Advances in the diagnosis and treatment of dipeptidyl-peptidase-like protein 6 antibody- associated encephalitis / 中华神经科杂志

Dipeptidyl-peptidase-like protein 6 (DPPX) antibody-associated encephalitis, mediated by DPPX antibodies in serum and/or cerebrospinal fluid, is a novel autoimmune encephalitis. It presents with multifocal and diverse neurological disorders characterized by the triad of prodromal weight loss and/or gastrointestinal symptoms (diarrhea predominately), cognitive-mental dysfunction, and central nervous system hyperexcitability. Brain magnetic resonance imaging shows non-specific abnormalities. Immunotherapy can result in clinical improvements, but the disease is prone to relapse.

Autoimmune glial fibrillary acidic protein astrocytopathy / 中华神经科杂志

Autoimmune glial fibrillary acidic protein astrocytopathy is a curable autoimmune inflammatory disease of the central nervous system. Meninges, brain, spinal cord and optic nerve are mainly involved. Radial paraventricular enhancement and/or long segment lesions of spinal cord with central enhancement can be seen on magnetic resonance imaging. Brain biopsy shows perivascular inflammation with microglia activation. The disease is sensitive to steroid therapy. Glial fibrillary acidic protein antibody is considered as a specific biomarker of the disease.

Monitoring time of interictal epileptiform discharges by long-term video EEG in patients with epilepsy / 浙江大学学报·医学版

To optimize the monitoring time of interictal epileptiform discharges (IED) in patients with epilepsy by long-term video electroencephalogram (VEEG).The cumulative percentages of IED detected by VEEG in 346 epilepsy patients (349 times) with different purposes, different waking sleep states and different MRI findings were retrospectively analyzed. According to the purposes, there were 164 patients (165 times) for clarifying diagnosis, 124 patients (124 times) for preoperative evaluation and 58 patients (60 times) for adjustment of medications. According to MRI results, there were responsible lesions in 98 patients (98 times) and no responsible lesions in 173 patients (174 times).Among 346 patients (349 times), IED was detected within 24 h in 231 patients (times). The percentage of detection in patients with purpose of preoperative evaluation was higher than those with purpose of diagnosis and medication adjustment. The detection of LED was gradually increased in first 8 h with 59.0%, then stably in 24 h. 46.8% IED was recorded during sleep time, particularly in the second stage of sleep. The cumulative percentage of IED in patients with abnormal MRI findings was higher in all periods. It reached 83.7% within 8 h, and then tended to be stable.The study shows that LED should be monitored by VEEG at least 8 hours and should include the second stage of sleep in patients with epilepsy. Patients with refractory epilepsy and with abnormal lesions on MRI should record IED more frequently.

Effects of Antiepileptic Drugs on the Carotid Artery Intima-Media Thickness in Epileptic Patients

BACKGROUND AND PURPOSE: It has been reported that taking antiepileptic drugs (AEDs) may increase the risk of atherosclerosis. We performed a meta-analysis to evaluate the carotid artery intima-media thickness (CA-IMT) as a surrogate factor for atherosclerosis in epileptic patients. METHODS: We searched NCBI (PubMed), ISI Web of Knowledge, EMBASE, and the Cochrane Library databases for studies of the association between AEDs and CA-IMT in epileptic patients. A random-effects meta-analysis was used to pool results across studies. RESULTS: Fifteen studies involving 1,775 epileptic patients were included in the analysis. The overall CA-IMT was significantly larger among users of AEDs [mean difference (MD)=0.09 mm, 95% confidence interval (CI)=0.06–0.12 mm). When stratified by age, the MD was similar in adult patients (MD=0.09 mm, 95% CI=0.06–0.13 mm), but no significant difference was observed in children (MD=0.03 mm, 95% CI=0.00–0.07 mm). Regarding specific AEDs, monotherapy with carbamazepine (CBZ) or valproic acid (VPA) was associated with a larger CA-IMT, while phenytoin monotherapy was not and the result for lamotrigine was inconclusive. CONCLUSIONS: This study suggests that using AEDs is associated with the CA-IMT in patients with epilepsy, particularly for adult patients. In particular, CBZ and VPA may be related to a significant increase in CA-IMT.

