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06 January 2016, Volume 31 Issue 1 Previous Issue    Next Issue

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Children epilepsy center: A comprehensive multidisciplinary mode of diagnosis and treatment LIU Xiao-yan 2016, 31(1): 2.  DOI: 10.7504/ek2016010601 Abstract ( )   There are various pathogenesis and complex clinical manifestation in childhood epilepsies. The steroid hormone，ketogenic diet，surgery or nerve regulation could be  treatment choices if antiepileptic drug therapy fails，depending on individual conditions. At present many new technologies involving different field have be applied to diagnosis and therapy，especially the preoperative evaluation，including genes，structure and functional neruoimaging，neuroelectrophysiology，neuropsychological assessment and so on. Taking neurology，neurosurgery and neuroelectrophysiology as the core，and  through cooperation with neruoimaging， neurodevelopment，neuropsychology and other related departments，the children epilepsy center carries out diagnosis，treatment and management on patients with epilepsy in multidisciplinary and multimodel pattern. There are not enough experience and consensus about the use of some new technologies in childhood epilepsies now，so it needs to explore the clinical methods and management modes suitable for children epilepsy center.

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Anti-epileptic drug treatment strategies for children with intractable epilepsy YANG Lin，HUANG Shao-ping 2016, 31(1): 5.  DOI: 10.7504/ek2016010602 Abstract ( )   Intractable epilepsy often requires a combination of drugs. To reduce the seizures in children， minimize the side effects of drugs， and improve the patient’s quality of life， it is very important to be familiar with the main mechanisms and pharmacokinetics of antiepileptic drugs， and correctly choose drugs according to the types of seizure and syndrome. Also every time to add a new drug， it must consider the dosage and speed of titration of the drug to avoid serious adverse effects.

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Neuroimaging in the multimodel diagnosis and localization of epileptogenic foci and cortical functional area        YE Jin-tang 2016, 31(1): 10.  DOI: 10.7504/ek2016010603 Abstract ( )   Epilepsy is a clinically common disease of chronic nervous system in children，and refractory epilepsy accounts for 20% or so. Epilepsy surgery is the most effective treatment for refractory epilepsy. Children’s epilepsy has many similar characteristics with adults’epilepsy. But children’s epilepsy has obvious differences in etiology，imageology，electro-clinical characteristics and surgical methods，which is very important for the preoperative evaluation，especially for the surgical indication and timing. The discussion and decision should be made by the doctors of neurology，neurosurgery and other departments in the mature children’s epilepsy center.

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Neuroelectrophysiology in the multimodel assessment of the epileptogenic foci and cortical functional area QIAO Hui，TAO Xiao-rong，QI Lei 2016, 31(1): 14.  DOI: 10.7504/ek2016010604 Abstract ( )   Epilepsy affects the development of children’s physical and mental health seriously. Surgery is an advisable measure to control seizure for refractory epilepsy， and advances in epilepsy surgery have propelled its usage in pediatrics.Successful surgery highly depends on the accuracy of the preoperative evaluation and localization. Semiology，neuroimaging，neuroelectrophysiology are the main components of anatomo-electro-clinical system. Given the speciality of children’s nerve system，there are some criteria different from those of adults. The value of neuroelectrophysiology in preoperative evaluation of epilepsy and the matters ought to be paid attention to in pediatrics.

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Application of stereoelectroencephalography to children in epilepsy surgery ZHOU Wen-jing 2016, 31(1): 17.  DOI: 10.7504/ek2016010605 Abstract ( )   Stereoelectroencephalography（SEEG） is developing rapidly in China recently. The overview of the development，the indications，contraindications and complications in childhood epilepsy surgery，implantation techniques and concept of the epileptic zone，multimodel images processing techniques were reviewed and explained.

