Publications by authors named "Shimpei Baba"

37 Publications

Epilepsy in patients with focal cortical dysplasia may be associated with autism spectrum disorder.

Epilepsy Behav 2021 May 3;120:107990. Epub 2021 May 3.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

Introduction: Patients with epilepsy associated with focal cortical dysplasia (FCD) may be associated with autism spectrum disorder (ASD). Therefore, the purpose of this study was to compare surgically treated patients with epilepsy secondary to FCD and normal volunteers without epilepsy and to review the neuropathological findings of patients with FCD.

Methods: This study involved 38 patients with medically intractable focal onset epileptic seizures who underwent epilepsy surgery (Group 1). All patients had epilepsy associated with FCD. These patients and 38 normal volunteers without epilepsy (Group 2) were administered the autism spectrum quotient (AQ) test, and the groups were compared.

Results: The 38 patients in Group 1 included 16 females and 22 males (age range 20-60, mean age, 33.0; standard deviation (SD), 11.8 years). The normal volunteers in Group 2 included 22 females and 16 males (age range 20-57, mean age, 30.6 years; SD, 8.8 years). Total AQ scores were significantly higher in Group 1 than Group 2 (p = 0.027). Patients with FCD I showed a higher AQ score than those with FCD II in the AQ test (p ≤ 0.001).

Conclusion: Patients with epilepsy secondary to FCD were associated with higher ASD score than normal volunteers. This tendency was seen more strongly in patients with FCD I than FCD II.
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http://dx.doi.org/10.1016/j.yebeh.2021.107990DOI Listing
May 2021

Nutritional Intervention Facilitates Food Intake after Epilepsy Surgery.

Brain Sci 2021 Apr 17;11(4). Epub 2021 Apr 17.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka 430-8558, Japan.

Background: We investigated whether nutritional intervention affected food intake after epilepsy surgery and if intravenous infusions were required in patients with epilepsy. We hypothesized that postoperative food intake would be increased by nutritional intervention. The purpose of this study was to compare postoperative food intake in the periods before and after nutritional intervention.

Methods: Between September 2015 and October 2020, 124 epilepsy surgeries were performed. Of these, 65 patients who underwent subdural electrode placement followed by open cranial epilepsy surgery were studied. Postoperative total food intake, rate of maintenance of food intake, and total intravenous infusion were compared in the periods before and after nutritional intervention.

Results: A total of 26 females and 39 males (age range 3-60, mean 27.1, standard deviation (SD) 14.3, median 26 years) were enrolled. Of these, 18 females and 23 males (3-60, mean 28.2, SD 15.1, median 26 years) were in the pre-nutritional intervention period group, and eight females and 16 males (5-51, mean 25.2, SD 12.9, median 26.5 years) were in the post-nutritional intervention period group. The post-nutritional intervention period group showed significantly higher food intake ( = 0.015) and lower total infusion ( = 0.006) than the pre-nutritional intervention period group.

Conclusion: The nutritional intervention increased food intake and also reduced the total amount of intravenous infusion. To identify the cut-off day to cease the intervention and to evaluate whether the intervention can reduce the complication rate, a multicenter study with a large number of patients is warranted.
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http://dx.doi.org/10.3390/brainsci11040514DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8073881PMC
April 2021

Freedom From Seizures Might Be Key to Continuing Occupation After Epilepsy Surgery.

Front Neurol 2021 12;12:585191. Epub 2021 Feb 12.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.