Rapid resolution of subdural hematoma after targeted epidural blood patch treatment in patients with spontaneous intracranial hypotension / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Subdural hematoma (SDH) is a common complication of spontaneous intracranial hypotension (SIH). To date, the management of SDH caused by SIH remains controversial. In this paper, we reviewed the clinical course of SDH in patients with SIH, and discuss the underlying mechanism and attributing factors for rapid resolution of subdural hematomas after epidural blood patch (EBP) surgery.</p><p><b>METHODS</b>We retrospectively reviewed a cohort of seventy-eight SIH patients diagnosed and treated with targeted EBP in our neurology center. Patients who received early CT/MRI follow-up after EBP operation were included.</p><p><b>RESULTS</b>A series of four cases of SIH complicated with SDHs were evaluated. Early follow-up neuroimages of these patients revealed that SDHs could be partially or totally absorbed just two to four days after targeted epidural blood patch treatment.</p><p><b>CONCLUSION</b>Targeted epidural blood patch can result in rapid hematoma regression and good recovery in some patients with a combination of SDH and SIH.</p>

Γ-secretase inhibitor DAPT prevents neuronal death and memory impairment in sepsis associated encephalopathy in septic rats / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Brain dysfunction is a frequent complication of sepsis, usually defined as sepsis-associated encephalopathy (SAE). Although the Notch signaling pathway has been proven to be involved in both ischemia and neuronal proliferation, its role in SAE is still unknown. Here, the effect of the Notch signaling pathway involved γ-secretase inhibitor DAPT on SAE in septic rats was investigated in a cecal ligation and puncture (CLP) model.</p><p><b>METHODS</b>Fifty-nine Sprague-Dawley rats were randomly divided into four groups, with the septic group receiving the CLP operation. Twenty-four hours after CLP or sham treatment, rats were sacrificed and their hippocampus was harvested for Western blot analysis. TNF-α expression was determined using an enzyme-linked immunosorbent assay (ELISA) kit. Neuronal apoptosis was assessed by TUNEL staining, and neuronal cell death was detected by H&E staining. Finally, a novel object recognition experiment was used to evaluate memory impairment.</p><p><b>RESULTS</b>Our data showed that sepsis can increase the expression of hippocampal Notch receptor intracellular domain (NICD) and poly (adenosine diphosphate [ADP]-ribose) polymerase-1 (PARP-1), as well as the inflammatory response, neuronal apoptosis, neuronal death, and memory dysfunction in rats. The γ-secretase inhibitor N-[N-(3,5-difluorophenacetyl)-1-alanyl]-S-phenylglycine t-butyl ester (DAPT) can significantly decrease the level of NICD and PARP-1, reduce hippocampal neuronal apoptosis and death, attenuate TNF-α release and rescue cognitive impairment caused by CLP.</p><p><b>CONCLUSION</b>The neuroprotective effect of DAPT on neuronal death and memory impairment in septic rats, which could be a new therapeutic approach for treating SAE in the future.</p>

Postictal language testing in patients with temporal lobe epilepsy: a preliminary study / 中华神经科杂志

Objective Postictal language testing can provide useful diagnostic information for seizure lateralization.However no such a study based on non-English language was done previously.We investigated the latency of language recovery in Chinese patients with temporal lobe epilepsy (TLE).Methods Complex partial seizures in patients with TLE were extracted from our video-electroencephalogram (EEG) database.For all patients,consciousness testing started as soon as seizures were detected.When they were alert and cooperative,they were asked to read out a sentence “昨晚他们听到老在电台里讲话”which was printed on a card.When the patients were able to read the sentence correctly,the language function was considered recovered.Results Totally 65 complex partial seizures from 22 cases of TLE (11 left and 11 right) were included.Patients were cooperative to language testing in 54 seizures (83％).The latency for consciousness recovery (CRL) and latency for consciousness language recovery (LRL) were not associated with seizure duration,but the seizure lateralization.The CRL (median,161 s) and LRL (281 s) in the left TLE were statistically significantly longer than that in the right TLE (30 s,54 s respectively).Using 150 s recovery time as bound language recovery ratio was 87％ (27/31) in right TLE and 13％ (3/23) in left TLE.Conclusion Postictal language testing based on ideographic Chinese words helps to establish seizure lateralization in patients with TLE.

