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.

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.

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 belonging to the genus Echinococcus.Hepatic echinococcosis is a life-threatening disease,mainly differentiated into alveolar and cystic forms,associated with Echinoccus multilocularis(E.multilocularis)and Echinococcus granulosus(E.granulosus)infection,respectively.Cystic 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 expan-sion is associated with a host immune reaction and the subsequent development of a fibrous layer,called the pericyst;old cysts typically present internal septations and daughter cysts.E.multilocularis has a tumorlike,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-todate 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.

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 mo). 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 amitriptyline for prophylactic use in children with CVS.

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,

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.

Rh-incompatible hemolytic disease of the newborn in Hefei

BACKGROUND Anti-D antibody is not the common cause of Rh-isoimmunization in Chinese neonatal jaundice.Recent change in national population policy has followed by an increase in Rh-isoimmunization related hemolytic disease of the newborn(HDN).Unfortunately,regional status of Rh-HDN is unavailable.We hypothesize that Rh-HDN in our region is most commonly due to anti-E antibody.AIM To investigate the prevalence of hemolytic disease of the newborn due to Rhisoimmunization in Hefei City.METHODS Retrospective review of data obtained from Children’s Hospital of Anhui and Hefei Blood Center between January 2017 and June 2019.Status of minor blood group antibody was studied in the corresponding mothers.RESULTS Totally 4138 newborns with HDN admitted during the study period and 116(2.8%)received blood exchange transfusion(BET).Eighteen newborns(0.43%)with proven Rh-incompatible HDN were identified.All were not the first-born baby.Thirteen mothers were RhD(+)(72%)and five were RhD(-).The distribution of Rh-related antibodies in mothers was ten anti-E(55%),five anti-D(27%),and for one anti-C,anti-c,and anti-E/c(6%)each.Thirteen(72.2%)were qualified for BET,relative risk for BET was 28.9 as compared to other types of HDN,but only 10 received due to parenteral refusal.All(100%)RhD related HDN received BET which is not significantly different from RhE related HDN(81.8%).CONCLUSION As expected,all Rh-incompatible HDN newborns were not the first-born.Contrary to the Caucasian population,anti-D induced HDN is not the most common etiology.In our region,anti-E(11/18,61%)is the most common cause of Rh-HDN.

Cerebrolysin improves sciatic nerve dysfunction in a mouse model of diabetic peripheral neuropathy

To examine the effects of Cerebrolysin on the treatment of diabetic peripheral neuropathy, we first established a mouse model of type 2 diabetes mellitus by administering a high-glucose, high-fat diet and a single intraperitoneal injection of streptozotocin. Mice defined as diabetic in this model were then treated with 1.80, 5.39 or 8.98 m L/kg of Cerebrolysin via intraperitoneal injections for 10 consecutive days. Our results demonstrated that the number, diameter and area of myelinated nerve fibers increased in the sciatic nerves of these mice after administration of Cerebrolysin. The results of several behavioral tests showed that Cerebrolysin dose-dependently increased the slope angle in the inclined plane test（indicating an improved ability to maintain body position）, prolonged tail-flick latency and foot-licking time（indicating enhanced sensitivity to thermal and chemical pain, respectively, and reduced pain thresholds）, and increased an index of sciatic nerve function in diabetic mice compared with those behavioral results in untreated diabetic mice. Taken together, the anatomical and functional results suggest that Cerebrolysin ameliorated peripheral neuropathy in a mouse model of type 2 diabetes mellitus.

Clinical features of SARS-CoV-2-associated encephalitis and meningitis amid COVID-19 pandemic

BACKGROUND Since the severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)epidemic,numerous studies have been published on SARS-CoV-2-related encephalitis/meningitis,but it has not been established if there are specific clinical characteristics of encephalitis/meningitis associated with SARS-CoV-2 infection.AIM To identify the specific clinical features of cases of encephalitis/meningitis associated with SARS-CoV-2 infection in the context of this virus infection pandemic and investigate their relationship with SARS-CoV-2 infection.METHODS We searched PubMed,and included single case reports and case series with full text in English,reporting original data of coronavirus disease-19(COVID-19)patients with encephalitis/meningitis and a confirmed recent SARS-CoV-2 infection.Clinical data were extracted.RESULTS We identified 22 articles(18 single case reports and 4 case series)reporting on a total of 32 encephalitis/meningitis patients with confirmed SARS-CoV-2 infection.SARS-CoV-2 infection was confirmed through reverse transcriptase-polymerasechain-reaction(RT-PCR)in 96.88%of cases.A total of 22(68.75%)patients had symptoms of SARS-CoV-2 infection in about 1 wk(7.91 d)preceding the onset of neurologic symptoms.The most common neurological symptoms were consciousness disturbance(59.38%),seizure(21.88%),delirium(18.75%),and headache(18.75%).Four cases were confirmed by positive RT-PCR results in cerebrospinal fluid(CSF),one was confirmed by positive RT-PCR results in postoperative brain tissue,and one by the presence of SARS-CoV-2 antibodies in CSF.The mainly damaged targets identified by neuroimaging included the temporal lobe(15.63%),white matter(12.5%),frontal lobe(9.38%),corpus callosum(9.38%),and cervical spinal cord(9.38%).Eighty percent of patients had electroencephalograms that showed a diffuse slow wave.Twenty-eight(87.5%)patients were administered with specific treatment.The majority(65.63%)of patients improved following systemic therapy.CONCLUSION Encephalitis/meningitis is the common neurological complication in patients with COVID-19.The appropriate use of definitions and exclusion of potential similar diseases are important to reduce over-diagnosis of SARS-CoV-2 associated encephalitis or meningitis.