We hypothesized that epilepsy surgery for adult patients with temporal lobe epilepsy (TLE) who obtained freedom from seizures could provide opportunities for these patients to continue their occupation, and investigated continuity of occupation to test this postulation. Data were obtained from patients who had undergone resective surgery for medically intractable TLE between October 2009 and April 2019 in our hospital. Inclusion criteria were as follows: (1) ≥16 years old at surgery; (2) post-operative follow-up ≥12 months; (3) seizure-free period ≥12 months. As a primary outcome, we evaluated employment status before and after surgery, classified into three categories as follows: Level 0, no job; Level 1, students or homemakers (financially supported by a family member); and Level 2, working. Neuropsychological status was also evaluated as a secondary outcome. Fifty-one (87.9%) of the 58 enrolled TLE patients who obtained freedom from seizures after surgery continued working as before or obtained a new job (employment status: Level 2). A significant difference in employment status was identified between before and after surgery ( = 0.007; Wilcoxon signed-rank test). Twenty-eight patients (48.3%) were evaluated for neuropsychological status both before and after surgery. Significant differences in Wechsler Adult Intelligence Scale-III scores were identified between before and after surgery ( < 0.05 each; paired -test). Seizure freedom could be a factor that facilitates job continuity, although additional data are needed to confirm that possibility. Further investigation of job continuity after epilepsy surgery warrants an international, multicenter study.
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http://dx.doi.org/10.3389/fneur.2021.585191DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7907155PMC
February 2021

Case Report: Late-Onset Temporal Lobe Epilepsy Following Subarachnoid Hemorrhage: An Interplay Between Pre-existing Cortical Development Abnormality and Tissue Damage.

Front Neurol 2021 9;12:599130. Epub 2021 Feb 9.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.

Epileptogenicity following brain insult depends on various factors including severity of the resulting lesion and extent of brain damage. We report a 54-year-old female patient who developed medically refractory epilepsy resulting from the interplay of pre-existing and post-insult pathologies. She presented with subarachnoid hemorrhage (SAH) due to a ruptured aneurysm and underwent clipping surgery. Seizures started 3 months post-operatively. MRI revealed cerebral ischemia and hemosiderin deposits in the left temporal lobes, and left hippocampal atrophy was suspected. As anti-seizure medications and vagus nerve stimulation failed to control her seizures, she underwent left temporal lobe resection and placement of a ventriculoperitoneal shunt for the post-operative complication of hydrocephalus. She remains seizure-free to date. Neuropathology revealed a previously undiagnosed focal cortical dysplasia (FCD) type 1a. Brain insult likely had a second hit effect in the late onset of epilepsy in this patient with pre-existing mild MCD, in whom secondary epilepsy can be attributed to the interplay of multiple underlying pathologies.
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http://dx.doi.org/10.3389/fneur.2021.599130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7901922PMC
February 2021

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

Epilepsy Behav 2021 03 6;116:107772. Epub 2021 Feb 6.

Department of Epilepsy and Neurosurgery, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu, Shizuoka 4308558, Japan. Electronic address:

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.
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http://dx.doi.org/10.1016/j.yebeh.2021.107772DOI Listing
March 2021

Phase Lag Analyses on Ictal Scalp Electroencephalography May Predict Outcomes of Corpus Callosotomy for Epileptic Spasms.

Front Neurol 2020 15;11:576087. Epub 2020 Dec 15.

Comprehensive Epilepsy Center, Seirei-Hamamatsu General Hospital, Hamamatsu, Japan.

We aimed to clarify the patterns of ictal power and phase lag among bilateral hemispheres on scalp electroencephalography (EEG) recorded pre-operatively during epileptic spasms (ESs) and the correlation with the outcomes following corpus callosotomy. We enrolled 17 patients who underwent corpus callosotomy for ESs before 20 years of age. After corpus callosotomy, seven patients did not experience further ESs (favorable outcome group), and the remaining 10 patients had ongoing ESs (unfavorable outcome group). We used pre-operative scalp EEG data from monopolar montages using the average reference. The relative power spectrum (PS), ictal power laterality (IPL) among the hemispheres, and phase lag, calculated by the cross-power spectrum (CPS) among symmetrical electrodes (i.e., F3 and F4), were analyzed in the EEG data of ESs from 143 pre-operative scalp video-EEG records. Analyses were conducted separately in each frequency band from the delta, theta, alpha, beta, and gamma range. We compared the means of those data in each patient between favorable and unfavorable outcome groups. Among all frequency bands, no significant differences were seen in the individual mean relative PSs in the favorable and unfavorable outcome group. Although the mean IPLs in each patient tended to be high in the unfavorable outcome group, no significant differences were found. The mean CPSs in the delta, theta, and gamma frequency bands were significantly higher in the unfavorable than in the favorable outcome group. Using the Youden index, the optimal cutoff points of those mean CPS values for unfavorable outcomes were 64.00 in the delta band (sensitivity: 100%, specificity: 80%), 74.20 in the theta band (100, 80%), and 82.05 in the gamma band (100, 80%). Subanalyses indicated that those CPS differences originated from pairs of symmetrical electrodes in the bilateral frontal and temporal areas. Ictal power and laterality of the ictal power in each frequency band were not associated with the outcomes of CC; however, the phase lags seen in the delta, theta, and gamma frequency bands were larger in the unfavorable than in the favorable outcome group. The phase lags may predict outcomes of CC for ESs on pre-surgical scalp-ictal EEGs.
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http://dx.doi.org/10.3389/fneur.2020.576087DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7793812PMC
December 2020

