Risk factors for intensive-care-unit-acquired weakness

Wang et al reported 1063 cases from the initial 14 d of intensive care unit(ICU)stay,and analyzed relevant data such as age,comorbidities,recent dosages,vapor pressure dosages,duration of mechanical ventilation,length of ICU stay,and rehabilitation therapy,which are closely related to ICU-acquired weakness(ICUAW).It is suggested that the length of ICU stay and the duration of mechanical ventilation are the main factors.ICU-AW is the most common neuromuscular injury in the ICU,which affects clinical progression and outcomes of patients.This manuscript helps to improve the early recognition of ICU-AW,thereby reducing mortality and improving prognosis.

Therapeutic effect of the metaverse on mental health

López del Hoyo et al collections reported the meta verse based on the virtual reality,augmented reality and artificial intelligence could be used in the therapy of mental health,although there were still some challenges.This manuscript reported that the meta verse is a prospective method to improve the prognosis of mental health problems.

Gp78 regulates PMP22 and causes ER stress and autophagy in EV71-VP1-overexpressing mouse Schwann cells

Background:During Enterovirus type 71(EV71)infection,the structural viral protein 1(VP1)activates endoplasmic reticulum(ER)stress associated with peripheral myelin protein 22(PMP22)accumulation and induces autophagy.However,the specific mechanism behind this process remains elusive.Methods:In this research,we used the VP1-overexpressing mouse Schwann cells(SCs)models co-transfected with a PMP22 silencing or Autocrine motility factor receptor(AMFR/gp78)overexpressing vector to explore the regulation of gp78 on PMP22 and its relationship with autophagy and apoptosis.Results:The activity of gp78 could be influenced by EV71-VP1,leading to a decrease in the ubiquitination and degradation of PMP22,resulting in PMP22 accumulation in ER.In VP1-overexpressing mouse SCs,all three ER stress sensors,including pancreatic endoplasmic reticulum kinase(PERK),activating transcription factor 6(ATF6)and inositol-requiring enzyme 1(IRE1)and the related downstream signals(C/EBP-homologous protein(CHOP)and Caspase 12)were activated,as well as the ER-resident chaperone Glucose-regulated protein 78(GRP78).In addition,VP1 upregulated the autophagy marker Microtubule-associated protein 1 light chain 3 beta(LC3B),while PMP22 silencing or gp78 overexpression reversed the phenomenon.Meanwhile,PMP22 silencing or gp78 overexpression increased proliferation of EV71-VP1-transfected mouse SCs.Conclusion:Gp78 could regulate PMP22 accumulation through ubiquitination degradation and cause ER stress and autophagy in EV71-VP1-overexpressing mouse SCs.Therefore,the gp78/PMP22/ER stress axis might emerge as a promising therapeutic target for myelin and neuronal damage induced by EV71 infection.

Analysis of Smooth Cepstral Peak Prominence in Hypokinetic Dysarthria Associated With Parkinson’s Disease

Smoothed cepstral peak prominence(CPPs)is a measurement of the distance from the prominent cepstral peak to the linear regression line directly beneath it.Variations of CPPs data acquisition and analysis lead to the complexity of the clinical cut-off values,and there are no agreeable values for a specific voice disorder,such as hypokinetic dysarthria associated with Parkinson’s disease(PD).This study examined the CPPs in people with hypokinetic dysarthria associated with PD compared with healthy participants.Results demonstrated significant differences in speech tasks of sustained vowel and connected speech,with CPPs of connected speech more sensitive to dysphonia and gender difference in PD participants.Males in PD participants presented higher CPPs for sustained vowels and lower CPPs for connected speech than females.It is implied that a consistent clinical application protocol is necessary,and multiple acoustic measures are needed to ensure the accuracy of clinical decisions.

