Antiseizure medication treatment outcomes in new-onset pediatric epilepsy.

BACKGROUND: Antiseizure medications (ASMs) are the primary treatment for epilepsy; however, some prospective cohort studies in adults suggested that the efficacy of the third and subsequent ASM treatments are poor. Thus, we aimed to assess the outcomes of ASM treatment in new-onset pediatric epilepsy. METHODS: We retrospectively studied 281 pediatric patients diagnosed with epilepsy, in which the first ASM was prescribed between July, 2015, and June, 2020, at Hiroshima City Funairi Citizens Hospital. We reviewed their clinical profiles and seizure outcomes at the end of the study in August, 2022. Seizure freedom was defined as having no seizures for the previous 12 months or longer. RESULTS: Age at the onset of epilepsy ranged from 22 days to 186 months (mean: 84 months). The most frequent classifications of the types and syndromes of epilepsy were focal epilepsy (n = 151, 53.7%), followed by generalized epilepsy (n = 30, 10.7%), and self-limited epilepsy with centrotemporal spikes (n = 20, 7.1%). During the first ASM regimen, 183 out of the 281 (65.1%) patients became seizure free. During the second ASM regimen, 47 out of the 92 (51.1%) patients became seizure free. Only 15 out of the 40 (37.5%) patients who tried the third and subsequent ASM regimen became seizure free, while none became seizure free after the sixth and subsequent ASM regimen. CONCLUSIONS: The efficacy of ASM treatment after the third and subsequent regimen was poor in children, as well as in adults. It is important to reconsider whether there are indications for treatments other than ASM.

[A case suspected Sensory Guillain-Barré syndrome subsequent to Campylobacter jejuni enteritis].

A 9-year-old girl was admitted to our hospital with severe plantar pain, 7 days after the onset of Campylobacter jejuni enteritis. On admission, extremity strength and the deep tendon reflex were normal; however, there was difficulty in walking owing to plantar pain. Motor nerve conduction test showed no abnormalities. No spinal cord protein cell dissociation. Lumbar spine-enhanced MRI showed a 4th and 5th lumbar vertebrae nerve root contrast-enhanced effect. Gabapentin was effective in minimizing her pain, eventually enabling the patient to walk. Antiganglioside antibody tests on admission showed multiple positive results. Six months after the initial onset of symptoms, she had recovered completely. She was suspected with sensory Guillain-Barré syndrome (GBS). GBS subsequent to Campylobacter jejuni enteritis has been recognized as an acute motor axonal neuropathy; hence, this report is considered to be valuable.

Efficacy of long-term adrenocorticotropic hormone therapy for West syndrome: A retrospective multicenter case series.

OBJECTIVES: Long-term adrenocorticotropic therapy (LT-ACTH), which consisted of 2-4 weeks of daily injections of adrenocorticotropic hormone (ACTH) and subsequent months of weekly injections, was tried for relapsed West syndrome (WS) or other intractable epilepsies in small case reports. Our aim was to explore the efficacy of LT-ACTH for preventing WS relapse, as well as the prevalence of its adverse events. METHODS: This is a retrospective, nationwide, multicenter case series of patients with WS who underwent LT-ACTH. Clinical information of the patients and protocol of LT-ACTH were collected from participating institutes in this study. We defined clinical response to ACTH as achievement of hypsarrhythmia and epileptic spasms resolution. Patients who responded to daily ACTH injections were identified and assessed whether they experienced WS relapse during/after the weekly ACTH injection period. The outcome was measured by the nonrelapse rate at 24 months after daily ACTH injections using the Kaplan-Meier method. RESULTS: Clinical information of 16 children with WS was analyzed. The median age at LT-ACTH initiation was 14.5 months (range: 7-68 months). Thirteen (81%) patients had previously undergone conventional ACTH treatment. The LT-ACTH regimens comprised a median of 16 days of daily injections (range: 11-28 days) and 10 months of weekly injections (range: 3-22 months). Seven patients experienced WS relapse during/after subsequent weekly ACTH period, and the nonrelapse rate at 24 months after daily injections was estimated at 60.6% (95% confidence interval: 32.3%-80.0%). Height stagnation, hypertension, and irritability were observed; lethal adverse events were not reported. SIGNIFICANCE: Our study firstly explored the efficacy of LT-ACTH for preventing WS relapse. LT-ACTH might be a treatment option for patients with relapsed or intractable WS; however, we note that our study is limited by its small sample size and the lack of an appropriate control group.