Suicide risk and associated risk factors in adult patients with epilepsy / 中华神经科杂志

Objective To investigate the relationships of suicide risk and its associated risk factors in adult patients with epilepsy.Methods All 211 adult patients with epilepsy from the epilepsy clinic of the Second Affiliated Hospital,Zhejiang University School of Medicine,were enrolled to evaluate the presence of suicide risk and depressive disorder by using the suicide module and the depressive disorder module of the Mini International Neuropsychiatric Interview (MINI).Demographic variables for age,gender,employment status,marital status,years of education,and seizure factors for age of onset,types of seizure,seizure frequency and epilepsy duration,result of MRI and types of antiepileptic drugs were also recorded.We compared the differences of risk factors between the two groups with or without suicide risk and investigated the relationships between the depressive disorder and suicide risk.Results The suicide risk of the patients was 21.3％ (45/211),and 17.1％ (36/211) of the patients had depressive disorder.The suicide risk of the epilepsy patients associated with depressive disorder was 75.0％ (27/36),and the suicide risk of the epilepsy patients associated with no depressive disorder was 10.3％ (18/175).There was statistical difference between the two groups (x2 =74.525,P ＜ 0.01).About 60.0％ (27/45) of the patients with suicide risk was accompanied with depressive disorder.As suicide risk increased,the proportion of concurrent depressive disorder elevated.There was significant statistical difference in the rates of depressive disorder among the different suicide risk groups.Conclusions The patients with epilepsy have suicide risk.The suicide risk is higher in patients with depressive disorder.

Quantitative EEG and event-related potentials (P300) in partial epilepsy / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>Quantitative EEG and event-related potential P300 were used to evaluate impairment of cerebral function in patient with partial epilepsy.</p><p><b>METHODS</b>W value was calculated (power of EEG δ and θ rhythm divided by power of α and β rhythm ) for the extent of focal cortical dysfunction. The W values in left partial epilepsy group, right partial epilepsy group and control group during interictal period compared. The latency, amplitude and reaction time of P300 potential change were observed in each groups.</p><p><b>RESULTS</b>The W values in F(8), T(4) and T(6) regions in patients with left partial epilepsy (P <0.05). The W values in T(3). O(1) regions of patients with left partial epilepsy were higher than those in patients with right partial epilepsy (P < 0.05). Furthermore, the W value in T(6) regions of patients with a disease course longer than 5y was significantly higher than that of patients with a disease course 1-5 y or less than 1y; the W value in O(2) regions of patients with a disease course longer than 5y was significantly higher than that of patients with a disease course between 1-5y (P < 0.05). In patients with right or left partial epilepsy, the total abnormal rate of P300 was 54. 76%, the latency, amplitude and reaction team were significantly different to the control group. The abnormal rate of P300 in left and right partial epilepsy groups were 77. 78% and 37.50%, respectively, and the former is significantly higher than the latter. The amplitudes of P300 in C(z) and P(z) of left partial epilepsy were significantly lower than those of right partial epilepsy and control group (P < 0.05). The latency and reaction time of P300 in C(z) and P(z) of all partial epilepsy were significantly longer than those of control group (P < 0.05), however, no difference was found between left and right partial epilepsy.</p><p><b>CONCLUSION</b>In partial epilepsy the cortical dysfunction occurs ipsilaterally to the epileptogenic zone, and extent of cortical dysfunction is positively correlated with duration of disease course. Cerebral dysfunction in left partial epilepsy is more severe than that in right partial epilepsy.</p>

Comparative proteomics of rat brain in the BCNU-induced model of cortical dysplasia / 中华急诊医学杂志

Objective To screen the differential proteins in the brain (neocortex and hippocampus) between the rats with cortical dysplasia (CD) and control ones,and investigate the role of their alteration in the development of epilepsy in CD.Methods Cortical dysplasia was induced in rat pups via in utero delivery of BCNU.A two-dimensional electrophoresis (2-DE)-based approach was used to construct the expression profiles of proteins in both the neocortex and hippocampus at different age groups (postnatal day 7 and 60) and to detect proteome changes between CD rats and control ones.Following gel image analysis,protein spots that differed in abundance between CD and control rats were identified by using Matrx-assisted laser desorption/ionization (MALDI) mass spectrometry (MS) and MS/MS.Results A total of 57 kinds of protein were screened out (P ＜ 0.05),in which 35 were found up-regulated and 22 were down-regulated compared with the control,35 from neonatal stage (postnatal day 7) and others from adult stage.Finally,12 among them were identified,including tubulin,alpha-lB,Beta-actin,tubulin beta-2A,GAP-43,UbCKmit,GAPDH and TMBr-3,etc.Conclusions Changed expression of specific proteins which were found in our study are involved in construction of brain 's cytoskeleton,synaptic function,mitochondrial function and so forth.Thus,they may be related to the pathogenic mechanisms of epileptogenicity of CD.

