ABSTRACT
Infants suffering from angiostrongylus eosinophilic meningitis (AEM) is rare, while AEM can cause severe consequences.The diagnostic value of high-throughput sequencing for AEM was studied by analyzing 2 AEM children (< 2 years old) in the Department of Neurology, Shenzhen Children′s Hospital in 2019.Case 1 mainly pre-sented intermittent fever, vomiting, mental fatigue and bregma bulge.Case 2 mainly manifested intermittent fever, cough, vomiting and convulsion.Due to hypereosinophils in patients′ peripheral blood and cerebrospinal fluid (CSF), and abundant DNA sequences from a cantonensis in CSF and positive antibody test, the patients were diagnosed with AEM.The patients were treated with albendazole to deworm, and small doses of methylprednisolone to reduce inflammation.The clinical characteristics of AEM infant are not typical, and high-throughput sequencing technology can assist the diagnosis of AEM.
ABSTRACT
Epilepsy is one of the major clinical features of tuberous sclerosis complex, and it is drug-resistant in the majority of cases.Surgical resection is an effective way to resolve the seizures.Precise preoperative evaluation is critical to the surgical outcome.Preoperative evaluation mainly aims to determine the range of the epileptogenic zone and the functional areas that should be preserved.Because of the complexity of the epileptogenic mechanism and brain network, there isn′t a single and specific measure that can accurately position the epileptogenic zone, so it is necessary to comprehensively evaluate and localize the epileptogenic zone by using multiple methods, including collection of a detailed medical history, symptomatic analysis during the attack of seizures, magnetic resonance imaging, positronemission tomography, electroencephalogram, neuropsychological evaluation, etc.In this paper, the rational use of above-mentioned approaches and comprehensive analysis of their results were summarized, which play an essential role in contro-lling seizures in children with tuberous sclerosis complex and refractory seizures.
ABSTRACT
Objective To investigate the efficacy and safety of ketogenic diet (KD) and antiepileptic drugs (AEDs) in the children with drug refractory Dravet syndrome (DS).Methods Thirty-two cases of drug refractory DS were enrolled into the Department of Neurology,Shenzhen Children's Hospital Affiliated to Shantou University Medical School from July 2016 to December 2017,and they were divided into 2 groups:KD group and AEDs group (16 cases for each group),respectively.KD was added to as an additional therapy for KD group,and oral AEDs were administered only in AEDs group.In KD group,oral AEDs were not adjusted for the first 3 months.AEDs could be adjusted within a limited range in 2 groups after 3 months.The clinical efficacy,improvement of cognitive function,retention rate and side effects were observed and compared after 3,6,12 months of treatment.The average monthly seizure frequency within 3 months before enrollment was recorded as the baseline.The clinical efficacy was assessed by comparing the seizure frequency of each observation period with the baseline.Results In KD group,after 3,6,12 months' follow-up,KD therapy was maintained in 15,14,12 patients.The number of patients whose seizure reduction over 50％ was 10,12,11 cases,respectively.The number of patients whose seizure reduction over 90％ was 7,9,10 cases,respectively.The number of patients who were seizure free was 3,6,8 cases,respectively.In AEDs group,after 3,6,12 months' therapy,the number of patients whose seizure reduction over 50％ was 6,7,8 cases,respectively,the number of patients whose seizure reduction over 90％ was 3,3,4 cases,respectively.The number of patients who were seizure-free was 2,1,2 cases,respectively.There was a significant difference in the seizure reduction between 2 groups after 6,12 months (P ＜ 0.05).Furthermore,the incidence of status epilepticus (SE) was significantly reduced in KD group,and non-fever related status epilepticus (NFSE) was preferentially improved.There was a significant difference in the incidence of SE between before and after treatment in the KD group (P ＜ 0.05).After 12 months,there was a significant difference in the incidence of SE between 2 groups (P ＜ 0.05).After 6,12 months of treatment,the patients in KD group had significant improvements in adaption,gross motor and language quotients by Gesell Developmental Scale compared to the AEDs group (all P ＜ 0.05).Eleven of 12 children who adhered to the therapy for 1 year in KD group had improvement of developmental quotient ≥ 1 grade,however,7 cases of 16 children in AEDs group had this improvement.The incidence of adverse effect in the KD group and the AEDs group was 37.5％ (6/16 cases) and 56.3％ (9/16 cases),respectively,and the difference was not significant (P ＞ 0.05).Conclusions KD can not only reduce seizure frequency and relieve SE,but also improve the cognitive function of drug refractory DS.The adverse reaction ratio of KD does not increase significantly compared to AEDs.Therefore,KD is effective and safe therapy for children with drug-resistant DS.