"Struggling status" as a form of status epilepticus in an infant with hypothalamic hamartoma: A case report with video-electroencephalogram.

Seizure 2021 Feb 5;85:39-41. Epub 2021 Jan 5.

Department of Child Neurology, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Naka-ku, Hamamatsu, Shizuoka, 430-8558, Japan.

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http://dx.doi.org/10.1016/j.seizure.2020.12.010DOI Listing
February 2021

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

Front Neurol 2020 3;11:591477. Epub 2020 Dec 3.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.

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.
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http://dx.doi.org/10.3389/fneur.2020.591477DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7744758PMC
December 2020

Higher intelligence may be a risk factor for postoperative transient disturbance of consciousness after corpus callosotomy.

Epilepsy Behav 2021 02 11;115:107617. Epub 2020 Dec 11.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

Introduction: Corpus callosotomy (CC) is an established surgical option for palliative treatment of medically intractable epilepsy, especially for seizures with drop attacks. We postulated that specific risk factors for post-CC transient disturbance of consciousness (pCTDC) are associated with CC. The purpose of this study was to review patients with intractable epilepsy who underwent CC and to statistically analyze risk factors for pCTDC.

Methods: Inclusion criteria for patients who underwent CC between January 2009 and November 2019 were: (1) ≥2 years old and (2) followed up for more than 8 months. The state of consciousness before and after CC was evaluated with the Glasgow coma scale. We statistically assessed predictors for pCTDC as the primary outcome.

Results: Fifty-six patients (19 females, 37 males) were enrolled, and the age range was 2-57 years old. Thirty-seven (66.1%) patients developed pCTDC. The mean period from the beginning of the state of pCTDC to recovery to their baseline conscious level was 4.9 days (range: 2-25 days). All three (100%) normal intelligence level patients, 13 (81%) of 16 patients with a moderately impaired level of intelligence, and 21 (57%) of 37 patients with a severely impaired level of intelligence exhibited pCTDC. Univariate (p = 0.044) and multivariate (p = 0.006) logistic regression analyses for predictors of pCTDC showed that intellectual function was statistically significant.

Conclusion: Two-thirds of patients developed pCTDC. One risk factor for pCTDC may be higher intellectual function.
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http://dx.doi.org/10.1016/j.yebeh.2020.107617DOI Listing
February 2021

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.

Neuropediatrics 2021 04 23;52(2):133-137. Epub 2020 Nov 23.

Department of Child Neurology, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.

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.
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http://dx.doi.org/10.1055/s-0040-1716903DOI Listing
April 2021

Postoperative persistent fever may be a risk factor for hydrocephalus in hemispherical disconnection surgery.

Epilepsy Behav 2020 11 26;112:107466. Epub 2020 Sep 26.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

Introduction: Hemispherical disconnection surgery such as hemispherotomy or posterior quadrant disconnection (PQD) surgery sometimes induces hydrocephalus. We postulated that some risk factors for postoperative hydrocephalus can be managed perioperatively. The purpose of this study was to clarify and statistically analyze perioperative risk factors for postoperative progressive hydrocephalus.

Methods: We reviewed patients who underwent hemispherotomy or PQD. We compared patients with and without progressive hydrocephalus with multivariate and univariate logistic regression analysis to identify risk factors for hydrocephalus.