The First Pilot Epigenetic Type Improvement of Neuropsychiatric Symptoms in a Polymorphic Dopamine D2 (-DRD2/ANKK (Taq1A)), OPRM1 (A/G), DRD3 (C/T), and MAOA (4R) Compromised Preadolescence Male with Putative PANDAS/CANS: Positive Clinical Outcome with Precision-Guided DNA Testing and Pro-Dopamine Regulation (KB220) and Antibacterial Therapies

Pediatric autoimmune neuropsychiatric disorders associated with or without streptococcal and other bacterial infections (PANDAS/CANS) are emerging as a featured pediatric disorder. Although there is some controversy regarding treatment approaches, especially related to the behavioral sequelae, we have hypothesized in other published work that it is characterized by the rapid onset of Reward Deficiency Syndrome (RDS) in children. We propose utilizing a multi-systems biological approach involving the coupling of genetic addiction risk testing and pro-dopamine regulation (KB220/POLYGEN®) to help induce “dopamine homeostasis” in patients with PANDAS, especially those with known DNA-induced hypodopaminergia. This case study examines a 12-year-old Caucasian male with no prior psychiatric issues who presented with a sudden onset of severe anxiety, depression, emotional liability, and suicidal ideation. The patient underwent genotyping and the genetic addiction risk score (GARS) testing, which revealed risk polymorphisms in the dopamine D2 (-DRD2/ANKK (Taq1A), OPRM1 (A/G), DRD3 (C/T), and MAOA (4R) genes. These polymorphisms have been linked to hypodopaminergia. The patient was subsequently placed on research ID-KB220ZPBMPOLY (POLYGEN®), and albeit the possibility of bias, based upon self and parental assessment, a marked rapid improvement in psychiatric symptoms was observed. In the second phase of treatment (102 days utilizing KB220), the patient received standard antibody testing, which was positive for Lyme. Antibacterial therapy started immediately, and KB220z was discontinued to provide a wash-out period. A monotonic trend analysis was performed on each outcome measure, and a consistently decreasing trend was observed utilizing antibacterial therapy. Our recommendation, albeit only one case, is to utilize and further research a combined therapeutic approach, involving precision-guided DNA testing and pro-dopamine regulation along with antibacterial therapy, as well as glutathione to address offensive enhanced cytokines, in patients with suspected PANDAS/CANS.

Etiologies and Outcome of Children with Purulent Meningitis at the Yaounde Gyneco-Obstetric and Pediatric Hospital (Cameroon)

Background: Bacterial meningitis is one of the most severe infections in infants and children. It is associated with high mortality and neurological sequelae. In order to improve the prognosis of infants and children with purulent meningitis, we decided to conduct this study whose main objective was to identify the main pathogens responsible and describe the outcome in infants and children aged 2 months to 15 years admitted for purulent meningitis at the Yaounde Gyneco-Obstetric and Pediatric Hospital (YGOPH). Method: This was a cross-sectional study with retrospective data collection and consecutive sampling. Our study was conducted from 1 January 2009 to 31 December 2013. The patients included in the study were infants and children aged from 2 months to 15 years who were admitted for bacterial meningitis at the YGOPH, confirmed by bacteriological examination of cerebrospinal fluid (CSF) with identification of the pathogen by culture or soluble antigen. The data was analyzed using SPSS Version 18.0 and Excel 2007. The Chi-square test was used to determine the association of various variables. The significance threshold was set as P 0.05. Results: We selected 171 cases of purulent meningitis who represented 1.54% of admitted patients. The sex ratio was 1.2. We noted that 45% of our patients were aged 2 months to 1 year. The main presenting complaints were fever (98.8%), seizures (44.4%) and vomiting (28.7%). Haemophilus influenzae was found in 67 children (39.2%), followed by Streptococcus pneumoniae in 54 children (31.6%) and Neisseria meningitidis in 17 children (9.9%). Acute complications (status epilepticus, coma) were seen in 33% of patients. The statistically significant (P 0.05) factors for poor prognosis were aged from 2 months to 1 year (P = 0.0004), coma (P = 0.32), intracranial hypertension (P = 0.0001), the pathogen (P = 0.0032Pneumococcus), a delay of more than three days between the onset of the disease and the treatment (P = 0.0134) and brain abscess (P = 0.0001). We identified 32 deaths (18.7%) and 17 cases (9.9%) with neurological sequelae before discharge. Conclusion: The incidence of acute bacterial meningitis remains high in our context. The main causes were Haemophilus influenzae, Streptococcus pneumoniae and Neisseria meningitis. The mortality rate was high with poor prognosis factors such as age less than 12 months, delayed care, pneumococcal meningitis, coma, brain abscess, and intracranial hypertension. Focus should be placed on strengthening the routine immunization on vaccine-preventable diseases of infants and children against Haemophilus influenzae, Pneumococcus and Meningococcus.

