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Objectives To study the course of West syndrome (WS) and coronavirus disease-19 (COVID-19) in children with WS who contracted SARS-CoV-2 infection. Methods This ambispective study was conducted at a tertiary-care center in North India between December 2020 and August 2021 after approval from the Institute Ethics Committee. Five children with WS, positive for COVID-19 based on RT-PCR, fulflled the inclusion criteria. Results One child with COVID-19 during the frst wave was retrospectively included while four children (of the 70 children screened) were prospectively enrolled. The median age at onset of epileptic spasms was 7 mo (2 boys), and that at presentation with COVID-19 was 18.5 mo. Three had underlying acquired structural etiology. Three were in remission following standard therapy, while two had ongoing spasms at the time of COVID-19 illness. During the illness, two of those in remission continued to be in remission while one child had a relapse. The children with ongoing epileptic spasms had variable course [one had persistent spasms and other had transient cessation lasting 3 wk from day 2 of COVID-19 illness, but electroencephalography (on day 8 of COVID-19 illness) continued to show hypsarrhythmia]. Fever was the most typical symptom (and sometimes the only symptom) of COVID-19, with a duration ranging from 1–8 d. Two children had moderate COVID-19 illness requiring hospitalization, while the rest had a mild illness. All the afected children had complete recovery from COVID-19. Conclusion The severity of COVID-19 illness in children with WS is often mild, while the subsequent course of WS is variable.
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Objective:To analyze the characteristics of stereoelectroencephalography (SEEG) in children with drug-resistant epileptic spasms (ES), and to explore the surgical strategy of children with spastic seizure under the guidance of SEEG.Methods:The clinical data of 156 children with ES who were preoperatively evaluated in the Department of Neurosurgery Ward 3, Tsinghua University Yuquan Hospital from January 2014 to December 2021 were retrospectively reviewed.All children were evaluated in the second stage of stereotactic electrode placement after a non-invasive preoperative evaluation.The characteristics of intracranial EEG, surgical strategy and prognosis were analyzed.Results:A total of 19 eligible children were included, involving 13 boys and 6 girls.The age of first onset and surgical age of them ranged 1 month to 4 years, and 2 years to 13 years, respectively.The SEEG was divided into 3 types in children with ES at the onset.Five children were SEEG type A, presenting with the focal seizure discharges at the beginning and a gradual propagation to widespread fast-wave bursts.Ten children were SEEG type B, presenting a focal leading spike followed by diffused fast-wave bursts.Four children were SEEG type C, presenting a diffuse fast wave rhythm onset.Although some electrode discharges appeared slightly " leading", they covered more than one brain region.After focal resection or thermocoagulation, 13/19 patients did not have the onset of seizures, and 5/19 and 8/19 were graded as SEEG type A, and B, respectively.During the intermittent period of SEEG attacks in children with SEEG type A and B, a significant phenomenon of focal epileptic discharge consistent with the onset of the attack was observed, and surgical removal of these areas effectively controlled spastic seizures.Conclusions:Epileptic spasms may be triggered by a focal neocortical discharge.Intracranial EEG showed that the focal seizure onset evolves into spasm or a focal " leading spike" is a good indicator of surgical prognosis.
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OBJECTIVES@#To study the factors influencing the short-term (28 days) efficacy of initial adrenocorticotropic hormone (ACTH) therapy for infantile epileptic spasms syndrome (IESS), as well as the factors influencing recurrence and prognosis.@*METHODS@#The clinical data were collected from the children with IESS who received ACTH therapy for the first time in the Department of Pediatric Neurology, Xiangya Hospital of Central South University, from April 2008 to January 2018 and were followed up for ≥2 years. The multivariate logistic regression analysis was used to evaluate the factors influencing the short-term efficacy of ACTH therapy, recurrence, and long-term prognosis.@*RESULTS@#ACTH therapy achieved a control rate of seizures of 55.5% (111/200) on day 28 of treatment. Of the 111 children, 75 (67.6%) had no recurrence of seizures within 12 months of follow-up. The possibility of seizure control on day 28 of ACTH therapy in the children without focal seizures was 2.463 times that in those with focal seizures (P<0.05). The possibility of seizure control on day 28 of ACTH therapy in the children without hypsarrhythmia on electroencephalography on day 14 of ACTH therapy was 2.415 times that in those with hypsarrhythmia (P<0.05). The possibility of recurrence within 12 months after treatment was increased by 11.8% for every 1-month increase in the course of the disease (P<0.05). The possibility of moderate or severe developmental retardation or death in the children without seizure control after 28 days of ACTH therapy was 8.314 times that in those with seizure control (P<0.05). The possibility of moderate or severe developmental retardation or death in the children with structural etiology was 14.448 times that in those with unknown etiology (P<0.05).@*CONCLUSIONS@#Presence or absence of focal seizures and whether hypsarrhythmia disappears after 14 days of treatment can be used as predictors for the short-term efficacy of ACTH therapy, while the course of disease before treatment can be used as the predictor for recurrence after seizure control by ACTH therapy. The prognosis of IESS children is associated with etiology, and early control of seizures after ACTH therapy can improve long-term prognosis.