Results: Twenty-four patients underwent hemispherectomy or PQD (age: 25 days-45 years old, mean: 13.3 years, median: 8 years, standard deviation: 13.9 years, 14 males). Among them, five patients (21%) required hydrocephalus treatment. Persistent fever was a risk factor for progressive hydrocephalus (multivariate analysis: p = 0.024, univariate analysis: p < 0.001).

Conclusion: Postoperative persistent fever may be a manageable risk factor for postoperative hydrocephalus in hemispherotomy and PQD surgery.
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http://dx.doi.org/10.1016/j.yebeh.2020.107466DOI Listing
November 2020

Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate.

Pediatr Neurol 2020 12 2;113:33-41. Epub 2020 Sep 2.

Department of Child Neurology, Okayama University Hospital, Okayama, Japan; Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

Background: We aimed to demonstrate the biochemical characteristics of vitamin B6-dependent epilepsy, with a particular focus on pyridoxal 5'-phosphate and pyridoxal in the cerebrospinal fluid.

Methods: Using our laboratory database, we identified patients with vitamin B6-dependent epilepsy and extracted their data on the concentrations of pyridoxal 5'-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters. We compared the biochemical characteristics of these patients with those of other epilepsy patients with low pyridoxal 5'-phosphate concentrations.

Results: We identified seven patients with pyridoxine-dependent epilepsy caused by an ALDH7A1 gene abnormality, two patients with pyridoxal 5'-phosphate homeostasis protein deficiency, and 28 patients with other epilepsies with low cerebrospinal fluid pyridoxal 5'-phosphate concentrations. Cerebrospinal fluid pyridoxal and pyridoxal 5'-phosphate concentrations were low in patients with vitamin B6-dependent epilepsy but cerebrospinal fluid pyridoxal concentrations were not reduced in most patients with other epilepsies with low cerebrospinal fluid pyridoxal 5'-phosphate concentrations. Increase in 3-O-methyldopa and 5-hydroxytryptophan was demonstrated in some patients with vitamin B6-dependent epilepsy, suggestive of pyridoxal 5'-phosphate deficiency in the brain.

Conclusions: Low cerebrospinal fluid pyridoxal concentrations may be a better indicator of pyridoxal 5'-phosphate deficiency in the brain in vitamin B6-dependent epilepsy than low cerebrospinal fluid pyridoxal 5'-phosphate concentrations. This finding is especially helpful in individuals with suspected pyridoxal 5'-phosphate homeostasis protein deficiency, which does not have known biomarkers.
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http://dx.doi.org/10.1016/j.pediatrneurol.2020.08.020DOI Listing
December 2020

Posttraumatic epilepsy may be a state in which underlying epileptogenicity involves focal cortical dysplasia.

Epilepsy Behav 2021 01 23;114(Pt A):107352. Epub 2020 Aug 23.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

Introduction: The occurrence rate of posttrauma epilepsy ranges widely from 1% to 30%. Little is known about the underlying epileptogenesis of traumatic brain injury (TBI)-related epilepsy (TRE), because no comparison between TRE and TBI without epilepsy has been performed in terms of neuropathology. Therefore, we postulated that different neuropathological factors may be present between TRE and TBI without epilepsy. The purpose of this study was to clarify differences between TRE and TBI without epilepsy.

Methods: We studied patients who experienced severe head trauma and underwent brain surgery. The age range of the patients was 9-71 years old. Patients with medically resistant epilepsy were included in the Epilepsy group, and patients without epilepsy were included in the nonepilepsy group. Pathological findings, age, sex, and cause of head trauma were statistically compared between these two groups.

Results: This study involved 10 patients, nine of whom met the inclusion criteria. Pathological findings for all patients in the Epilepsy group included focal cortical dysplasia (FCD) (p = 0.012).

Conclusion: The difference between TRE and TBI without epilepsy was underlying FCD in patients with TRE.
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http://dx.doi.org/10.1016/j.yebeh.2020.107352DOI Listing
January 2021

Removal of a temporal lobe cavernous angioma to control epileptic seizures in a patient with tuberous sclerosis complex.