Causes of developmental delay in children of 5 to 72 months old at the child neurology unit of Yaounde Gynaeco-Obstetric and Paediatric Hospital (Cameroon)

Background: According to the World Health Organization, about 5% of children world-wide of 14-year-old and under have a moderate to severe developmental disability, and up to 15% of children under 5-year-old are developmentally delayed. Purpose: To determine the prevalence, socio-demographic profile, aetiologies, and the clinical presentation of developmental delay in children less than 6-year-old at the child neurology unit in a university-affiliated hospital in Yaounde. Materials and methods: It was a crosssectional descriptive study carried out in Yaounde Gynaeco-Obstetric and Paediatric Hospital (Cameroon) from August to December 2012. Children aged between 5 - 72 months with a developmental quotient less than 70 were enrolled. Developmental delay (DD) was diagnosed and classified using the Denver developmental screening test (DDST). Data concerning the child (age, gender, severity of DD), the mother (age, age at conception, educational level, marital status), history of pregnancy and delivery, perinatal and postnatal events, results of para-clinical explorations (EEG, CT-scan, genetic tests), the severity of DD and the probable or demonstrate cause of DD were recorded on a standardized questionnaire. The chisquare test was used to compare variables. Results: During the study period, 2171 children aged 5 - 72 months consulted the paediatric department of the hospital, 296 were examined at the child neurology unit of which 153 had a developmental quotient less than 70, giving a hospital prevalence of 7.0% and a prevalence of 51.7% at the child neurology unit. The mean age was 26.6 ± 18.0 months and there were 56% males. The main reason for consulting was tonus disorder (43.8%) and the developmental area of parental concern was the motor domain (90.2%). Regarding the clinical presentation, 75.2% of our population were children with cerebral palsy. DD was severe, mild, moderate and profound respectively in 14.2%, 13.5%, 12.2%, and 11.1%. Gross DD represented 90.2% of all DD children. The causes of DD were hypoxic-ischemic encephalopathy (41.8%), epilepsy (13.7%), sequelae of meningitis (6.5%), sequelae of kernicterus (6.5%), and infectious embryofoetopathies (5.2%). Conclusion: Developmental delay is frequent in paediatric neurology, with perinatal disorders being the leading aetiologies in Cameroon. Prevention of perinatal hypoxic-ischemic encephalopathy risk factors needs to be reinforced.

Clinical Presentations of Pediatric Horner Syndrome

Purpose: To understand the multiple signs of Horner syndrome and to recommend protocols for pediatricians to obtain an accurate diagnosis of Horner syndrome. Methods: The medical records of 17 pediatric patients with Horner syndrome, neonates to eighteen years of age, were collected and analyzed. Data recorded included age, presenting symptoms, other medical history, allergies, medications, pupil size, presence of anhidrosis, and presence of ptosis. From the available pupil sizes, average degree of anisocoria was calculated. Results: All 17 patients had other clinical findings of Horner syndrome in addition to anisocoria. On initial evaluation, 100% had ptosis and 25% had anhidrosis. Of the available pupil size data, the average level of anisocoria was 2.06 mm, with a standard deviation of 1.17 mm. Conclusion: Physicians are reminded to measure pupil size to determine the degree of anisocoria when present, as it may help distinguish benign conditions from underlying pathology. Educating pediatricians on measurement of anisocoria and additional signs of Horner syndrome will help with proper referral patterns.