A quadratic linear-parabolic model-based EEG classification to detect epileptic seizures

The two-point central difference is a common algorithm in biological signal processing and is particularly useful in analyzing physiological signals.In this paper,we develop a model-based classification method to detect epileptic seizures that relies on this algorithm to filter electroencephalogram(EEG) signals.The underlying idea was to design an EEG filter that enhances the waveform of epileptic signals.The filtered signal was fitted to a quadratic linear-parabolic model using the curve fitting technique.The model fitting was assessed using four statistical parameters,which were used as classification features with a random forest algorithm to discriminate seizure and non-seizure events.The proposed method was applied to 66 epochs from the Children Hospital Boston database.Results showed that the method achieved fast and accurate detection of epileptic seizures,with a92% sensitivity,96% specificity,and 94.1% accuracy.

Mild Encephalopathy/Encephalitis with a Reversible Splenial Lesion(MERS):A Report of Five Neonatal Cases

Mild encephalopathy/encephalitis with a reversible splenial(MERS) lesion is a clinic-radiological entity. The clinical features of MERS in neonates are still not systemically reported. This paper presents five cases of MERS, and the up-to-date reviews of previously reported cases were collected and analyzed in the literature. Here we describe five cases clinically diagnosed with MERS. All of them were neonates and the average age was about 4 days. They were admitted for the common neurological symptoms such as hyperspasmia, poor reactivity and delirium. Auxiliary examinations during hospitalization also exhibited features in common. In this report, we reached following conclusions. Firstly, magnetic resonance imaging revealed solitary or comprehensive lesions in the splenium of corpus callosum, some of them extending to almost the whole corpus callosum. The lesions showed low intensity signal on T1-weighted images, homogeneously hyperintense signal on T2-weighted images, fluid-attenuated inversion recovery and diffusion-weighted images, and exhibited an obvious reduced diffusion on apparent diffusion coefficient map. Moreover, the lesions in the magnetic resonance imaging disappeared very quickly even prior to the clinical recovery. Secondly, all the cases depicted here suffered electrolyte disturbances especially hyponatremia which could be easily corrected. Lastly, all of the cases recovered quickly over one week to one month and majority of them exhibited signs of infections and normal electroencephalography.

Late-onset Leigh syndrome without delayed development in China:A case report

BACKGROUND Leigh syndrome(LS)is one of the most common mitochondrial diseases in infants and children.LS often manifests as early-onset with delayed phenotypic development.However,late-onset LS with normal development and white matter lesions in the brain is rarely reported,thereby highlighting the phenotypic variability of LS expression.CASE SUMMARY We report a 12-year-old boy who presented with an unusual late-onset and fulminant form of LS that is maternally inherited without developmental delay.The patient was admitted to the hospital with symptoms of ptosis and somnolence,and died within 2 mo.Analysis of peripheral blood leukocytes showed a homoplasmic m.9176T>C mutation in the patient.Magnetic resonance imaging also revealed lesions in bilateral white matter as well as symmetrical lesions in the basal ganglia and brain stem.The patient was diagnosed with LS.The patient was treated with vitamin C,vitamin D,and adenosine-triphosphate.The patient died within 2 mo of hospital admission.CONCLUSION LS can present in both infants and older children with different phenotypes.

Recurrent headaches may be caused by cerebral toxoplasmosis

AIM: To establish seroprevalence and provide characteristics of Toxoplasma gondii(TG) infection in children with recurrent headaches. METHODS: The study was performed in 178 children aged 7-17 years admitted consecutively to the Department of Pediatric Neurology from November 2009 to July 2011. The children were surveyed with a questionnaire with the help and assistance of their parents and blood samples taken on admission were studied for the presence of specific anti-TG Ig M, Ig G antibodies and Ig G avidity using enzyme immunoassay Platelia Toxo Ig M, Ig G. RESULTS: The study showed that 19 children(8 boys, 11 girls; 8-17 years old, mean age 14.36 years) hadhigh serum anti-TG Ig G antibody levels(range: 32.2 > 240 UI/m L, mean 120.18 UI/m L; positive value for Ig G was ≥ 9 UI/m L). The avidity index(AI) ranged from 0.202 to 0.925(scale: ≥ 0.5 high AI). The results for Ig M antibodies were all negative and the obtained results ranged from 0.113 to 0.25 U/m L(mean = 0.191 IU/m L) and all values below 0.8 IU/m L were considered negative. The most frequent complaints found in the seropositive patients were headaches that affected the frontal(13 children), occipital(4) and parietal areas(5). Headaches usually had a pulsating(in 7 patients) and squeezing(6) character and rarely were piercing, dull or expanding. Interestingly, 8 children did not feel discomfort during the headaches, probably because they did not have sufficiently increased intracranial pressure yet. The headaches usually appeared 1-2 times/mo, lasted for 2-6 h, and had a mean intensity of 5.5 points in a 10 point subjective scale. The comorbidities included epilepsy(5 patients), various infections in 3 children(chronic eustachitis, chronic rhinitis, chronic purulent tonsillitis, streptococcal pharyngitis, meningitis, allergic diseases), disturbances of behavior, deficits of attention, and ocular and motor concentration disorders in 1 child. The electroencephalographic and neuroimaging studies performed in our patients had a very limited value in establishing cerebral toxoplasmosis.CONCLUSION: Ten point six seven percent of the studied children had markedly increased serum anti-TG Ig G antibodies and high AI indicated chronic infestation. It is suggested that tests for TG infection should be introduced to routine diagnostics in patients with recurrent headaches.