Seizure focus in the frontal interhemispheric fissure leads to ipsilateral isolated eye deviation.

OBJECTIVE: Few studies have examined the localization of seizures presenting with ictal eye deviation (ED) in the absence of other motor symptoms. We aimed to investigate differences in the localization of the ictal onset zone (IOZ) between patients with isolated ED and those with ED plus head turning (HT) during focal seizures. METHODS: We reviewed intracranial video-EEG data for 931 seizures in 80 patients with focal onset epilepsy in whom the IOZ could be confirmed. The 233 seizures in 49 patients with ED were classified into two semiological groups based on initial ED and the presence/absence of HT: (1) isolated ED (i.e., ED without HT), and (2) ED + HT (i.e., ED with HT). We analyzed the localization and lateralization of IOZs in each semiological group. We performed multivariate logistic regression analysis using a mixed-effects to determine the associations between IOZs and isolated ED/ED + HT. RESULTS: A total of 183 IOZs in 24 patients were included in the isolated ED group, while a total of 143 IOZs in 31 patients were included in the ED + HT group. Sixty-eight IOZs of eight patients in the isolated ED group were located in the ipsilateral frontal interhemispheric fissure (F-IHF). Only ipsilateral F-IHF was significantly associated with isolated ED (odds ratio [OR], 2.43; 95% confidence interval [CI], 0.37-4.49; P = 0.021). The contralateral lateral frontal cortex (latF) (P = 0.007) and ipsilateral mesial temporal region (mT) (P = 0.029) were significantly associated with ED + HT. CONCLUSION: The present study is the first to demonstrate that seizures with an F-IHF focus tend to present with initial ipsilateral isolated ED. This finding may aid in identifying the seizure focus in patients with isolated ED prior to resection.

Possible Role of High-Dose Barbiturates and Early Administration of Parenteral Ketogenic Diet for Reducing Development of Chronic Epilepsy in Febrile Infection-Related Epilepsy Syndrome: A Case Report.

We describe the efficacy of high-dose barbiturates and early administration of a parenteral ketogenic diet (KD) as initial treatments for acute status epilepticus (SE) in an 8-year-old girl with febrile infection-related epilepsy syndrome (FIRES). The patient was admitted to our hospital with refractory focal SE. Abundant epileptic discharges over the left frontal region were observed on electroencephalogram (EEG). Treatment with continuous infusion of thiamylal for 4 hours, increased incrementally to 40 mg/kg/h, successfully ended the clinical SE, and induced a burst-suppression coma. The infusion rate was then gradually decreased to 4 mg/kg/h over the next 12 hours. Parenteral KD was administered from days 6 to 21 of illness. Continuous infusion of thiamylal was switched to midazolam on day 10 without causing seizures or EEG exacerbations. The patient has remained seizure free in the 15 months since hospital discharge. The effectiveness of KD for the treatment of FIRES has attracted attention amongst clinicians, but KD treatment may need to last for 2 to 4 days before it can stop SE, a time period that could cause irreversible brain damage. Considering the severity of SE in our patient and the dose of barbiturates needed to treat it, we consider this case to have had a good clinical outcome. The results suggest that rapid termination of seizure using high-dose barbiturates in conjunction with early administration of parenteral KD could reduce the development of chronic epilepsy in patients with FIRES.

Case Report: Four Cases of Panayiotopoulos Syndrome Evolving to Juvenile Myoclonic Epilepsy.