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Child , Humans , Infant , Adrenocorticotropic Hormone/therapeutic use , Spasms, Infantile/drug therapy , Treatment Outcome , Seizures , Electroencephalography/adverse effects , Spasm/drug therapyABSTRACT
Resumen Recientemente la Liga Internacional contra la Epilepsia (ILAE) socializó la clasificación propuesta para síndromes epilépticos de inicio neonatal y hasta los primeros 2 años de edad, dividiéndolos en síndromes epilépticos autolimitados y las encefalopatías epilépticas y del desarrollo (DEEs). En esta revisión nos dedicaremos a las DEEs, definidas como trastornos donde existe deterioro del desarrollo relacionado tanto con la etiología subyacente independiente de la actividad epileptiforme como con la encefalopatía epiléptica. Estas incluyen en el período neonatal la encefalopatía epiléptica infantil temprana o síndrome de Ohtahara y la encefalopatía mioclónica temprana, ahora agrupadas bajo la denominación de encefalopatías epilépticas y del desarrollo infantil temprano (EIDEE). El síndrome de espamos epilépticos infantiles, la epilepsia de la infancia con crisis migratorias y el síndrome de Dravet forman parte de las encefalopatías de inicio en el lactante. La importancia del reconocimiento temprano de las encefalopatías epilépticas radica no solo en el control de las crisis epilépticas, sino en detener el deterioro intentando cambiar el curso de la enfermedad. Es fundamental conocer la etiología evitando medicamentos que puedan exacerbar las crisis y empeorar el curso, aplicando medicina de precisión así como identificando pacientes candidatos a cirugía temprana de epilepsia.
Abstract The International League Against Epilepsy (ILAE) recently socialized the proposed classification for epileptic syndromes of neonatal onset and up to the first 2 years of age, dividing them into self-limited epileptic syndromes and epileptic and developmental encephalopathies (DEEs). In this review we will focus on DEEs, defined as disorders in which there is developmental impairment related to both the underlying aetiology independent of epileptiform activity and epileptic encephalopathy. These include early infantile epileptic encephalopathy or Ohtahara syndrome and early myoclonic encephalopathy in the neonatal period, now grouped under the name of epileptic and early childhood developmental encephalopathies (EIDEE). Infantile epileptic spasms syndrome, childhood epilepsy with migratory crises and Dravet syndrome are part of the infant-onset encephalopathies. The importance of early recognition of epileptic encephalopathies lies not only in the control of epileptic seizures, but also in stopping deterioration by trying to change the course of the disease. It is essential to know the etiology, avoiding medications that can exacerbate seizures and worsen the course, applying precision m edicine as well as identifying candidate patients for early epilepsy surgery.
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Objective:To explore the clinical manifestations and surgical outcomes of pediatric epilepsy patients with epileptic spasms (ES) as the main form of seizure, so as to analyze the correlative factors with prognosis and improve the understanding of the operation and preoperative positioning for such patients.Methods:The clinical data of patients with ES who underwent surgery therapy from June 2014 to December 2015 in Pediatric Epilepsy Center, Peking University First Hospital were collected and retrospectively analyzed.Demographic characteristics, seizure forms, etiology, electroencephalogram (EEG), cranial magnetic resonance imaging (MRI), operative methods, pathological findings as well as surgical outcomes evaluated by Engel classification during follow-up of the subjects were collected.Correlative factors with the prognosis were explored by comparing the data between patients with optimal outcome (Engel Ⅰ) and those with poor outcomes (Engel Ⅱ-Ⅳ).Results:A total of 25 pediatric patients were enrolled, including 16 males (64.0%) and 9 females (36.0%). The age of onset was (0.81±0.68) years, the age at operation was (2.98±1.63) years, and the course of disease was (2.17±1.48) years.Besides, 84.0% (21/25 cases) of the ES patients had multiple forms of seizures and partial seizure (19 cases) was the most common.MRI of the heads of all the children showed definite lesions, including 11 patients (44.0%) with lesions limited to one brain lobe and 14 patients (56.0%) involving multiple brain lobes or hemisphere.The most common etiology was focal cortical dysplasia (13 cases), followed by intracranial developmental tumors (3 cases). All patients underwent resection surgery, including resection of lesion (3 cases), single brain lobe resection (9 cases), multiple brain lobe dissection (3 cases) and hemisphere dissection (10 cases). During a follow-up period of 4.0 to 5.5 years, 1 patient was lost.Among the remaining 24 cases, 18 (75.0%) cases achieved good outcomes and wee classified as EngelⅠ, 2 cases (8.3%) and 4 cases(16.7%) were classified as Engel Ⅱand Ⅳ, respectively.The univariate comparison between the good epilepsy prognosis group and the poor epilepsy prognosis group showed that, patients whose EEG abnormalities are consistent with the anatomical lesions during the inter ictal tend to have good prognosis( P=0.006). Conclusions:(1) Optimal therapeutic effects were observed in ES patients with definite lesions treated by surgical therapy.(2) Interictal EEG consistent with the lesion side may suggest a good prognosis for surgical treatment.(3) Structural causes should be screened as soon as possible if a patient with ES is drug-refractory and presents clues of focal origin.
