain-Barré syndrome (GBS) combined with hyponatremia in Southern China and its risk factors for prognosis.Methods:The retrospective cohort study involved patients who met the diagnostic criteria of GBS from 18 upper first-class hospitals of 6 provinces/cities in southern China (south of Huaihe River) from January 1, 2013 to September 30, 2016. The clinical data of these patients were collected. According to serum sodium levels, they were divided into hyponatremia group (serum sodium concentration<135 mmol/L) and normal serum sodium group (serum sodium concentrations≥135 mmol/L). Based on Medical Research Coucil sum scores at nadir, these patients were divided into mild GBS group (>40), moderate GBS group (30-40), and severe GBS group (<30). Furthermore, according to the Hughes GBS disability scale (H-GBS-DS) scores at discharge, these GBS patients with hyponatremia were divided into favorable prognosis group (H-GBS-DS<3) and poor prognosis group (H-GBS-DS≥3). The incidence of hyponatremia in patients from the mild GBS group, moderate GBS group, and severe GBS group were compared. Multivariate Logistic regression analysis was performed to determine the clinical risk factors for hyponatremia in GBS patients. The clinical data of hyponatremia patients from favorable prognosis group and poor prognosis group were compared; multivariate Logistic regression analysis was used to determine the risk factors for poor prognosis in GBS patients with hyponatremia.Results:(1) Among the 570 patients, 354 had mild GBS, 94 had moderate GBS, and 122 had severe GBS; 134 GBS patients were combined with hyponatremia, 436 GBS patients had normal serum sodium. The hyponatremia incidence in mild, moderate and severe GBS groups increased successively, ( P<0.05). Multivariate Logistic regression analysis showed that facial paralysis ( OR=1.979, 95%CI: 1.172-3.342, P=0.011), respiratory muscle paralysis ( OR=3.218, 95%CI: 1.611-6.428, P=0.001), secondary pulmonary infection ( OR=4.822, 95%CI: 2.835-8.201, P=0.000), severe GBS ( OR=2.611, 95%CI: 1.444-4.721, P=0.001) and length of hospital stay ( OR=1.029, 95%CI: 1.009-1.050, P=0.004) were risk factors for hyponatremia in GBS patients. (2) Among 134 GBS patients with hyponatremia, 80 had poor prognosis and 54 had favorable prognosis. As compared with the favorable group, the poor prognosis group had significantly lower proportion of patients with extraocular muscle paralysis, statistically higher proportions of patients with respiratory muscle paralysis and secondary pulmonary infection, significantly different severities of GBS, signficantly higher proportion of patients accepted intravenous immunoglobulin (IVIG) and hormone treatments, statistically longer length of hospital stay ( P<0.05). Respiratory muscle paralysis ( OR=25.590, 95%CI: 9.433-69.423, P=0.000), moderate GBS ( OR=17.030, 95%CI: 8.441-34.361, P=0.000), and severe GBS ( OR=51.042, 95%CI: 24.596-105.926, P=0.000) were independent risk factors for poor short-term prognosis of GBS patients with hyponatremia. Conclusions:Severe GBS patients with facial paralysis, respiratory muscle palsy, secondary pulmonary infection, and long hospital stay trend to have hyponatremia. Hyponatremia patients with respiratory muscle paralysis and moderate/severe GBS have poor short-term prognosis.

Recent advance in detection of phrenic nerve conduction in diagnosis of amyotrophic lateral sclerosis / 中华神经医学杂志

Respiratory failure is the main causes of death in patients with amyotrophic lateral sclerosis (ALS). Detection of phrenic nerve conduction is an important method for objective assessment of respiratory function, which can make up for the limitations of pulmonary function test (PFT), and has a certain clinical value in improving the quality of life of ALS patients by early and effective measures. This article focuses on the neurophysiology and edetection methods of phrenic nerve conduction and its clinical application in ALS to improve the understanding of clinicians and better serve clinic.