Panayiotopoulos syndrome (PS) is a self-limited focal epilepsy appearing in childhood. Seizures in PS are self-limiting and do not usually continue into adulthood. Juvenile myoclonic epilepsy (JME) is the most common type of idiopathic generalized epilepsy, developing around puberty and continuing throughout adulthood. We describe four cases of PS in childhood in which JME developed in adolescence. Age at onset ranged from 4 to 8 years for PS, and 11 to 14 years for JME. JME developed after PS subsided, with the interval between last PS seizure and first JME seizure ranging from 1 to 10 years. No link between the two conditions has previously been described. Since PS is considered to show good prognosis and to be self-limiting, long-term observation has been considered unnecessary. No definitive factors were found to predict future evolution to JME in our series, so longer-term follow-up may be warranted for all PS patients.

Hemispherotomy can cause post-operative strabismus.

BACKGROUND: Hemispherotomy, which involves disconnecting hemispherical fibers, is a treatment option for medically intractable epilepsy. As various neurological disorders can cause strabismus, we hypothesized that hemispherotomy can cause post-operative strabismus in patients with medically intractable epilepsy. METHODS: Nineteen patients underwent the Hirschberg test before and after hemispherical disconnection surgery. Among the 19 patients, 16 patients (six females and 10 males; mean age, 12.2 years; range, 0.17-43 years) who underwent hemispherotomy were included in this study. RESULTS: The difference in the angle between the left and right eyes was significantly widened (p = 0.025). Nine (56%) of 16 patients exhibited post-operative chronic strabismus as evaluated with the Hirschberg test. Intermittent strabismus was noticed by family members or caregivers in 10 (63%) of 16 patients. Patients older than 12 years did not show post-operative strabismus as evaluated by the Hirschberg test. CONCLUSION: Hemispherotomy can cause or worsen post-operative strabismus in pediatric patients.

Three Cases of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome With Focal Cortical Dysplasia Type IIId.

Hemiconvulsion-hemiplegia-epilepsy syndrome (HHES) is a subset of acute encephalopathy characterized by infantile-onset with acute hemiconvulsive febrile status and subsequent unilateral cerebral atrophy and hemiparesis. In the chronic phase, patients with HHES develop epilepsy, typically displayed as intractable focal seizures. The patients are often intractable with antiepileptic drugs and need surgical treatment. Although viral encephalitis and genetic abnormalities are presumed to be the underlying etiology, the pathogenesis remains mostly unknown. We describe three cases of successful functional hemispherotomy for intractable epilepsy in HHES. Patients developed acute asymmetrical convulsive status following viral infections during the ages of 17-30 months. Their seizures were intractable with antiepileptic drugs and required hemispherotomy. On the basis of the pathological findings, all cases were diagnosed as focal cortical dysplasia (FCD) type IIId. The epileptogenic mild cortical malformations may be the cause of HHES.

Symmetry of ictal slow waves may predict the outcomes of corpus callosotomy for epileptic spasms.

We aimed to analyse the ictal electrographic changes on scalp electroencephalography (EEG), focusing on high-voltage slow waves (HVSs) in children with epileptic spasms (ES) and tonic spasms (TS) and then identified factors associated with corpus callosotomy (CC) outcomes. We enrolled 17 patients with ES/TS who underwent CC before 20 years of age. Post-CC Engel's classification was as follows: I in 7 patients, II in 2, III in 4, and IV in 4. Welch's t-test was used to analyse the correlation between ictal HVSs and CC outcomes based on the following three symmetrical indices: (1) negative peak delay: interhemispheric delay between negative peaks; (2) amplitude ratio: interhemispheric ratio of amplitude values for the highest positive peaks; and (3) duration ratio: interhemispheric ratio of slow wave duration. Ages at CC ranged from 17-237 months. Four to 15 ictal EEGs were analysed for each patient. The negative peak delay, amplitude ratio and duration ratio ranged from 0-530 ms, 1.00-7.40 and 1.00-2.74, respectively. The negative peak delay, amplitude ratio and duration ratio were significantly higher in the seizure residual group (p = 0.017, <0.001, <0.001, respectively). Symmetry of ictal HVSs may predict favourable outcomes following CC for ES/TS.

A Case of Kawasaki Disease Accompanied by Encephalitis and Several Kinds of Arrhythmia during the Acute Phase.