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Neuropsychological evaluation for epilepsy surgery and neural development assessment in children with epilepsy CHEN Qian 2016, 31(1): 22.  DOI: 10.7504/ek2016010606 Abstract ( )   The role of a neuropsychological assessment in the routine care of children with epilepsy is to provide anobjective information of cognitiveand psychological function,whichcan provide clues tothe lateralization or localization of the function lesion and the epileptic zone. It’s aspecifialjob to evaluate the neuropsychological status for children with epilepsy. The objective of this review is to introduce the neuropsycholoical assessment methods including a core set of cognitive and psychological domains that should beassessed to provide an objective account of intelligence quotient，attention，nonverbal cognitive function，executive function，somatosensory and motor function，memory function and Wada test，etc. Clinicians should be aware of the content of the core cognitive function screening.

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Electro-clinical features of children epilepsy and localization value in diagnosis SHAO Xiao-qiu 2016, 31(1): 26.  DOI: 10.7504/ek2016010607 Abstract ( )   Children，especially young children with epilepsy have  unique eletro-clinical characteristics that are different from those of adults. To fully understand the characteristics of epileptic seizures in childhood has an important value for proper classification and localization of epileptogenic zone. In addition，epilepsy in children with developmental and early acquired lesions usually present generalized patterns of seizures and epileptiform discharges，which is common in young children and may cause a delay surgical intervention that result in the children developmental delay of psychomotor. Being familiar with the electro-clinical features of epileptic pathology in children is crucial for selecting the appropriate surgical candidates.

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Surgical indications and timing for medically intractable epilepsy in children CAI Li-xin 2016, 31(1): 31.  DOI: 10.7504/ek2016010608 Abstract ( )   Epilepsy is a common chronic neurological disorder in children. Among them， about 20% are drug resistant. Epilepsy surgery is often the most effective treatment option for medically intractable epilepsy. Although there are many common features between pediatric and adult epilepsy surgery，there are also many differences unique to children in etiology， neuro-imaging， electrical-clinical features and operative techniques. These differences are critical during the many stages of the pediatric epilepsy surgery， especially for choosing correct surgical indications and timing. On the other hand， the presurgical discussion and decision should be best performed at a special pediatric epilepsy center with the input of a team of pediatric neurologists， surgeons and some other specialists.

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Use  of  corticosteroids  in  refractory  epilepsy PAN Gang，ZHOU Shui-zhen 2016, 31(1): 36.  DOI: 10.7504/ek2016010609 Abstract ( )   Epilepsy is a common neurological disease in children. Although most patients are seizure-free after treatment of antiepileptic drugs，a few patients have no response，called refractory epilepsy. Refractory epilepsy has huge influence on patients’ physical and mental development. It is necessary to find an alternative for them. As one of the drugs for epilepsy，corticosteroids have good effect on refractory epilepsy. The clinician should know the indications，dose and course of corticosteroids used in refractory epilepsy.

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Application  of  ketogenic  diet  therapy  in  refractory   epilepsy LIAO Jian-xiang 2016, 31(1): 41.  DOI: 10.7504/ek2016010610 Abstract ( )   The ketogenic diet（KD） has been used in the treatment of epilepsy in China mainland since 2004. Indications include glucose transporter protein 1（GLUT-1） deficiency，pyruvate dehydrogenase deficiency（PDHD）， myoclonus astatic epilepsy （Doose syndrome）， tuberous sclerosis complicated with epilepsy，Rett syndrome，Dravet syndrome，infant spasms，Landau Kleffner syndrome，Lafora disease，and super-refractory status epilepticus. The contraindications include fat metabolic pathway inborn errors，porphyrins diseases，and patients who are not able to cooperate with. The KD therapy complications are less，and by conservative treatment most are reversible. Transition from ordinary diet to KD often lasts 1—2 weeks， 2∶1—4∶1 diet can  produce ketosis of clinical therapeutic effect. It is recommended that KD might be tried at least for three months. Good responders should maintain the therapy for 2 years or so. It often takes 2 to 3 months for KD to turn back to normal diet. KD therapy should be combined with close follow-up and essessment，and it is considered safe in clinical practice.