Imaging and clinical characteristics of Marchiafava-Bignami disease: six cases / 中华神经科杂志

Objective To investigate the presentation and radiologic findings of MarchiafavaBignami disease (MBD).Methods Six cases of MBD who were diagnosed and treated in our hospital were retrospectively analyzed,including the imaging examination(such as cranial CT,magnetic resonance imaging (MRI)),laboratory tests,clinical symptoms and prognosis.Results Six cases of MBD disease were presented with swelled,thickened corpus callosum,iso-or hypo-intensity on T1WI,hyper-intensity on T2WI and fluid attenuated inversion recovery and restricted diffusion on diffusion weighted imaging.Coma of acute onset was the major clinical finding in case 1,case 4 and case 6,which showed the lesions in the entire corpus callosum and extended to the white matter,and the prognosis of these cases were worse.Slow reaction and memory decrease were the clinical findings in case 2 and case 3,which showed the genu and splenium of corpus callosum involvement on MRI and CT,and returned to normal with aggressive treatment.Numbness and weakness of the lower lambs were the major clinical finding in case 5,which showed that the lesion was limited in the splenium and body of corpus callosum on MRI.The patient recovered after treatment.Conclusions MBD may present with various clinical forms,but have characteristic imaging findings.Outset form and neuroimaging characteristics of MBD are critical for improvement of the clinical outcome.

Study of intravenous thrombolysis with recombinant tissue plasminogen activator(rtPA)in isolatedpenetrating artery territory infarcts / 中华急诊医学杂志

Objective To investigate the safety and efficacy of intravenous thrombolytic therapy with recombinant tissue plasminogen activator(rtPA)in patients with isolated penetrating artery territory infarct (IPAI).Methods Data of retrospectively collected clinical,laboratory,and radiological from 75 consecutive patients with acute ischemic stroke treated with intravenous rtPA therapy from June 2009 to April 2011.Etiological classification was carried out according to the Chinese Ischemic Stroke Classification of Subgroups(CISS).The rates of hemorrhagic transformation(HT)and clinical outcomes of patients were compared between IPAI group and non-IPAI group.Results All 75 patients with mean age of 67.4years and 25(33.3％)fenale,were treated with intravenous rtPA.Before treatment,their average score of the National Institutes of Health Stroke Scale(NIHSS)was 12.3 ± 6.4,and mean length of time from onset to treatment was 239.6 ±97.5 minutes.After thrombolytic therapy,the radiological HT was found in 24 patients(32％).Symptomatic intracraneal hemorrhage(ICH)occurred in 4 patients(5.3％).Of 22 (29.3％)patients with IPAI,only one experienced HT.Logistic regression analysis suggested that IPAI wasan individualized predictor used alone for determining the low risk of HT.In the patients with IPAI,82％ of them had an individual clinical outcome(mRS ＜ 2)one month after onset,and the neurological outcomes were better in patients with IPAI than those in patients with non-IPAI(P ＜ 0.01).Conclusions The risk of hemorrhagic complication was low and the clinical outcome was good in patients with isolated penetrating artery territory infarct after intravenous thrombolytic therapy with rtPA.Imaging diagnosis of IPAI might facilitate the treatment with rtPA in this cohort of patients.

Effects of long-term blood pressure variability on cerebral microbleeds / 中华神经科杂志