Although central nervous system complications occasionally accompany during the acute phase of Kawasaki disease, clinically problematic arrhythmia is quite rare. We report a case accompanied by encephalitis and several kinds of problematic arrhythmia. Following the diagnosis of Taussig-Bing anomaly and coarctation of the aorta, the patient underwent aortic arch reconstruction, an arterial switch operation, and ventricular septal defect closure. No significant arrhythmias were observed. At the age of 5 years, the patient presented with a fever, rash, conjunctival hyperemia, and redness of the lips and fingertips. He was subsequently diagnosed with Kawasaki disease. The patient also presented with disorientation, and electroencephalography revealed overall slow-wave activity, indicating encephalitis. The patient received high-dose immunoglobulin and steroid pulse therapy. Sinus arrest was detected on day 10, and an atrial flutter with a 2 : 1 to 4 : 1 atrioventricular conduction block occurred on day 20. Although cardioversion succeeded in alleviating the atrial flatter, the patient experienced significant sinus arrest. The sinus arrest was alleviated 3 days later. Kawasaki disease-induced vasculitis and the arterial switch operation may both have influenced the sinus node dysfunction. Although sinus node function recovered, the possibility of progression into the sinus node dysfunction in the future should be considered.

Low Body Mass Index and Low Intelligence Quotient Are Infection Risk Factors in Vagus Nerve Stimulation.

BACKGROUND: Risk factors for infection after vagus nerve stimulation (VNS) device implantation represent an important issue but remain unclear. We hypothesized that specific risk factors for infection would be associated with VNS device implantation. This study reviewed patients with epilepsy who underwent VNS device implantation and undertook a statistical analysis of risk factors for surgical site infection (SSI). METHODS: We reviewed all medical records for patients who underwent VNS therapy in our facility between August 2011 and May 2018. Age, sex, height, body weight, body mass index (BMI), intelligence quotient (IQ), surgical incision opening time, blood loss, epilepsy classification, activities of daily living, and generator replacement were statistically compared between cases with and without SSI. RESULTS: We performed 208 VNS device implantation surgeries at our facility during the study period. Among these, 150 patients underwent initial implantation, 56 patients underwent first generator replacement, and 2 patients underwent second replacement. Six patients (2.7%) with initial implantation and 3 patients (5.4%) with first replacement showed SSI. Low BMI was a risk factor for infection at initial implantation (P < 0.0012) using a BMI within 1.78 kg/m2 of the cutoff for being underweight (100% sensitivity, 25% specificity). Low IQ (P = 0.0015) was also a risk factor for SSI. CONCLUSIONS: This study identified low BMI and low IQ at initial implantation as risk factors for infection.

[The Efficacy of Everolimus for Refractory Seizures in Childhood Onset Epilepsy with Tuberous Sclerosis Complex].

Everolimus is a mammalian target of rapamycin (mTOR) inhibitor that has cytoreductive effects on subependymal giant cell astrocytoma and renal angiomyolipoma in tuberous sclerosis complex (TSC). Recent studies have also shown its efficacy against refractory seizures in TSC. We investigated the efficacy of everolimus in nine patients with TSC, who were admitted to the TSC clinic in Seirei Hamamatsu General Hospital and who suffered from refractory seizures. At the start of treatment, patients ranged from 1 month to 23 years of age, and were refractory to a mean of 5.4 antiepileptic agents. Main seizures were focal in six patients and generalized in three patients. After 0.5 to 4.0 years (mean=2.4 years), three patients (33%) were seizure-free and two patients (22%) experienced >90% reduction in seizures. Everolimus may therefore be effective in the treatment of refractory seizures in TSC. (Received February 20, 2019; Accepted April 2, 2019; Published June 1, 2019).

Analysis for the Association Between Corpus Callosum Thickness and Corpus Callosotomy Outcomes for Patients With Epileptic Spasms or Tonic Spasms.