Heliyon 2020 Jun 23;6(6):e04229. Epub 2020 Jun 23.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Japan.

Purpose: A patient with tuberous sclerosis complex (TSC) and a left temporal cavernous angioma (CA) presented with treatment-resistant epilepsy. We evaluated the patient to determine the best treatment option.

Patient And Methods: A 7-year-old boy with TSC exhibited weekly impaired awareness seizures and was diagnosed with TSC based on the modified Gomez's criteria. The presence of cortical tubers had been noted by his physicians. However, left temporal CA had not been diagnosed. He was referred to our facility for further treatment at the age of 33. Presurgical evaluation in our facility revealed the brain tubers and left temporal CA. Based on his seizure semiology, magnetic resonance imaging, scalp electroencephalogram, and long-term video monitoring, we determined his seizures were from the CA and not the TSC network. We then performed intraoperative-electrocorticography (ECoG).

Results: Because the ECoG showed epileptiform discharges from the surrounding area of the CA but not from other areas, we removed the CA. He has been seizure-free for more than 10 years.

Conclusion: The higher likelihood of TSC as well as greater familiarity with this disorder might lead physicians to overlook the possibility of CA.
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http://dx.doi.org/10.1016/j.heliyon.2020.e04229DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322044PMC
June 2020

Epilepsy surgery reduced seizure frequency for patients with severe motor and intellectual disorders.

Epilepsy Behav 2020 05 20;106:107031. Epub 2020 Mar 20.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Japan.

Introduction: Little is known about epilepsy surgery for patients with severe motor and intellectual disorders (SMIDs). We hypothesized that epilepsy surgery could reduce epileptic seizure frequency in these patients. The purpose of this study was to compare pre- and postoperative seizure frequency in patients with SMIDs.

Methods: A total of 288 surgeries were performed for pediatric patients, including those with SMIDs, from 2009 to 2018. Inclusion criteria were as follows: Oshima classification 1 (intelligence quotient <20 and bedridden), ≥2 years old, proven ictal events evaluated by long-term video electroencephalography, and ≥1-year follow-up. Seizure frequency and the number of antiseizure medications (ASMs) were compared between pre- and postepilepsy surgery. Patients' respiratory and feeding conditions were also examined to determine comorbidities.

Results: Nineteen patients (5 girls, 14 boys; age: 2 to 12 years) fulfilled the inclusion criteria. One patient underwent focus resection, 2 patients underwent total corpus callosotomy, and 16 patients underwent vagus nerve stimulation therapies. Of 19 patients, 16 (84.2%) had daily seizures, and 3 (15.8%) had weekly seizures before surgery. Epilepsy surgery significantly reduced seizure frequency (p = 0.029). Five patients (26.3%) had status epilepticus (SE) before surgery, which disappeared in all but one after surgery (p = 0.046). The number of ASMs did not change between before and after surgery (p = 0.728).

Conclusion: Epilepsy surgery reduced the frequency of epileptic seizures and improved SE even among patients with compromised respiratory function and compromised food intake.
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http://dx.doi.org/10.1016/j.yebeh.2020.107031DOI Listing
May 2020

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

Sci Rep 2019 12 24;9(1):19733. Epub 2019 Dec 24.

Comprehensive Epilepsy Center, Seirei-Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu, 430-8558, Japan.

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.
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http://dx.doi.org/10.1038/s41598-019-56303-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6930281PMC
December 2019

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

Front Neurol 2019 20;10:1233. Epub 2019 Nov 20.

Epilepsy Center, Seirei-Hamamatsu General Hospital, Shizuoka, Japan.

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.
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http://dx.doi.org/10.3389/fneur.2019.01233DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6879674PMC
November 2019

Hemispherotomy can cause post-operative strabismus.

Brain Dev 2020 Jan 11;42(1):41-47. Epub 2019 Sep 11.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Japan.

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.
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http://dx.doi.org/10.1016/j.braindev.2019.08.011DOI Listing
January 2020

Corpus callosotomy for drug-resistant spasms associated with tuberous sclerosis complex.