Evaluating visual outcomes using optical coherence tomography(OCT)in pediatric multiple sclerosis and other neuroinflammatory conditions

Optical coherence tomography(OCT)is a technology that is widely used to assess structural abnormalities in the retina for a variety of pediatric conditions.The introduction of this instrument has allowed for widespread access to minimally invasive standardized,reproducible quantified structural assessments of the optic nerve and retina.This has had important implications in pediatric optic neuropathies,populations in whom monitoring of disease activity is essential to make treatment decisions.OCT has had particular relevance for inflammatory optic neuropathies,as onset of an inflammatory optic neuropathy may herald the onset of a chronic inflammatory disorder of the central nervous system(CNS)such as multiple sclerosis,neuromyelitis optica spectrum disorder(aquaporin 4 antibody positive),and myelin oligodendrocyte glycoprotein(MOG)associated disorders.This paper will focus on the application of OCT technology to this group of disorders in pediatrics.After reviewing pediatric-specific anatomic and practical issues pertinent to OCT,we will review knowledge related to the use of OCT in inflammatory pediatric optic neuropathies,with a focus on structural outcomes and their correlation with functional outcome metrics.

Autoimmune complications and clinical outcomes of herpes simplex encephalitis in children: A case series

Objective:To report the neurologic prognosis and autoimmune complications of 16 cases of childhood herpes simplex virus encephalitis.Methods:The study was conducted atŞanlıurfa Training and Research Hospital,Turkey from June 2017 to August 2019.The study included 16 pediatric patients aged between 6 months and 17 years(median age 77.7 months)who were diagnosed with herpes simplex virus type 1 encephalitis by pediatric infectious disease and pediatric neurology clinics.Patients were followed using patient records,and interviews at the pediatric neurology clinic or via the telephone.Clinical and demographic data,received therapies,neurologic prognosis and complications were evaluated.Results:Patients with and without autoimmune encephalitis were compared in terms of age,sex,symptom duration before treatment,initial cerebrospinal fluid protein,glucose,red blood count and white blood count but no significant difference was found.Autoimmune complications were seen in four patients.N-methyl-D-aspartate encephalitis was observed in three patients and choreoathetosis was seen in one patient.The average follow-up period was 48.3 months.Twenty-five percent of the patients were receiving multiple antiepileptic drug(AED)treatment,43.8%were receiving single AED treatment and 31.3%were not receiving AED treatment at the end of the follow-up.Motor disability was observed in 12.5%and drug-resistant epilepsy was observed in 6.3%who had autoimmune complications.Conclusions:Seizures and movement disorders were controlled with immunotherapy and autoantibodies should be studied routinely.Treatment should be started early upon recognition of autoimmune complications through follow-up by measuring autoantibody levels and clinical examination results.Effective prevention and curative treatment modalities are needed to avoid herpes simplex virus encephalitis complications.

Reading impairment after neonatal hypoglycemia with parietotemporo-occipital injury without cortical blindness:A case report

BACKGROUND Perinatal brain injury may lead to later neurodevelopmental disorders,whose outcomes may vary due to neuroplasticity in young children.Recent neuroimaging studies have shown that the left parietotemporal area(which includes the left inferior parietal lobe)is associated with phonological awareness and decoding skills,which are essential skills for reading acquisition in children.However,the literature on the effect of perinatal cerebral injury on the development of phonological awareness or decoding ability in childhood is limited.CASE SUMMARY We report the case of an 8-year-old boy who presented with reading difficulty following a perinatal injury in the parieto-temporal-occipital lobes.The patient was born at term and was treated for hypoglycemia and seizures during the neonatal period.Diffusion-weighted brain magnetic resonance imaging on postnatal day 4 revealed cortical and subcortical hyperintensities in the parieto-temporo-occipital lobe.At the age of 8 years,physical examination was unremarkable,aside from mild clumsiness.Despite occipital lobe injury,the patient had adequate visual acuity,normal eye movement,and no visual field defects.Full-scale intelligence quotient and verbal comprehension index on Wechsler Intelligence Scale for Children-Fourth Edition were 75 and 90,respectively.Further assessment revealed adequate recognition of Japanese Hiragana letters.However,he had significantly slower reading speed in the Hiragana reading test than control children.The phonological awareness test revealed significant errors(standard deviation+2.7)in the mora reversal task.CONCLUSION Patients with perinatal brain injuries in the parietotemporal area require attention and may benefit from additional reading instructions.