BACKGROUND: This retrospective study is designed to determine whether the thickness of the corpus callosum can predict corpus callosotomy outcome in pediatric patients with epileptic or tonic spasms. METHODS: We retrospectively studied 25 patients (18 boys) with intractable childhood-onset epileptic or tonic spasms who underwent corpus callosotomy between March 2008 and January 2017. Seizure outcomes were classified as favorable (class I and II of Engel's outcome classification) or unfavorable (class III and IV of Engel's outcome classification) at 12 months postoperatively. We measured the corpus callosum area on the midline and maximum cerebral area on the para-midline in sagittal magnetic resonance images just before surgery. We statistically analyzed the associations between surgical outcomes and corpus callosum area, corpus callosum area/maximum cerebral area (corpus callosum/cerebrum ratio), or age at magnetic resonance imaging just before surgery, using univariate and multivariate logistic regression analyses. RESULTS: Age at surgery ranged from six to 237 months (mean: 119). Main seizure types were epileptic spasms in 17 patients and tonic spasms in eight. Favorable outcomes occurred in 10 (40%) patients and unfavorable outcomes in 15 (60%). Both corpus callosum area and corpus callosum/cerebrum ratio did not show significant associations with the outcomes in the univariate and multivariate analyses. The 95% confidence intervals of corpus callosum/cerebrum ratio strongly overlapped between the favorable and unfavorable outcome groups. CONCLUSIONS: Our data failed to support that corpus callosum thickness on the sagittal image is associated with corpus callosotomy outcomes in pediatric patients with epileptic spasms or tonic spasms.

Side Slit Guide Pipe for Precise Placement of Depth Electrodes.

BACKGROUND: Using a stereotactic technique, surgeons can accurately place a depth electrode (DE), but sometimes the DE deviates from the intended target due to movement of the electrode or leakage of cerebrospinal fluid when placing the electrode. If DEs can be anchored before removing the catheter insertion guide pipe, more accurate placement may be possible. METHODS: We made a side slit guide pipe. When the DEs were anchored to the dura or the edge of the burr hole, the DE did not move when the guide pipe was removed. We measured the distance between the planned target and the tip of the electrode in 13 patients (3 female and 10 male patients; age range, 7-43 years; mean age 23.0 years; median age 27 years) with medically intractable epilepsy who underwent DE placement with stereotactic neuronavigation guidance. RESULTS: There were 30 DEs implanted. The mean distance from the planned target to the tip of the DE was 0.570 mm (range, 0.3-1.2 mm; median 0.5 mm; SD 0.212). The mean distance from the planned target to the tip of the DE with dural anchoring was 0.467 mm (range, 0.3-0.6 mm; median 0.45 mm; SD 0.121) and with burr hole edge anchoring was 0.596 mm (range, 0.3-1.2 mm; median 0.50 mm; SD 0.224; P = 0.205). CONCLUSIONS: DEs can be anchored using the side slit guide pipe for more precise placement.

Successful corpus callosotomy for post-encephalopathic refractory epilepsy in a patient with MECP2 duplication syndrome.

BACKGROUND: Patients with MECP2 duplication syndrome present with distinct facial anomalies and clinical features such as global developmental delay, recurrent respiratory infections, and epileptic seizures. Approximately half of all patients develop epileptic seizures which are refractory in most cases despite active medical management. Furthermore, no previous reports have discussed the efficacy of surgical treatment for seizures in patients with MECP2 duplication syndrome. CASE REPORT: In the present report, we describe a case of MECP2 duplication syndrome in a 15-year-old boy who developed epileptic seizures following influenza-associated acute encephalitis. His frequent epileptic spasms, tonic, atonic, and partial seizures were refractory to multiple antiepileptic medications. Electroencephalography revealed continuous diffuse epileptic discharge, resulting in regression. A total corpus callosotomy (CC) was performed at the age of 14 years and 7 months. His seizures markedly decreased following CC, although he continued to experience brief partial seizures approximately once per month. Post-operative examination revealed that his epileptic discharges had disappeared, and that his developmental state had returned to pre-encephalopathy levels. CONCLUSION: Our findings indicate that CC may represent a valuable surgical option for children with medically refractory generalized seizures following acute encephalopathy, irrespective of genetic disorders such as MECP2 duplication syndrome.

Short duration of focal onset awareness and impaired awareness seizure are characteristic features of epilepsy in elderly patients.