Objective To assess the reproducibility of long-term blood pressure variability in ischemic stroke and the association between the variability and cerebral microbleeds.Methods Patients with ischemic stroke at the previous 1-6 months were consecutively recruited and followed up 12-18 months.Blood pressure measurements were taken at every interview.Blood pressure variability indicated visit-to visit variability and was quantified by calculating the maximum ( Max),standard deviation (SD) and coefficient of variation (CV).For these variability might positively correlated with mean of blood pressure,the additional variability measure,SD independent of mean (SDIM),was also calculated.To determine the reproducibility of mean and variability measurements,the intraclass correlation (ICC) was also calculated.MRI was performed at baseline and the end of the study.Cerebral microbleeds were rated using Microbleed Anatomical Rating Scale (MARS).Multiple Logistic regression was used to assess the association between the visit-to-visit blood pressure variability and cerebral microbleeds.Results A total of 720 patients were recruited,of whom 595 (82.6％) subjects were present for 14 blood pressure readings during follow-up.The visit-to-visit blood variability measurements were moderately reproducible according to the ICC:0.46-0.72 for systolic blood pressure (SBP) and 0.42-0.69 for diastolic blood pressure ( DBP),respectively,P ＜0.01 for all measures.Patients with cerebral microbleeds were more likely to have higher mean blood pressure and variability for both SBP and DBP regardless the distribution of cerebral microbleeds.Being SBP Max,SBP SD,SBP CV,SBP SDIM(OR=1.036,95％ CI l.021-1.052,P=0.000; OR=1.060,95％CI 1.001-1.122,P =0.046; OR =1.084,95％ CI 1.000-1.175,P =0.049; OR =1.065,95％ CI 1.002-1.132,P =0.044) and DBP SD,DBP CV ( OR =1.111,95％ CI 1.000-1.233,P =0.049;OR =1.091,95％ CI 1.001-1.190,P =0.047) were the independently risk factors of cercbral microbleeds at deep region; SBP Max( OR =1.049,95％ CI 1.029-1.068,P =0.000) and DBP SD、DBP CV(OR =1.236,95％ CI 1.107-1.379,P=0.000;OR =1.188,95％ CI 1.087-1.298,P=0.000)independently associated with cerebral microbleeds at infratentorial location.There was no significant relation between the long-term variability of blood pressure and cerebral microbleeds at lobar region. Conclusions This study indicates that long-term blood pressure variability is substantial and independently associated with cerebral microbleeds in deep or infratentorial but not with that in lobar region.The different relations between the variability and cerebral microbleeds might indicate the heterogenic mechanisms of cerebral microbleeds.

Clinical characteristics and gene mutation analysis of cerebrotendinous xanthomatosis / 中华神经科杂志

Objective To investigate the clinical presentation,laboratory features,imaging findings and CYP27A1 gene mutations of cerebrotendinous xanthomatosis (CTX) for improving the recognition and the early diagnosis and treatment of the disease.Methods Medical records and 8 months follow-up data of one patient who had been clinical diagnosed as CTX were collected and the pedigree and gene mutation analysis of the patient were carried out.Meanwhile,the clinical characters of CTX were analyzed according to the data from our patient and the review of the literature. Results Patient was a 36 years old male manifested with mental retardation, bilateral corticospinal tract and corticonuclear tract impairment,cerebellar lesions and peripheral neuropathy; head MRI indicated symmetric abnormal signals of bilateral basal ganglia,cerebellar dentate nucleus softening and calcification lesions; Achilles tendon MRI indicated markedly thickened Achilles tendon; gene mutation analysis showed sterol-27-hydroxylase gene( CPY27A1 )C→T homozygous mutation in 1016 nucleotide of exon 5.Ursodesoxycholic acid was given as treatment.In 8 months of follow up,for the first 6 months,the patient took medicine regularly and the illness condition was stable.But for the nearly 2 months,the patient voluntarily stopped medicine and the illness condition was worse.Conclusions CPY27A1 gene C→T homozygous mutation in 1016 nucleotide of exon 5 leads to CTX in the patient, which conforms to the characteristic of autosomal recessive disorder. CTX has some characteristic clinical manifestations,such as Achilles tendon thickening,intelligent declining and so on.But lack of specificity of early radiographic examination makes CTX easy to be delayed diagnosis and treatment.CYP27A1 gene mutation analysis has an important significance for early diagnosis of CTX,which should be paid more attention,while the early application of chenodeoxycholicacid treatment can delay the progression of the disease.