Ketogenic diet poses a significant effect on imbalanced gut microbiota in infants with refractory epilepsy

AIM To investigate whether patients with refractory epilepsy and healthy infants differ in gut microbiota(GM),and how ketogenic diet(KD) alters GM.METHODS A total of 14 epileptic and 30 healthy infants were recruited and seizure frequencies were recorded. Stool samples were collected for 16 S r DNA sequencing using the Illumina Miseq platform. The composition of GM in each sample was analyzed with MOTHUR,and intergroup comparison was conducted by R software.RESULTS After being on KD treatment for a week,64% of epileptic infants showed an obvious improvement,with a 50% decrease in seizure frequency. GM structure in epileptic infants(P1 group) differed dramatically from that in healthy infants(Health group). Proteobacteria,which had accumulated significantly in the P1 group,decreased dramatically after KD treatment(P2 group). Cronobacter predominated in the P1 group and remained at a low level both in the Health and P2 groups. Bacteroides increased significantly in the P2 group,in which Prevotella and Bifidobacterium also grew in numbers and kept increasing.CONCLUSION GM pattern in healthy infants differed dramatically from that of the epileptic group. KD could significantly modify symptoms of epilepsy and reshape the GM of epileptic infants.

Cardiac involvement in Duchenne and Becker muscular dystrophy

Duchenne and Becker muscular dystrophy(DMD/BMD) are X-linked muscular diseases responsible for over 80% of all muscular dystrophies. Cardiac disease is a common manifestation,not necessarily related to the degree of skeletal myopathy; it may be the predominant manifestation with or without any other evidence of muscular disease. Death is usually due to ventricular dysfunction,heart block or malignant arrhythmias. Not only DMD/BMD patients,but also female carriers may present cardiac involvement. Clinically overt heart failure in dystrophinopathies may be delayed or absent,due to relative physical inactivity. The commonest electrocardiographic findings include conduction defects,arrhythmias(supraventricular or ventricular),hypertrophy and evidence of myocardial necrosis. Echocardiography can assess a marked variability of left ventricular dysfunction,independently of age of onset or mutation groups. Cardiovascular magnetic resonance(CMR) has documented a pattern of epicardial fibrosis in both dystrophinopathies' patients and carriers that can be observed even if overt muscular disease is absent. Recently,new CMR techniques,such as postcontrast myocardial T1 mapping,have been used in Duchenne muscular dystrophy to detect diffuse myocardial fibrosis. A combined approach using clinical assessment and CMR evaluation may motivate early cardioprotective treatment in both patients and asymptomatic carriers and delay the development of serious cardiac complications.

Hepatic echinococcosis:Clinical and therapeutic aspects

Echinococcosis or hydatid disease （HD） is a zoonosis caused by the larval stages of taeniid cestodes belong- ing to the genus Echinococcus. Hepatic echinococcosis is a life-threatening disease, mainly differentiated into alveolar and cystic forms, associated with Echinoc- cus multilocularis （E. multi/ocular/s） and Echinococcus granulosus （E. granulosus） infection, respectively. Cys- tic echinococcosis （CE） has a worldwide distribution, while hepatic alveolar echinococcosis （AE） is endemic in the Northern hemisphere, including North America and several Asian and European countries, like France, Germany and Austria. E. granulosus young cysts are spherical, unilocular vesicles, consisting of an internal germinal layer and an outer acellular layer. Cyst expansion is associated with a host immune reaction and the subsequent development of a fibrous layer, called the per/cyst; old cysts typically present internal septa- tions and daughter cysts. E. multilocularis has a tumor-like, infiltrative behavior, which is responsible for tissue destruction and finally for liver failure. The liver is the main site of HD involvement, for both alveolar and cystic hydatidosis. HD is usually asymptomatic for a long period of time, because cyst growth is commonly slow; the most frequent symptoms are fatigue and abdominal pain. Patients may also present jaundice, hepatomegaly or anaphylaxis, due to cyst leakage or rupture. HD diagnosis is usually accomplished with the combined use of ultrasonography and immunodiagnosis; furthermore, the improvement of surgical techniques, the introduction of minimally invasive treatments [such as puncture, aspiration, injection, re-aspiration （PAIR）] and more effective drugs （such as benzoimidazoles） have deeply changed life expectancy and quality of life of patients with HD. The aim of this article is to provide an up-to-date review of biological, diagnostic, clinical and therapeutic aspects of hepatic echinococcosis.