Epilepsy Behav 2019 09 5;98(Pt A):228-232. Epub 2019 Aug 5.

Department of Child Neurology, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu 430-8558, Japan.

Background: Corpus callosotomy (CC) has recently been adopted for the treatment of drug-resistant epileptic spasms and tonic spasms. In the present study, we investigated CC outcomes among patients with drug-resistant epileptic spasms or tonic spasms associated with tuberous sclerosis complex (TSC).

Methods: We retrospectively collected data from seven patients (3 women, 4 men) with diagnosed TSC and who were treated using CC at Seirei Hamamatsu General Hospital in Japan. All patients had experienced drug-resistant epileptic spasms (<3 s of muscular contraction) or tonic spasms (>3 s) prior to CC, which were confirmed via video-electroencephalogram monitoring.

Results: All patients exhibited multiple bilateral cortical tubers on brain magnetic resonance imaging. The main seizure types were epileptic spasms in four, tonic spasms in one, and both seizure types in two patients. Patients underwent total CC between the ages of 25 months and 21.5 years. Additional resection or disconnection was performed in two patients. The follow-up period after CC ranged between 9 months and 3.5 years. Three patients achieved remission from spasms following CC alone. Two other patients became free from spasms several months after CC but required an additional focus disconnection or medical treatment. The remaining two patients continued to show spasms or asymmetrical tonic seizures.

Conclusion: Total CC resulted in freedom from drug-resistant epileptic or tonic spasms in several patients with TSC. Stepwise progression from CC to additional resection or disconnection surgery may aid in the treatment of spasms secondary to TSC.
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http://dx.doi.org/10.1016/j.yebeh.2019.06.007DOI Listing
September 2019

Cranioplasty with Titanium Might Be Suitable for Adult Epilepsy Surgery After Subdural Placement Surgery To Avoid Surgical Site Infection.

World Neurosurg 2019 Nov 2;131:e503-e507. Epub 2019 Aug 2.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.

Background: The purpose of the present study was to compare the surgical site infection (SSI) rates between resorbable plates and titanium plates used for adult patients with intractable epilepsy who had undergone epilepsy surgery after subdural electrode placement.

Methods: We performed subdural electrode surgery, followed by epilepsy surgery, for 87 adult patients with intractable epilepsy. The epilepsy surgery included 75 focus resections and 12 corpus callosotomies. We compared the SSI rates between patients who had undergone cranioplasty with titanium and resorbable plates after epilepsy surgery.

Results: Of the 87 patients, 43 had undergone cranioplasty with resorbable plates (group A) and 44 had undergone cranioplasty with titanium plates (group B). The frequency of SSI was significantly greater in group A (7 patients; 16.3%) than in group B (1 patient; 2.3%; P = 0.03, Fisher's exact test). Univariate regression analysis also showed a significantly greater infection rate with the resorbable plates (P = 0.024).

Conclusion: For epilepsy surgery of adult patients after subdural electrode placement surgery, the SSI rate for cranioplasty was greater with resorbable plates than with titanium plates.
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http://dx.doi.org/10.1016/j.wneu.2019.07.201DOI Listing
November 2019

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

World Neurosurg 2019 Oct 9;130:e839-e845. Epub 2019 Jul 9.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

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/m 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.
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http://dx.doi.org/10.1016/j.wneu.2019.06.235DOI Listing
October 2019

Comprehensive analysis of coding variants highlights genetic complexity in developmental and epileptic encephalopathy.

Nat Commun 2019 06 7;10(1):2506. Epub 2019 Jun 7.

Department of Human Genetics, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.