The experience of diagnosis and treatment of neurological diseases combined with COVID-19: analysis of five cases / 中华神经科杂志

Objective:To investigate the clinical characteristics, diagnosis and treatment of neurological diseases combined with COVID-19.Methods:The data of five patients of neurological diseases combined with COVID-19 (including two suspected cases) in the Department of Neurology, Renmin Hospital of Wuhan University from January 1, 2020 to February 24, 2020 were retrospectively collected. The epidemiology, clinical manifestations, laboratory and radiologic characteristics, diagnosis, treatment and outcomes of these cases were described and analyzed.Results:Among the five patients, three were confirmed cases and two were suspected cases with a history of indirect contact with confirmed cases of COVID-19. The age of these patients was (76.4±9.3) years. Neurological diseases included cerebral infarction in two cases, cerebral hemorrhage, Guillain-Barre syndrome and Alzheimer′s disease each in one case. There were one acute case and one chronic case, and three patients were in the sequelae stage of acute diseases. One patient had an open airway as a result of Guillain-Barre syndrome. There were four cases of limb paralysis, half of whom were hemiplegic and quadriplegic respectively. The initial symptom was fever in all cases, and they showed a decrease in lymphocyte count. Four patients with chest computed tomographic scans showed bilateral lung lesions, of whom two cases showed multiple lung patches and ground glass shadows. Tests for 2019 novel coronavirus nucleic acid were positive in three patients and negative in two. The time from onset of COVID-19 to diagnosis was (6.6±5.6) d. As of February 24, two patients died and the remaining were still hospitalized.Conclusions:Neurological diseases, especially in elderly patients with limb paralysis, open airways and stroke, can be complicated by COVID-19, of which the prognosis is poor. Neurologists need to identify this situation early and pay attention to strengthening protection.

Status quo of the treatment of spinocerebellar ataxia towards its pathogenesis / 中华医学遗传学杂志

Spinocerebellar ataxia (SCA) is a group of autosomal dominant hereditary diseases. Based on their inheritance pattern, they can be divided into SCAs caused by expansion of microsatellite repeats or point mutations. Although SCAs may be diagnosed based on their clinical characteristics and results of genetic testing, their treatment still remains as a challenge. So far no drug has been approved by the US Food and Drug Administration or the European Medicines Agency. Strict preclinical trials are critical for the development of disease-modifying drugs.

Use of Magnetic Resonance Neurography for Evaluating the Distribution and Patterns of Chronic Inflammatory Demyelinating Polyneuropathy

OBJECTIVE: To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency.MATERIALS AND METHODS: Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2- weighted turbo spin-echo sequences were performed for neurography of the brachial and lumbosacral (LS) plexus and cauda equina, respectively. Clinical data and scores of the inflammatory Rasch-built overall disability scale (I-RODS) in CIDP were obtained.RESULTS: The bilateral extracranial vagus (n = 11), trigeminal (n = 12), and intercostal nerves (n = 10) were hypertrophic. Plexus hypertrophies were observed in the brachial plexus of 19 patients (61.3%) and in the LS plexus of 25 patients (80.6%). Patterns of hypertrophy included uniform hypertrophy (17 [54.8%] brachial plexuses and 21 [67.7%] LS plexuses), and multifocal fusiform hypertrophy (2 [6.5%] brachial plexuses and 4 [12.9%] LS plexuses) was present. Enlarged and/or contrast-enhanced cauda equina was found in 3 (9.7%) and 13 (41.9%) patients, respectively. Diameters of the brachial and LS nerve roots were significantly larger in CIDP than in controls (p < 0.001). The largest AUC was obtained for the L5 nerve. There were no significant differences in the course duration, I-RODS score, or diameter between patients with and without hypertrophy.CONCLUSION: MRN is useful for the assessment of distribution and characteristics of the peripheral nerves in CIDP. Compared to other regions, LS plexus neurography is more sensitive for CIDP.

Recent advance in pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经医学杂志

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a motor-sensory demyelinating neuropathy mediated by immunity. The pathogenesis is still unclear, and the clinical manifestations are complex. Its diagnoses are difficult, and the treatment effect is not good. At present, most studies consider it to be an autoimmune disease, but the pathogenic antigen and its target to the immune damage mechanism of the disease are still unclear. In this paper, we review the literature on the pathogenesis of CIDP in recent years, and introduce the specific mechanisms which cause CIDP from cellular and humoral immunity.