Association of HLA-B*1502 and*1511 Allele with Antiepileptic Drug-induced Stevens-Johnson Syndrome in Central China

Previous studies have demonstrated a strong association between carbamazepine（CBZ）-induced Stevens-Johnson syndrome（SJS） and HLA-B 1502 in Han Chinese. Here, we extended the study of HLA-B 1502 susceptibility to two different antiepileptic drugs, oxcarbazepine（OXC） and phenobabital（PB）. In addition, we genotyped HLA-B 1511 in a case of CBZ-induced SJS with genotype negative for HLA-B 1502. The presence of HLA-B 1502 was determined using polymerase chain reaction with sequence-specific primers（PCR-SSP）. Moreover, we genotyped HLA-B 1502 in 17 cases of antiepileptic drugs（AEDs）-induced cutaneous adverse drug reactions（cADRs）, in comparison with AEDs-tolerant（n=32） and normal controls（n=38） in the central region of China. The data showed that HLA-B 1502 was positive in 5 of 6 cases of AEDs-induced SJS（4 CBZ, 1 OXC and 1 PB）, which was significantly more frequent than AEDs-tolerant（2/32, 18 CBZ, 6 PB and 8 OXC） and normal controls（3/38）. Compared with AEDs-tolerant and normal controls, the OR for patients carrying the HLA-B 1502 with AEDs-induced SJS was 6.25（95% CI： 1.06–36.74） and 4.86（95% CI： 1.01–23.47）. The sensitivity and specificity of HLA-B 1502 for prediction of AEDs-induced SJS were 71.4%. The sensitivity and specificity of HLA-B 1502 for prediction of CBZ-induced SJS were 60% and 94%. HLA-B 1502 was not found in 11 children with maculopapular exanthema（MPE）（n=9） and hypersensitivity syndrome（HSS）（n=2）. However, we also found one case of CBZ-induced SJS who was negative for HLA-B 1502 but carried HLA-B 1511. It was suggested that the association between the CBZ-induced SJS and HLA-B 1502 allele in Han Chinese children can extend to other aromatic AEDs including OXC and PB related SJS. HLA-B 1511 may be a risk factor for some patients with CBZ-induced SJS negative for HLA-B 1502.

Psycho-cognitive behavioral problems in sleep disordered children

Sleep disturbances are common in childhood and adolescence. Sleep problems in early infants tend to be persistent and prominent in preschool and school-aged children. Chronic sleep disorders, especially in young children may lead to neurobehavioral problems and psycho-cognitive impairment. Sleep difficulties may be the result of underlying medical conditions, （breathing disorders） or psychological problems. Research studies have shown the association between sleep disorders and day time cognitive impairment, behavioral problems, poor school performance and inattention in children. Appropriate diagnosis and early management of sleep disorders in children lead to improvement of neurocognitive function and behavioral problems in these children.