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Epileptic spasms (ES)as an independent form of seizure,exist not only in infantile spasms but also in other epilepsy syndromes. Most ES start within 1 year of age and may also develop after 1 year of age. The onset of ES after 1 year is referred to as late onset epileptic spasms (LOS). The etiology,clinical characteristics,diagnosis,treat-ment and prognosis of LOS are different from infantile spasms and Lennox-Gastaut syndrome. Now,the progress in etiology,diagnosis and treatment of LOS were reviewed.
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Objective To summary the clinical characteristics of epileptic spasm(ES)that is the unique form of seizures in childhood. Methods A total of 149 patients with ES who visited our hospital from May 2009 to June 2015 were collected. The documents of clinical data, treatment and outcome were analyzed retrospectively. The statistical analysis of some clinical characteristics and prognosis of patients were performed. Results There were 97(65.1%) male and 52 (34.9%) female in the 149 patients. The onset age of ES was from 30 days to 42 months (median value was 6 months) including 17 patients more than 12 months.The peak age of onset was 4-7 months. One hundred and forty-seven patients manifested clustered spasm. One pair of twins showed single spasm.Other types of seizure were seen before ES onset in 13 (8.7%) patients, and after ES onset in 42 (28.2%) patients.Eighty-three patients (55.7%) were diagnosed as West syndrome (WS). There were 46 patients (30.9%) with cryptogenic cause, 103 patients (69.1%) diagnosed with symptomatic epilepsy, in which 69 patients were with a clear cause. The proportions of developmental delay in the cryptogenic and symptomatic patients were 74.5% and 92.2% after the onset of the ES. The degree of developmental delay was linearly related to the course of ES. The hyperarrhythmia of electroencephalogram (EEG) background was seen in 122 patients (81.9%), and there was no significant difference in hyperarrhythmia between different etiology groups or different onset age groups. The short-term efficiency of topiramate evaluated within one month was 30.5% in 59 newly diagnosed patients. The short-term effective rate of adrenocorticotropic hormone (ACTH) was 80.3% in total 76 patients, but single ACTH treatment was associated with an increased recurrent rate. Conclusion ES is a highly age dependent seizure type that mainly found in WS,which typically follows a EEG hyperarrhythmia and developmental delay. The treatment of ES is difficult. ACTH combined with effective antiepileptic drugs (AEDS) is currently the most reasonable drug treatment program.
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Objective To preliminarily study the efficacy and sfa ety of ketogenic diet( KD) adding treatment for epileptic spasm s in children.Methods The clinical features,efficacies,side effects and compli-ances of patients adding KD treatment in our hospital from July 2012 to December 2014 were analyzed. Results Totally 20 patients(10 months old to 8 years and 2 months old) of epileptic spasms with 14 boys and 6 girls were enrolled in the study.Among them 2 cases were less than 1 year old,10 cases were 1-3 years old,5 cases were 3-6 years old,and 3 casesw ere older thna 6 years.Twelve cases were cryptogenic epilepsy, 6 cases were symptomatic epilepsy(perian tal brain injury),1 case was brain malformation,and 1 case was severe viral encehp alitis sequela.Sp asm was the main episode of rm of all the cases.Amogn 20 caes s,7 cases were accompanied by some oteh r forms of episode, inlc uding secondarily generalized seizures, clonic seizures,tonic seizures,atypical absence seizures,and atypical absence status.The epileptic syndromes includ-ed infantile spasms or epileptic spasms(19 cases) and Lennox-Gastaut syndrome(1 case).The courses of disease were 11 days to 7 years and 9 months.One of them had severe liver function injury after oral antiepi-leptic medicine (11 days),and others were diagnosed intractable epilepsy.Overall,the KD dei t achieved the seizure-free state in 30%(6/20) of patients and a greater than 90% reduction of seizure frequency in one patient.In addition,12 patients got improvement in development of gross motor,while 6 of them had lingual developments.Severe side effects were not observed in follow-up studies.Eight cases com pleted the KD treat-ment for at least 6 months,12 cases stopped because of no efficacy,poor tolerance,diar hea and recrudesces. Conclusion KD adding treatment is probably an effective therapy for epileptic spasms with few side effects. While large samples and long-term observations are still desired to better recipes, and to provide possibly effective alternative to other therapies for epileptic spasms.