Clinical features and molecular genetic analysis of a pedigree of limb girdle muscular dystrophy / 中华神经科杂志

Objective To investigate the clinical features and analyze the molecular genetics of a pedigree of limb girdle muscular dystrophy (LGMD).Methods Pedigree analysis and clinical examination were performed in one four-generation family with LGMD.Electrophysiology and muscle biopsy were done in the affected members.With an informed consent, gene mutation, genome screening and linkage analysis were conducted in 26 members of this pedigree.Results Seven patients were identified.Pedigree analysis was consistent with autosomal dominant inheritance.Affected members had early presentation.Main features included proximal muscle weakness without dysarthria nor spasticity; electrophysiology and muscle biopsy revealed myopathic changes.LGMD1 A, 1B, 1C and facioscapulohumeral dystrophy genes were not detected by gene mutation analysis.Genome screening and linkage analysis did not reveal any linkage with the disease-causing gene and the reported loci of LGMD1D and LGMD1F genes.Conclusions The clinical manifestations of this LGMD family are highly heterogeneous, and the disease-causing gene of this family is not linked to any of the reported sites, suggesting this may be a new disease-causing locus, or a new genetic type of LGMD.

Multicenter randomized, double-blind, placebo-controlled trial of zonisamide as add-on therapy in patients with refractor partial seizures / 中华神经科杂志

Objective To evaluate the efficacy and tolerability of zonisamide (ZNS) as add-on therapy in patients with refractory partial seizures.Methods In this Chinese muiticenter, double-blind, randomized, placebo-contrclled trial, ZNS was compared with placebo add-on therapy in 217 patients (intent-to treat (ITT) population) with uncontrolled partial-onset seizures.All patients entered a 3-month baseline period followed by a 4-week titration interval and a 12-week maintenance period.The starting dose of ZNS group was 100 mg/d, increased by 100 mg/d every week and reached the goal of 400 mg/d.The main outcome was measured by the median of the percentage of decreased seizure frequency.The secondary ouwomes points included the percentage of patients who had seizure attacks decreased by more than 50%,and adverse events.Results The median of the percentage of decreased seizure frequency in ZNS group was 33.33%, and the placebo group was 0.Thirty-eight patients (34.23%) experienced more than 50% reduction in the seizure frequency in ZNS group, compared with 19.81% of patients (21 cases) in the placebo group (χ2 =5.7159,P =0.0168) ; Moreover, 13 (11.71%) patients in ZNS group and 5 patients in placebo group were seizure free, 25 patients in ZNS group and 16 patients in placebo group who had seizure attacks decreased by more than 50%.The availability rate in ZNS group was higher than placebo group (34.23% vs 19.81%, U=2.4701, P=0.0135).The most common adverse events in ZNS group were drowsiness, fatigue, decreased appetite, gastrointestinal complaints, insomnia and constipation.Conclusion Zonisamide treatment was generally well tolerated and was associated with significant reductions in seizure frequency as adjunctive treatment for partial-onset seizures.

The analysis of misdiagnosis and mismanagement of 145 patients with Guillain-Barr? syndrome in the emergency department / 中华急诊医学杂志

Objective To study the clinical characteristics of Guillain-Barr? syndrome(GBS) and the misdiagnosis and mismanagement in emergency department.Methods According to the diagnosis criteria of Chinese Journal of Neurology and Psychology,145 GBS in-hospital patients in our hospital from January 1,1994 to December 312004 were studied to find characteristics of GBS and auxiliary examinations.The reasons for GBS misdiagnosis and mismanagement were analysis.Results Most of the patients were young,the ratio of male to female was 2.5 to 1.Among them,mild-type was 34.5%,medium-type was 25.5%,severe-type was 13.9%,very severe-type was 7.6%,relapse-type was 4.1%,chronic-type was 12.4% and variation-type was 2.1%.The initial symptoms were multiplie.Bilateral limbs weakness and/or numbess were the most common symptom,and non-specificity asymmetrical weakness and/or numbess,headache,ophthalmalgia,distortion of angle of mouth or weak mastication were uncommon symptoms.Twenty-three patients(15.9%)were misdiagnosed in emergency department.71.3% patients developed albuminocytolgoic dissociation in cerebrospinal fluid.The content of protein in cerebrospinal fluid was correlated to the course of disease and uncorrelated to the patitent's condition.Conclusion GBS was a common cause of clinical acute flaccid paralysis,the mild-type has good prognosis and the mortality of very serere-type is high.GBS should be paid attention to in emergency department.