Short-term prognostic factors for Guillain-Barré syndrome in children / 中华神经医学杂志

Objective:To explore the short-term prognostic factors for Guillain-Barré syndrome (GBS) in children.Methods:The clinical data of children with GBS from 24 hospitals in 10 provinces/municipalities/autonomous regions in southern China (south of Huaihe River) from January 1, 2013 to September 30, 2016 were retrospectively analyzed. The factors affecting the short-term prognoses of children were explored.Results:In these 78 children (50 males and 28 females), the average age was 9.53±5.44 years, and 19 were under 5 years old. Fifty children had history of prodromal events; 28 children had cranial nerve involvement, and 22 had autonomic nerve involvement. Five children needed assisted respiration, and one died during hospitalization. There was no statistically significant difference in percentage of children having poor short-term prognosis (scores of Hughes GBS disability scale≥3 at discharge) between children with different ages, children having different days from onset to admission, children with different clinical classifications or electrophysiological classifications, children with different treatment plans, children having presence or absence of prodromal events, children having presence or absence of cranial nerve involvement ( P>0.05). The proportion of children having poor short-term prognosis in children with autonomic nerve involvement was significantly higher than that of children without autonomic nerve involvement (31.8% vs. 10.7%, P<0.05). Conclusion:The short-term prognosis of children with autonomic nerve involvement is poor.

Safety and efficacy of mechanical thrombectomy in minor stroke with large vessel occlusion / 中华神经医学杂志

Objective:To explore the efficacy and safety of mechanical thrombectomy (MT) in patients with minor stroke with large vessel occlusion (LVO).Methods:Twenty-three patients with minor stroke with LVO, admitted to our hospital from January 2017 to July 2019, were consecutively collected in our study; patients with contraindications of intravenous thrombolysis should be treated with direct thrombectomy, and the left were given bridging therapy (intravenous thrombolysis combined with MT). NIHSS scores were used to assess the degrees of neurological impairment at admission, and 12 h and 7 d after treatment. Vascular recanalization was assessed by modified cerebral infarction thrombolysis (mTICI) grading, with grading 2B-3 defined as successful recanalization. The prognoses 90 d after treatment were assessed by modified Rankin scale (mRS), and mRS scores≤2 was classified as having good prognosis. Safety indicators included symptomatic intracranial hemorrhage, incidence of complications, and mortality 90 d after treatment.Results:Twenty-two patients had successfully recanalization; 19 patients had mTICI grading 3 and 3 patients had grading 2B. The NIHSS scores were 3 (2, 5) at admission, 2 (2, 3) 12 h after treatment, and 2 (1, 2) 7 d after treatment, with significant difference ( χ2=14.028, P=0.001); NIHSS scores 12 h and 7 d after treatment were significantly lower than those at admission ( P<0.05). Sixteen patients (69.6%) enjoyed good prognosis and 7 patients (30.4%) had poor prognosis. In terms of safety, two patients had symptomatic intracranial hemorrhage,10 had systemic complications, and one died during 90-d of follow-up. Conclusion:MT is effective and safe in minor stroke patients with LVO.

Efficacy analysis of bridging therapy and direct endovascular therapy in ischemic stroke patients with large vessel occlusion within 4.5 h of onset / 中华神经医学杂志

Objective:To explore the efficacy of bridging therapy (BT) and direct endovascular therapy (DEVT) in patients with acute ischemic stroke induced by large vessel occlusion (LVO-AIS) within 4.5 h of onset.Methods:The clinical data of 154 patients with LVO-AIS within 4.5 h of onset, admitted to our hospital from January 2017 to July 2019, were retrospectively collected. Among them, 88 patients were hospitalized within 3 h of onset (54 accepted BT and 34 accepted DEVT); 66 patients were hospitalized within 3-4.5 h of onset (39 accepted BT and 27 accepted DEVT). The differences in clinical data and treatment efficacy between patients from the BT group and DEVT group that were hospitalized within 3 h of onset and within 3-4.5 h of onset, respectively, were compared. Multivariate Logistic regression was used to analyze the independent protective factors for favorable outcome 90 d after treatment in patients within 3.0-4.5 h of onset and within 3 h of onset, respectively.Results:(1) In patients within 3 h of onset: as compared with the DEVT group, the BT group had significantly higher improvement rate of neurological function at 24 h after treatment (41.2% vs. 70.4%) and higher percentage of patients enjoying favorable outcome 90 d after treatment (44.1% vs. 66.7%, P<0.05); multivariate Logistic regression analysis showed that BT was an independent protective factor for favorable outcome 90 d after treatment in patients within 3 h of onset ( OR=4.644, 95%CI: 1.238-12.805, P=0.041). (2) In patients within 3-4.5 h of onset: as compared with the BT group, the DEVT group had significantly higher proportion of patients having time from onset to groin puncture≤4 h, and significantly higher proportion of patients with favorable outcome 90 d after treatment ( P<0.05); multivariate Logistic regression analysis showed that the time from onset to groin puncture≤4 h was an independent protective factor for favorable outcome 90 d after treatment in patients within 3-4.5 h of onset ( OR=5.724, 95%CI: 1.192-11.676, P=0.024). Conclusion:For LVO-AIS patients, BT is the first choice in patients hospitalized in the early time window; and BT should be performed within 4 h of onset to the greatest extent for patients hospitalized in the late time window; if time from onset to groin puncture is not within 4 h, DEVT should be the first choice.