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Se realiza una revisión de las características electroencefalográficas de los espasmos infantiles, espasmos epilépticos o síndrome de West, y otras entidades relacionadas con este. Se enfatiza en los patrones más frecuentes, fundamentalmente en los 2 tipos de hipsarritmia: clásica y periódica o fragmentada, observados en el síndrome anteriormente mencionado. Se comenta en relación con el trazado de suppression-burst o paroxismos-supresión, y su correlación con los síndromes de Ohtahara, y Aicardi y Goutières, descritos respectivamente en 1976 y 1978. Se aclara que estos 2 patrones no son exclusivos de estos síndromes, y pueden ser observados en otras entidades en el neonato, como la encefalopatía anóxica isquémica, la meningitis neonatal bacteriana y trastornos metabólicos, entre otros.
A review was made on the encephalographic characteristics of infantile spasms, epileptic spasms, or West syndrome, and other related entities. Emphasis was made on the most frequent patterns, mainly the two types of hypsarrhythmia, classical and periodic, and fragmented, which are observed in this syndrome. Likewise, comments were made on the suppression-burst or burst-suppression tracing and its correlation with Ohtahara, and Aicardi and Goutières syndromes that were described in 1976 and 1978, respectively. It was clarified that these two patterns are not exclusive of these syndromes and may be also observed in other illnesses affecting the neonates such as anoxic-ischemic encephalopathy, neonatal bacterial meningitis and metabolic disorders, among others.
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Humans , Spasms, Infantile/diagnosis , Electroencephalography/methodsABSTRACT
Objective To explore the characteristics of etiology,clinical,electroencephalogram(EEG) and prognosis of infantile spasms(IS) with focal seizures(FS).Methods The significance of age onset,seizure patterns and atteration,etiology,video-EEG(VEEG) and evolution of FS correlating to epileptic spasms(ES),which occurred in 12 cases with IS by means of clinical observation,cranial CT or MRI,VEEG monitory and follow up were investigated.Twelve cases were divided into group A,B,C according to the stages of FS occurring prece-ding,coinciding and following ES.Results Ten cases with IS were identified from focal cortical dysplasia,tuberous scleroses complex,temporal lobe cyst or scleroses and hypoxic-ischemic encephalopathy and others.Clinical manifestation presented FS at certain times during the course of the disease,and other characteristics of frequent attacks,asymmetric spasms or tonic spasms,some atypical seizures in the eyes and the head,motionless staring and focal motor seizures and other.The correlation of ES to FS occurred during one ictal episode as follows:FS→ES(6 cases),ES→FS→ES(1 case),ES→FS(3 cases).FS appeared more frequently,atypical,predominantly involving ocular,facial,oral movement or generalized convulsion,migrating or alternating seizures,associated with epileptic discharges of posterior parietal-occipital and parietal-temporal-occipital origins in group A and early period of group B;whereas in the late period of group B and group C,FS occurred less frequently and more stable,presenting complex partial seizures or secondarily generalized seizures originated mainly from frontal and surrounding areas in the lateralized or bilateral hemispheres.Regarding the natural evolution and development of IS,2 cases with early FS developed into IS;6 cases with IS evolved into Lennox-Gastaut syndrome or symtomatic generalized epilepsy,4 cases got into FS,1 case maintained in a peculiarly epileptic state with FS as well as ES until the operation at 4.5 years old,and the other case was not identified clearly.Conclusions IS coincided with FS is a special kind of aged-related FS associated with secondarily generalized seizures and epileptic encephalopathy.The multiple etiology,seizure patterns,ictal-interictal EEG,clinical evolution and prognosis of the disorder,indicate a complicated interaction of the immature cortico-subcortical abnormalities in the critical developmental period and thereafter,can be identified as a peculiar form of clinical epileptic syndrome.