Relationship between plasma homocysteine level and stroke / 中国组织工程研究

BACKGROUND: Hyperhomocysteinemia has been suggested to be a possible independent risk factor for stroke.OBJECTIVE: To explore the relationship between hyperhomocysteinemia and cerebral infarction and hemorrhage, and analyze the factors that affect plasma homocysteine level.DESIGN: Case-controlled clinical trial.SETTING: Department of Neurology, Second Hospital Affiliated to Medical College of Zhejiang University.PARTICIPANTS: Totally 57 patients including 21 with cerebral hemorthage and 36 with brain infarction were treated in the Department of Neurology, Second Hospital Affiliated to Medical College of Zhejiang University Between January and November 2003. Twenty-eight healthy volunteers were also recruited from the subjects coming for routine physical examination.METHODS: Two milliliters of fasting venous blood was collected from all subjects in the morning for detecting the contents of plasma homocysteine,vitamin B12, folic acid, creatinine and so on. All patients were scored for clinical neurological impairment, with the hematoma volume calculated in patients with brain hemorrhage determined on the basis of CT scanning.acid, vitamin B12, clinical neurological impairment score and hematoma volume.RESULTS: Valid results were obtained from all the 57 stroke patients and in male and female patients of both cerebral infarction group and cerebral hemorrhage group than that of the subjects of the same gender in the control group [(25.2±21.4), (18.3±10.9), (11.5±2.9) μmol/L for male subjects;(22.8±18.9), (14.7±7.4), (10.8±2.6) μmol/L for female subjects, P＜ 0.05-0.01].The level of homocysteine was similar between cerebral infarction group and cerebral hemorrhage group, homocysteic acid level showed obvious inverse correlation with folic acid level (r=-0.442, -0.531, P ＜ 0.05), but without relation to vitamin B12 level (r=-0.086, -0.111, P ＞ 0.05). Homocysteine level was not obviously correlated to the neurological impairment scores in cerebral infarction group (r=-0.139, P ＞ 0.05), nor was it related to the scores or hematoma volume in cerebral hemorrhage group (r=0.225,0.425, P ＞ 0.05).CONCLUSION: Hyperhomocysteinemia is risk factor for cerebral infarction and hemorrhage. Plasma homocysteine level is inversely correlated with folic acid level, but not obviously related to vitamin B12, clinical neurologicla impairment score or hematoma volume.

Relationship between the homocysteine level and stroke / 中国康复理论与实践

@#ObjectiveTo study the relationship between the homocysteine (Hcy) level and stroke. Methods57 cases, including cerebral haemorrhage (CH, 21 cases) and cerebral infarction (CI, 36 cases) , and 28 healthy cases as control group were selected to measure their level of Hcy, VitB12 , folic acid and creatinine(Cr). At the same time, the clinical neurological disfunction scale(CNDS) of all patients was evaluated and the hematom volume (HV) of the CH group was assayed. ResultsThe Hcy level of both CI and CH group was significantly higher than that of the control group(P<0.05 or P<0.01). No significant difference was found between CI and CH group on Hcy level. The Hcy level showed a very strong or strong reverse association with folic acid level in the CI and CH group (r=-0.442,P=0.007 or r=-0.531,P=0.013), but no significant correlation with VitB12 level. No correlation was found between Hcy level and CNDS in both the CI and CH group, either no correlation between Hcy level and HV in the CH group. ConclusionThere is a close relationship between hyperhomocysteine and stroke. Hcy level is reversely associated with folic acid level but not correlated with VitB12 level.

Features of clinical and therapy in juvenile myoclonic epilepsy: an analysis of 87 cases / 中华神经科杂志

Objective To analyze clinical characteristics of patients with juvenile myoclonic epilepsy in China. Methods Eighty-seven patients were retrospectively studied in the aspects of family history, febrile seizures, clinical features, EEG, treatment effect. Results There was a female preponderance of incidence. In contrast to the earlier studies we found a high incidence of febrile seizures and a low incidence of family history. myoclonic seizures began at age of ( 13.1?3.4) years. That combined with generalized tonoclonic seizures began at age (14.3?3.8) years. Absence seizures began at age (10.0?3.3) years. The correct diagnosis was delayed at a mean of 2.2 years from onset of the disease. The incidence of abnormal EEG discharge could be enhanced by hyperventilation, photic stimulation and sleep. Sixteen patients who had received carbamazepine or phenytoin were experienced aggravation of seizures. Forty-five patients who received monotherapy with sodium valproate remained seizure-free in a follow-up longer than 0.5 years. Conclusions Failure to recognize JME may result in uncontrolled seizures, and even aggravated of seizures by using antiepilepsy drugs. Effective treatment was achieved with small doses of sodium valproate.