Posterior quadrantic disconnection maintains the activity of isolated temporal-parietal-occipital nerve tissue: neuroprotective measures in the surgical treatment of epilepsy

Extensive lesions involving the posterior quadrant of the cerebral hemisphere （temporal, parietal, and occipital lobes） induce intractable epilepsy. These patients are potential candidates for surgical treatmenttu. Maintenance of isolated nerve tissue activity after surgery plays a crucial role in the neuroprotective effects of neurosurgery treatment. Disconnection surgery of the posterior quadrant is used to completely isolate nerve fibers, while blood supply at the isolated lobes is maintained. Subsequently, cavities caused by cystic or necrotic nerve tissues should be reduced as much as possible,

Cyclic vomiting syndrome in children: Experience with 181 cases from southern Iran

AIM： To evaluate the clinical presentation, response to prophylactic therapy and outcome of children with cyclic vomiting syndrome （CVS） in Shiraz, Iran. METHODS： During a period of 11 years （March 1994 to March 2005）, 181 consecutive children with a final diagnosis of CVS were evaluated, treated and followed in our center. Patients were randomized to receive either amitriptyline or propranolol as prophylactic treatments. RESULTS： There were 88 boys and 93 girls with mean age of onset of symptoms of 4.9 ± 3.3 years （range, neonatal period to 14 years）, the mean age at final diagnosis was 6.9 years （range, 1.5 to 14）, and the mean duration between the onset of the first attack and the final diagnosis of CVS was 2 ± 1.81 years （range, 1/6 to 8）. The mean duration of each attack was 4.26 days （range, from few hours to 10 d） and the mean interval between the attacks was 1.8 mo （range, 1 wk to 12 too）. The time of onset of the attacks was midnight to early morning in about 70% of cases. Amitriptyline was effective in 46 out of 81 （56%） patients （P 〈 0.001）. Propranolol appeared to have a superior action and was effective in 74 out of 83 （92%） patients （P 〈 0.0001）. CONCLUSION： There is a significant lag time between the onset of clinical symptoms and the final diagnosis of CVS in our area. In patients with typical clinical presentations of CVS, who are examined by an experienced physician, invasive workup is not necessary. Propranolol appears more effective than arnitriptyline for prophylactic use in children with CVS.

不同组织病理亚型的中央颞叶癫的~1H-MR波谱

此研究的目的是分析质子MR波谱（1^H-MRS）对不同组织病理亚型的中央颞叶癫痫（MTLE）的定向价值，并且将结果与临床、MRI和癫痫发作的预后数据进行相关分析。回顾性研究了一组35例经癫痫手术切除的病人，评价了海马的质子MR波谱。采用LC Model方法获得代谢物浓度，计算了NAA／Cr、NAA／Cho、NAA／（Cr＋Cho）、Cho／Cr值和不对称系数。

Voltage gated calcium channel antibody-related neurological diseases

Voltage gated calcium channel(VGCC) antibodies are generally associated with Lambert-Eaton myasthenic syndrome. However the presence of this antibody has been associated with paraneoplastic as well as nonparaneoplastic cerebellar degeneration. Most patients with VGCC-antibody-positivity have small cell lung cancer(SCLC). Lambert-Eaton myasthenic syndrome(LEMS)is an autoimmune disease of the presynaptic part of the neuromuscular junction. Its classical clinical triadis proximal muscle weakness, areflexia and autonomic dysfunction. Fifty to sixty percent of LEMS patients have a neoplasia, usually SCLC. The co-occurrence of SCLC and LEMS causes more severe and progressive disease and shorter survival than non-paraneoplastic LEMS. Treatment includes 3,4 diaminopyridine for symptomatic purposes and immunotherapy with prednisolone, azathioprine or intravenous immunoglobulin in patients unresponsive to 3,4 diaminopyridine. Paraneoplastic cerebellar degeneration(PCD) is a syndrome characterized with severe, subacute pancerebellar dysfunction. Serum is positive for VGCC antibody in 41%-44% of patients, usually with the co-occurrence of SCLC. Clinical and electrophysiological features of LEMS are also present in 20%-40% of these patients. Unfortunately, PCD symptoms do not improve with immunotherapy. The role of VGCC antibody in the immunopathogenesis of LEMS is well known whereas its role in PCD is still unclear. All patients presenting with LEMS or PCD must be investigated for SCLC.