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Neuromodulation in the treatment of childhood refractory epilepsy LIU Qiang-qiang，XU Ji-wen 2016, 31(1): 45.  DOI: 10.7504/ek2016010611 Abstract ( )   Epileptic surgery for children with refractory epilepsy can result in good clinical outcome. For those not suitable for surgery，the neuromodulation as one of treatment methods can be considered. With the development of neuromodulation，vagus nerve stimulation，deep brain stimulation and the responsive neurostimulator system now are available for clinic. The safety and effectiveness of those methods for refractory epilepsy have been confirmed by clinical research. At the same time，those technologies for children with intractable epilepsy also have a good effect. Rational selection and use of neuromodulation can improve clinical outcomes and quality of life in children with refractory epilepsy.

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Clinical features and follow-up studies of 84 children with multiple sclerosis ZHU Hong-min， BAO Xin-hua，ZHANG Yao 2016, 31(1): 51.  DOI: 10.7504/ek2016010612 Abstract ( )   Objective    To study the clinical features，treatment and prognosis of multiple sclerosis（MS） in children in order to improve the the diagnosis and treatment level. Methods    The clinical data of 84 MS children hospitalized from Nov. 1993 to Dec. 2014 in Department of Pediatrics，Peking University First Hospital were analyzed retrospectively and the follow-ups were summarized. Results    Main clinical manifestations included visual acuity decrease，weakness，headache， fever， encephalopathy and seizure. CSF oligoclonal band was positive in 22/78 cases （28.2%），and CSF myelin-oligodendrocytes glycoprotein antibody was positive in 16/49 cases （32.7%）. Lesions on MRI were frequently observed in subcortical white matter （92.9%） and ventricular white matter （86.9%）， then in cerebellum（47.6%） in brainstem （36.9%）， basal ganglia （29.8%）， thalamus （21.4%）， and corpus callosum （16.7%）. All cases were treated with glucocorticoids at acute stage，combined with intravenous immune globulin （IVIG） in 53 cases （63.1%）. Complete remission was achieved in 69 cases （82.1%） after treatment. The cases were followed-up for （0.7—22.0） years. The average relapse times were （4.71±3.18）. The annual relapse rate was （0.65±0.52） times/year. A total of 61 cases （72.6%） relapsed within the first year after the onset of MS. Fifty-seven cases were followed up to December 2014， and 17/57 cases （12.3%） had movement disorder， and 17/57 cases （29.8%） had visual acuity decrease， and 21/53 （39.6%） had learning disabilities. Conclusion    MS with early onset （younger than 10 years） is not rare. The common presentation symptoms include visual acuity decrease， weakness， headache， fever， encephalopathy and seizure. The positive rate of CSF oligoclonal bands is low. MRI shows the lesions are frequently located in subcortical and ventricular white matter. Glucocorticoids are effective in the acute period. Relapse usually occurrs in the first year after onset. DMT can prolong the relapse interval.Visual acuity decrease and learning disabilities are the common sequelae of the children with MS.

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Clinical features of clinically mild encephalitis/encephalopathy with a reversible splenial lesion of corpus callosum in children GAO Yang，ZOU Li-ping，WANG Jian-jun，WANG Jian-wen，QIU Jia-jing，SHEN Wen-wen 2016, 31(1): 56.  DOI: 10.7504/ek2016010613 Abstract ( )   Objective    To compare the etiology and clinical characteristics of clinically mild encephalitis/encephalopathy with a reversible splenial lesion of corpus callosum in Chinese children with those cases from different countries. Methods    The clinical and imaging features of two children with the diagnosis of MERS were analyzed and pediatric MERS cases were screened from “China National Knowledge Infrastructure”，“China Biology Medicine”，“PubMed ” and “Web of Science” databases for analysis between January，2000 and February，2015. Results    A total of 115 cases were collected and the male to female ratio was 1.1 to 1， with onset age of 6 months to 14 years old. There was usually a certain infection before the onset. About 58.3% of infectious pathogens were detected， and influenza virus and rotavirus infection were very common pathogens， followed by bacterial infections and mycoplasma pneumoniae infection. The neurological symptoms manifested as mental and behavioral disorders （42.6%）， seizures （37.4%）， decreased consciousness （5.2%） or consciousness（25.2%） and dysarthria （6.9%）. The MR imaging （MRI） findings exhibited lesions in an isolated splenial or the white matter and entire corpus callosum， and completely disappeared at follow-up MRI. With anti-infection and symptomatic treatments，the clinical symptoms of most patients were relieved without leaving any serious neurological sequelae. Conclusion    Infection is a prevalent predisposing factor of MERS in children， with nonspecific clinical characteristics. The MRI examination plays a role of implication for diagnosis. And all clinical symptoms of patients will disappear within a few days and the prognosis of patients is good.