Association between electromyography and magnetic resonance neurography in patients with typical chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

Objective@#To explore the association among clinical features, electromyography (EMG) and magnetic resonance neurography (MRN) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). @*Methods@#A cross-sectional survey was conducted to enroll consecutively typical CIDP patients in Renmin Hospital of Wuhan University from May 2017 to May 2019. The Hughes Disability Scale (HDS) was used to evaluate the illness severity of the patients. The electrodiagnostic parameters including motor conduction velocity (MCV), compound muscle action potential (CMAP), F-wave latency, sensory nerve conduction velocity (SCV) and sensory nerve action potential (SNAP) of upper and lower limbs were analyzed. The patients whose response waveform can be elicited in all nerves were defined as group A, and those without response in one or more nerves as group B. MRN quantitative technique was used to calculate the cross-sectional area of nerves roots (nr-CSA) of brachial plexus and lumbosacral plexus. The linear regression method was used to analyze the correlation among clinical features, EMG and nr-CSA. @*Results@#A total of 32 patients with typical CIDP met the criteria, 75% (24/32) of whom were males. There were 16 patients in the mild group (group A) and 16 in the severe one (group B). The abnormal rate of F-wave latency was the highest. Cerebrospinal fluid (CSF) protein, HDS score were correlated significantly with the nr-CSA of brachial plexus and lumbosacral plexus in the two groups (group A: CSF protein and brachial plexus nr-CSA: r=0.498, P=0.004; CSF protein and lumbosacral plexus nr-CSA: r=0.479, P=0.007; HDS score and brachial plexus nr-CSA: r=0.650, P=0.000; HDS score and lumbosacral plexus nr-CSA: r=0.625, P=0.000. group B: CSF protein and brachial plexus nr-CSA: r=0.497, P=0.049; CSF protein and lumbosacral plexus nr-CSA: r=0.503, P=0.047; HDS score and brachial plexus nr-CSA: r=0.605, P=0.001; HDS score and lumbosacral plexus nr-CSA: r=0.648, P=0.000). MCV of median nerve and ulnar nerve was negatively correlated with nr-CSA of brachial plexus in the two groups (group A: MCV of median nerve and nr-CSA of brachial plexus: r=-0.309, P=0.019; MCV of ulnar nerve and nr-CSA of brachial plexus: r=-0.286, P=0.027. group B: MCV of median nerve and nr-CSA of brachial plexus: r=-0.660, P=0.000; MCV of ulnar nerve and nr-CSA of brachial plexus: r=-0.581, P=0.001). The F-wave latencies of median and ulnar nerves were positively correlated with nr-CSA of brachial plexus, and the CMAP amplitude of tibial nerve and SNAP amplitude of sural nerve were positively correlated with nr-CSA of lumbosacral plexus in group B. @*Conclusions@#Male patients with CIDP are predominant. The higher the nr-CSA in brachial plexus and lumbosacral plexus, the higher the CSF protein and disability score, and the larger the nr-CSA in brachial plexus, the slower the MCV in the median and ulnar nerve. For group B patients with more severe nerve injury, the larger nr-CSA of brachial plexus was, the longer F-wave latency of median and ulnar nerve was, and the larger nr-CSA of lumbosacral plexus was, the lower CMAP amplitude of tibial nerve and SNAP amplitude of sural nerve were. As a non-invasive test, MRN can be used to assist in the diagnosis of CIDP and to assess the severity of the disease.