PURPOSE: Diagnosing epilepsy in the elderly population can be difficult due to mimicking symptoms. Furthermore, epileptic symptoms can also be masked by various symptoms. We hypothesized that elderly patients with epilepsy exhibit specific clinical features among the various symptoms. PATIENTS AND METHODS: From 2009 to 2017, 177 patients who were older than 65 years were referred to our epilepsy center. Out of this group, the onset of symptoms occurred after reaching the age of 50 years in 152 of the patients, who were additionally being treated at our clinic. We divided their symptoms in accordance with their consciousness levels, which were defined as follows: full wakefulness level I, impaired awareness level II, and loss of consciousness level III. We also classified the duration of the symptoms as <10 seconds, ≥10 seconds but <1 minute, ≥1 minute but <5 minutes, ≥5 minutes but <10 minutes, ≥10 minutes but <1 hour, and ≥1 hour. RESULTS: Among the 152 patients analyzed (mean age 72.9 years, standard deviation 6.71, range 65-92), 84 patients had epilepsy (epilepsy group) while 68 did not exhibit epilepsy (nonepilepsy group). For the consciousness levels, there were more level I patients in the nonepilepsy vs epilepsy group (P<0.028), with symptom duration lasting <1 minute (sensitivity 0.857, specificity 1) in the epilepsy group. In contrast, there were more level II patients in the epilepsy vs the nonepilepsy group (P=0.015), with the duration of symptoms lasting <1 minute (sensitivity 0.8125, specificity 0.930) in the epilepsy group. For the level III consciousness, convulsions were more commonly seen in the epilepsy group (P=0.044). CONCLUSION: Symptoms that last <1 minute with awareness and impaired awareness might be epileptic in the elderly population.

Ictal Asystole Induced by Right Posterior Quadrant Epilepsy: Report of a Radically Treated Case.

BACKGROUND: Ictal asystole (IA) and ictal bradycardia (IB) are mainly seen with temporal or frontal lobe epilepsy. Many patients with these conditions undergo cardiac pacemaker therapy but not epilepsy surgery. CASE DESCRIPTION: We report the case of a 15-year-old boy with IA and IB secondary to right posterior quadrant epilepsy (PoQE) who underwent right posterior quadrant disconnection, but not cardiac pacemaker implantation. He has remained free from daily epileptic seizures, IA, and IB for more than 6 months postoperatively. This is the first report of a radically treated case with IA and IB caused by PoQE. CONCLUSIONS: Both temporofrontal lobe epilepsy and PoQE caused the IA and IB. Because a cardiac pacemaker only addresses arrhythmia, not epileptic seizures, radical treatment for both epilepsy and arrhythmia may be warranted for patients with medically intractable epilepsy.

Double match of 18F-fluorodeoxyglucose-PET and iomazenil-SPECT improves outcomes of focus resection surgery.

BACKGROUND: When the results of electroencephalography (EEG), magnetic resonance imaging (MRI), and seizure semiology are discordant or no structural lesion is evident on MRI, single-photon emission computed tomography (SPECT) and positron emission tomography (PET) are important examinations for lateralization or localization of epileptic regions. We hypothesized that the concordance between interictal 2-[18F]fluoro-2-deoxy-D-glucose (18FDG)-PET and iomazenil (IMZ)-SPECT could suggest the epileptogenic lobe in patients with non-lesional findings on MRI. METHOD: Fifty-nine patients (31 females, 28 males; mean age, 29 years; median age, 27 years; range, 7-56 years) underwent subdural electrode implantation followed by focus resection. All patients underwent 18FDG-PET, IMZ-SPECT, and focus resection surgery. Follow-up was continued for ≥ 2 years. We evaluated surgical outcomes as seizure-free or not and analyzed correlations between outcomes and concordances of low-uptake lobes on PET, SPECT, or both PET and SPECT to the resection lobes. We used uni- and multivariate logistic regression analyses. RESULTS: In univariate analyses, all three concordances correlated significantly with seizure-free outcomes (PET, p = 0.017; SPECT, p = 0.030; both PET and SPECT, p = 0.006). In multivariate analysis, concordance between resection and low-uptake lobes in both PET and SPECT correlated significantly with seizure-free outcomes (p = 0.004). The odds ratio was 6.0. CONCLUSION: Concordance between interictal 18FDG-PET and IMZ-SPECT suggested that the epileptogenic lobe is six times better than each examination alone among patients with non-lesional findings on MRI. IMZ-SPECT and 18FDG-PET are complementary examinations in the assessment of localization-related epilepsy.