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Case-control study on influencing factors of pathogenesis of autism spectrum disorders JI Yang，LIU Xiao-lin，YU Kuo 2016, 31(1): 60.  DOI: 10.7504/ek2016010614 Abstract ( )   Objective    Through the comparative analysis of influencing factor differences in the autistic spectrum disorders（ASD） children and healthy children， to further explore child autism factors and to provide reference for prevention and control of autism. Methods    A retrospective investigation with 1∶1 case-control study was conducted in 196 cases of ASD children and normal children diagnosed and treated in Jinzhou Women and Children’s Hospital between May 2014 and May 2015；through the home-made questionnaire for health factors，the differences between the two groups concerning development indicators and health influencing factors were compared. Results    There was no statistical difference in the average height and weight between the two groups（P＞0.05），but the size of head circumference of ASD group was greater than the control group，and the difference was statistically significant（t＝2.41， P＝0.02）.Conditional logistic regression analysis revealed there was statistical difference（all P＜0.05） in children’s ability of a glimpse of recognition and understanding （β＝-3.077） and head circumference （β＝0.341）. The test of regression coefficient of some variables showed that mother’s age older than 35 years old，family relationship，family history of neurological disorder and father engaged in manual labour had statistical significance，and were significantly correlated with the occurrence of diseases（all P＜0.05）. Some factors had positive relationship with the happening of the disease（all P＜0.05），namely irritable mood during pregnancy （β＝1.966， OR＝7.144），mental stimulation （β＝2.067， OR＝7.902） and pregnancy vomiting （β＝ 2.576， OR＝13.138）， but supplements （β＝ -2.435， OR＝0.088） and the normal quickening （β＝-2.008， OR＝0.134） were negatively associated with ASD （all P＜0.05）. Conclusion    The factors such as mother's age more than 35 years old， father engaged in mental labour， family tensions， family history of nervous disorders， mental stimulation， emotional irritability and pregnancy vomiting， may be the risk factors for ASD. If mother is pregnant before the age of 35， has supplements timely and keep happy during pregnancy，and in the process of children’s growth，parents pay attention to development of language and head circumference， and ofen accompany children， the occurrence of ASD can be reduced to a certain extant.

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Clinical and eletrophysiological features of non-convulsive status epilepticus in children with viral encephalitis YU Xiong-ying，YI Zhao-shi，ZHA Jian，et al 2016, 31(1): 64.  DOI: 10.7504/ek2016010615 Abstract ( )

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Progress in the treatment for systemic lupus erythematosus in children DENG Jiang-hong，LI Cai-feng 2016, 31(1): 67.  DOI: 10.7504/ek2016010616 Abstract ( )

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Report and gene analysis of Dravet syndrome in twins NI Yan，ZHANG Lin-mei，ZHOU Yuan-feng，et al 2016, 31(1): 72.  DOI: 10.7504/ek2016010617 Abstract ( )

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Methylmalonic aciduria combined with homocystinemia with abnormal psychological behavior as the first-onset manifestation：A report of 1 case DAI Gang-xu，ZHANG Yao，MEN Xi，et al 2016, 31(1): 75.  DOI: 10.7504/ek2016010618 Abstract ( )

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Mistaking of clonazepam in children：A report of 3 cases ZHUO Xiu-wei，DING Chang-hong，ZHANG Wei-hua 2016, 31(1): 78.  DOI: 10.7504/ek2016010619 Abstract ( )