ABSTRACT
Objective@#To investigate the efficacy and safety of ketogenic diet (KD) and antiepileptic drugs(AEDs) in the children with drug refractory Dravet syndrome (DS).@*Methods@#Thirty-two cases of drug refractory DS were enrolled into the Department of Neurology, Shenzhen Children′s Hospital Affiliated to Shantou University Medical School from July 2016 to December 2017, and they were divided into 2 groups: KD group and AEDs group (16 cases for each group), respectively.KD was added to as an additional therapy for KD group, and oral AEDs were administered only in AEDs group.In KD group, oral AEDs were not adjusted for the first 3 months.AEDs could be adjusted within a limited range in 2 groups after 3 months.The clinical efficacy, improvement of cognitive function, retention rate and side effects were observed and compared after 3, 6, 12 months of treatment.The average monthly seizure frequency within 3 months before enrollment was recorded as the baseline.The clinical efficacy was assessed by comparing the seizure frequency of each observation period with the baseline.@*Results@#In KD group, after 3, 6, 12 months′ follow-up, KD the-rapy was maintained in 15, 14, 12 patients.The number of patients whose seizure reduction over 50% was 10, 12, 11 cases, respectively.The number of patients whose seizure reduction over 90% was 7, 9, 10 cases, respectively.The number of patients who were seizure free was 3, 6, 8 cases, respectively.In AEDs group, after 3, 6, 12 months′ therapy, the number of patients whose seizure reduction over 50% was 6, 7, 8 cases, respectively, the number of patients whose seizure reduction over 90% was 3, 3, 4 cases, respectively.The number of patients who were seizure-free was 2, 1, 2 cases, respectively.There was a significant difference in the seizure reduction between 2 groups after 6, 12 months (P<0.05). Furthermore, the incidence of status epilepticus (SE) was significantly reduced in KD group, and non-fever related status epilepticus (NFSE) was preferentially improved.There was a significant difference in the incidence of SE between before and after treatment in the KD group (P<0.05). After 12 months, there was a significant difference in the incidence of SE between 2 groups (P<0.05). After 6, 12 months of treatment, the patients in KD group had significant improvements in adaption, gross motor and language quotients by Gesell Developmental Scale compared to the AEDs group (all P<0.05). Eleven of 12 children who adhered to the therapy for 1 year in KD group had improvement of developmental quotient ≥ 1 grade, however, 7 cases of 16 children in AEDs group had this improvement.The incidence of adverse effect in the KD group and the AEDs group was 37.5%(6/16 cases) and 56.3%(9/16 cases), respectively, and the difference was not significant (P>0.05).@*Conclusions@#KD can not only reduce seizure frequency and relieve SE, but also improve the cognitive function of drug refractory DS.The adverse reaction ratio of KD does not increase significantly compared to AEDs.Therefore, KD is effective and safe therapy for children with drug-resistant DS.
ABSTRACT
Dravet syndrome (DS) is an epileptic (developmental) encephalopathy which onsets in infancy,most DS children are drug resistant.However,the emergence of new antiepileptic drugs is providing more options to treat DS.In the recent years,the efficacy of nonpharmacologic therapies (such as neurostimulation and ketogenic diet) had been also confirmed in DS.Now,the latest progress on clinical treatment of DS was elaborated.Besides that,the therapies on neuropsychological damages and how to prevent and deal with the status epilepticus and sudden unexpected death in children with DS were briefly introduced.
ABSTRACT
High-frequency oscillations are used more and more widely on localizing epileptogenic zone and evaluating outcome as the surgery on epilepsy cases are increasing and the technique of electroencephalogram (EEG) are being improved.High-frequency oscillations are recorded by intracranial electrodes on most previous studies.However,recent reports describing high-frequency oscillations on scalp EEG recordings have created significant interest.Scalp high-frequency EEG provides a safe,non-invasive and simple method for us to study the special brain electrical activity.This paper summary the clinical applications and some questions on scalp high-frequency EEG.