Effectiveness of total corpus callosotomy for diffuse bilateral polymicrogyria: Report of three pediatric cases.

PURPOSE: Polymicrogyria, a malformation of the cerebral cortex, frequently causes epilepsy. Diffuse bilateral polymicrogyria (DBP) is related to poor epilepsy prognosis, but most patients with DBP are not good candidates for resective epilepsy surgery and effectiveness of corpus callosotomy (CC), a palliative surgery, for patients without resective epileptogenic cortices, has not been established in DBP. Because CC might be effective against DBP-related epilepsy, we conducted total CC in three pediatric DBP cases. METHODS: Case 1. A girl developed epilepsy at 3 months of age, with focal versive seizures and epileptic spasms. The electroencephalogram (EEG) showed a suppression-burst pattern. Total CC was performed at 6 months of age. Case 2. A female infant developed epilepsy on the day of birth, exhibiting epileptic spasms, generalized tonic-clonic seizures, and eye-deviating seizures. She had a history of clusters of tonic seizures. Total CC was performed at 1 year and 2 months of age. After CC, the epileptic focus of the tonic seizures was identified; a secondary resective surgery was conducted. Case 3. A girl developed multiple types of seizures at 3 years of age. Frequent atypical absence status was refractory to antiepileptic drugs. Total CC was conducted at 8 years of age. RESULTS: Case 1: Frequencies of both seizure types decreased. The background EEG changed to continuous high-voltage slow waves. Case 2: Clusters of tonic seizures were well-controlled. Case 3: Atypical absence seizures completely disappeared. CONCLUSION: CC could be effective for patients with DBP, whose habitual seizures include epileptic spasms and absence seizures.

Use of high b value diffusion-weighted magnetic resonance imaging in acute encephalopathy/encephalitis during childhood.

AIM: To determine the use of high b value diffusion-weighted imaging (DWI) in the diagnosis and assessment of acute febrile encephalopathy/encephalitis in childhood. SUBJECTS AND METHODS: We enrolled 22 children, for whom we examined DWI with b=1000s/mm2, DWI with b=3000s/mm2, and apparent diffusion coefficient (ADC) map with b=1000 during the acute phase of febrile encephalopathy/encephalitis. Clinical diagnoses included acute encephalopathy with biphasic seizures and late reduced diffusion (AESD; n=6), clinically mild encephalopathy/encephalitis with a reversible splenial lesion (MERS; n=6), and herpes simplex virus encephalitis (HSE; n=3), unclassified acute encephalopathy/acute encephalitis (n=2); acute encephalitis with refractory, repetitive partial seizures (AERRPS; n=1); other encephalopathy (n=1); infarction (n=1); head injury (n=1); or mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (n=1). The diagnostic quality of brain lesions was compared between b=1000 and b=3000 DWI images by visual inspection. In addition, we attempted a quantitative assessment using apparent diffusion coefficient (ADC) value and an index of signal intensity (SI) ratio, defined as the mean SI at the affected lesion divided by the mean SI at the pons. RESULTS: High intensity lesions were either visible only on b=3000 DWI (n=5; 2 AESD, 1 MERS, 1 HSE, and 1 unclassifiable encephalopathy) or more effectively identified on b=3000 DWI than on b=1000 DWI (n=17). The outcome of the former five subjects was favorable, without motor or intellectual sequelae. The mean SI ratio of b=3000 was significantly greater than that of b=1000 in AESD and MERS subgroups as well as in all 22 subjects. Mean ADC values were lower in the AESD and MERS than that in the HSE subgroups. CONCLUSION: We concluded that b=3000 DWI was superior to b=1000 DWI in detecting abnormal lesions in acute encephalopathy/encephalitis during childhood.