Screening for abnormal angle of trunk rotation among adolescents in Yichang city / 中华全科医师杂志

Total 3 483 students in 4 to 9 grade from primary and middle school in Xiling District of Yichang city were selected by stratified cluster random sampling method between September and October 2016 to participate in screening for abnormal angle of trunk rotation(ATR),including 1 797 boys and 1 686 girls aged 9-15 years.The ATR was measured with Scoliometer and the ATR >5°was defined as abnormal.The overall abnormal ATR rate was 7.60%(265/3 483), that in boys was 4.73%(85/1797)and in girls was 10.68%(180/1 686)(χ2=43.75,P<0.05).The abnormal ATR rate in age group 13-15 was higher than that in age group 9-12[10.62%(159/1 496)vs.5.33%(106/1 987),χ2=36.92,P<0.01].Students with low BMI had higher abnormal ATR rate than that with normal or higher BMI(χ2=30.00,P<0.01);and students with left handedness had lower abnormal ATR rate than that with right handedness or double handedness(χ2=6.59,P=0.04).The abnormal ATR rate was not associated with dietary habits or sports in the daily life of students.The results show that gender,age,BMI and handedness affect abnormal rate of ATR.

Clinical features and validation of Brighton criteria in Guillain-Barré syndrome in southern China:retrospective analysis of 1358 hospitalized patients in four years / 中华神经科杂志

Objective To analyze the clinical features and validation of Brighton criteria in Guillain-Barré syndrome (GBS) patients from southern China.Methods The clinical data of hospitalized GBS patients from 69 hospitals of 14 provinces/cities in southern China,the area south of the Huaihe River,between 1 January 2013 and 30 September 2016,were collected and analyzed retrospectively,and patients were classified according to the Brighton criteria of case definition,ranging from a highest (defined as level one) to a lowest (level four) level of diagnostic certainty.Results A total of 1 358 GBS patients were collected,including 51 cases with cranial nerve variants,157 with Miler-Fisher syndrome and 1 150 with classic GBS characterized by flaccid weakness of limbs.Among 1 150 cases of classic GBS,49.57％ (570/1 150) patients had antecedent events,with respiratory infection predominated (71.23％,406/570);83.74％ (963/1 150) presented limb weakness at onset,99.21％ (1 124/1 133) reached the peak within four weeks,with a score of 3.15 ± 1.16 for Hughes Disability Scale;99.56％ (1 128/1 133)developed bilateral weakness and 95.39％ (1 097/1 150) manifested flexia or hyporeflexia;the cerebrospinal fluid showed albuminocytologic dissociation in 80.58％ (772/958) patients whose lumbar puncture was performed;demyelinating GBS accounted for 48.14％ (401/833) and axonal subtype 18.01％ (150/833) respectively in patients with findings of nerve conduction studies available.According to Brighton criteria,the patients were stratified as level one in 44.09％ (507/1 150),level two in 45.74％ (526/1 150),level three in 7.57％ (87/1 150) and level four in 2.61％ (30/1 150) of all the patients,and 69.55％ (507/729),28.67％ (209/729),0％ (0/729) and 1.78％ (13/729),respectively in the patients with complete data (n =729).Conclusions In southern China,demyelinating subtype of GBS is predominant,whereas the proportion of axonal subtype is remarkably lower than that in northern China.The Brighton criteria have a high sensitivity for the diagnosis of GBS in southern China,and examination of cerebrospinal fluid and electrodiagnostic studies are necessary for stratified diagnosis.

The impact of botulinum toxin on the anxiety and depression in patients with hemifacial spasm and blepharospasm / 中国神经精神疾病杂志

Objective To explore anxiety and depression status in patients with hemifacial spasm (HFS) and benign essential blepharospasm (BEB), and the change after botulinum toxin type A (BTX-A) therapy. Methods Ninety idiopathic HFS patients and 90 BEB patients. The anxiety and depression statud was evaluated by using SAS and SDS before and after the injection of BTX-A. Results Before treatment, the SAS and SDS standard scores of the HFS patients were 41.25 ±6.35and 42.25 ±7.57, respectively. The SAS scores were 40.17 ± 8.36 in the male and 43.56 ± 6.10in female(P=0.031). The SDS scores were 40.25 ± 6.46 in the male and 45.48 ± 7.31 in the female(P=0.008). After treatment, the SAS and SDS standard scores were 30.12 ± 4.35(P=0.000)and 30.58 ± 4.89(P=0.000)respectively. There was significant difference inbetween before and after treatment. In BEB patients, before treatment, The SAS standard scores were 58.90±10.61, 58.78±9.89. The SAS of the male and female patients scored 56.45 ± 8.75, 60.89 ± 9.11 (P=0.017), The SDS of the male and female patients scored 57.90 ± 7.93, 60.12 ± 8.35 (P=0.011)respectively. After treatment, the SAS standard score were 38.17 ± 3.67 (P=0.000), and the SDS standard score were 38.12 ± 4.15 (P=0.001)respectively. these two scores were significantly different between before and after treatment. Conclusion HFS and BEB patients, especially female patients are associated with anxiety and depression. BTX-A can improve the symptoms of anxiety and depression.

Recent advance in role of acid sensing ion channels in process of epilepsy / 中华神经医学杂志

Acid sensing ion channels (ASICs) are voltage-independent,proton-gated,cation-selective channels composed of 6 different subunits which are encoded by 4 genes.These channels are sensitive to extracellular protons and open when the pH value decreases.Recently,accumulating evidences have suggested that activation of ASICs is involved in many physiological and pathological conditions associated with tissue acidification,including synaptic transmission,inflammation,ischemia,pain and epileptic seizures.Therefore,targeting these channels emerges as a potential promising therapeutic strategy to combat these diseases.This review will focus on the structure and distribution of ASICs,with special emphasis on their changes in the process of epilepsy,the influence they exert on seizure severity,as well as the possible mechanisms by which they modulate this disease.

Recent advance in oligodendrocyte differentiation, development and myelin formation / 中华神经医学杂志

Myelin is multi-layer lipid membrane that surrounds and insulates axons,which is synthesized by oligodendrocyte (OL) in the central nervous system.OLs develop from neural stem cells.Researchers found that many transcription factors,such as Olig2,Soxl0,Nkx2.2,Oligl and Zfp2l8,play very important roles in the process of OL differentiation,development and maturation.Myelin formation is effected by multiple signaling pathways,including Wnt,PI3K,BMP-Smad signaling pathway.The process of OL differentiation,development and myelination is highly regulated.Here,we mainly review the recent advance in axon surface ligands,transcription factors,epigenetics and other relevant factors for myelination.

Acid sensing ion channel 1a involving in development of cerebella Purkinje cells / 中华神经医学杂志

Objective To investigate the mechanism of acid sensing ion channel (ASIC) 1a in the development of Purkinje cells in the cerebellum.Methods Newborn C57BL/6 mice were chosen and Purkinje cells were obtained from these mice by in vitro culture.Purkinje cells were divided into experimental group and control group,and shRNA-ASIC1a or shRNA-control sequence was used to construct recombinant plasmid lentivirus infections at the early and late stages of cell developments.The morphological structures of Purkinje cells were detected by immunofluorescent staining and the dendritic branches were counted at the early and late stages of cell developments.Western blotting was employed to detect the calcium binding protein D-28K,glial fibrillary acidic protein,Zic,parvalbumin and N-methyl-D-aspartate receptor (NMDAR) expressions.Real time (RT)-PCR was used to detect the expressions of endoplasmic reticulum stress related factor CCAAT/enhancer binding protein homologous protein CHOP and protein kinase R-like ER kinase (PERK).Results Immunofluorescence indicated that Purkinje cells showed obvious morphological differences between the experimental group and control group;the dendrite growth and development in the control group were significantly better than those in the experimental group (P＜0.05);the number of 2nd and 3nd stage dendritic branches of Purkinje cells in the control group was significantly larger than that in the experimental group (P＜0.05).Western blotting showed that D-28K expression in the Purkinje cells of experimental group was significantly decreased as compared with that in the control group at the early stage (P＜0.05);NMDAR expression in the Purkinje cells of experimental group was significantly increased as compared with that in the control group at the early and late stages (P＜0.05).RT-PCR results showed that CHOP and PERK expressions in the Purkinje cells of experimental group were significantly higher than those in the control group (P＜0.05).Conclusion ASIC1a has an important role in the development of cerebella Purkinje cells.