Although there are many known Mendelian genes linked to epileptic or developmental and epileptic encephalopathy (EE/DEE), its genetic architecture is not fully explained. Here, we address this incompleteness by analyzing exomes of 743 EE/DEE cases and 2366 controls. We observe that damaging ultra-rare variants (dURVs) unique to an individual are significantly overrepresented in EE/DEE, both in known EE/DEE genes and the other non-EE/DEE genes. Importantly, enrichment of dURVs in non-EE/DEE genes is significant, even in the subset of cases with diagnostic dURVs (P = 0.000215), suggesting oligogenic contribution of non-EE/DEE gene dURVs. Gene-based analysis identifies exome-wide significant (P = 2.04 × 10) enrichment of damaging de novo mutations in NF1, a gene primarily linked to neurofibromatosis, in infantile spasm. Together with accumulating evidence for roles of oligogenic or modifier variants in severe neurodevelopmental disorders, our results highlight genetic complexity in EE/DEE, and indicate that EE/DEE is not an aggregate of simple Mendelian disorders.
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http://dx.doi.org/10.1038/s41467-019-10482-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6555845PMC
June 2019

Side Slit Guide Pipe for Precise Placement of Depth Electrodes.

World Neurosurg 2019 Jun 15;126:291-295. Epub 2019 Mar 15.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

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.
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http://dx.doi.org/10.1016/j.wneu.2019.03.067DOI Listing
June 2019

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

Pediatr Neurol 2019 06 30;95:79-83. Epub 2019 Jan 30.

Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

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.
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.012DOI Listing
June 2019

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

Neuropsychiatr Dis Treat 2018 30;14:2879-2887. Epub 2018 Oct 30.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan,

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 (<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 (=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 (=0.044).

Conclusion: Symptoms that last <1 minute with awareness and impaired awareness might be epileptic in the elderly population.
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http://dx.doi.org/10.2147/NDT.S179720DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214591PMC
October 2018

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

Brain Dev 2019 Mar 9;41(3):296-300. Epub 2018 Oct 9.

Department of Child Neurology, Seirei-Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu 430-8558, Japan.

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.
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http://dx.doi.org/10.1016/j.braindev.2018.09.008DOI Listing
March 2019

De novo PHACTR1 mutations in West syndrome and their pathophysiological effects.

Brain 2018 11;141(11):3098-3114

Department of Molecular Neurobiology, Institute for Developmental Research, Aichi Human Service Center, 713-8 Kamiya, Kasugai, Aichi, Japan.

Trio-based whole exome sequencing identified two de novo heterozygous missense mutations [c.1449T > C/p.(Leu500Pro) and c.1436A > T/p.(Asn479Ile)] in PHACTR1, encoding a molecule critical for the regulation of protein phosphatase 1 (PP1) and the actin cytoskeleton, in unrelated Japanese individuals with West syndrome (infantile spasms with intellectual disability). We then examined the role of Phactr1 in the development of mouse cerebral cortex and the pathophysiological significance of these two mutations and others [c.1561C > T/p.(Arg521Cys) and c.1553T > A/p.(Ile518Asn)], which had been reported in undiagnosed patients with intellectual disability. Immunoprecipitation analyses revealed that actin-binding activity of PHACTR1 was impaired by the p.Leu500Pro, p.Asn479Ile and p.Ile518Asn mutations while the p.Arg521Cys mutation exhibited impaired binding to PP1. Acute knockdown of mouse Phactr1 using in utero electroporation caused defects in cortical neuron migration during corticogenesis, which were rescued by an RNAi-resistant PHACTR1 but not by the four mutants. Experiments using knockdown combined with expression mutants, aimed to mimic the effects of the heterozygous mutations under conditions of haploinsufficiency, suggested a dominant negative effect of the mutant allele. As for dendritic development in vivo, only the p.Arg521Cys mutant was determined to have dominant negative effects, because the three other mutants appeared to be degraded with these experimental conditions. Electrophysiological analyses revealed abnormal synaptic properties in Phactr1-deficient excitatory cortical neurons. Our data show that the PHACTR1 mutations may cause morphological and functional defects in cortical neurons during brain development, which is likely to be related to the pathophysiology of West syndrome and other neurodevelopmental disorders.
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http://dx.doi.org/10.1093/brain/awy246DOI Listing
November 2018

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

World Neurosurg 2018 Dec 14;120:337-342. Epub 2018 Sep 14.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

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.
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http://dx.doi.org/10.1016/j.wneu.2018.09.030DOI Listing
December 2018

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

Acta Neurochir (Wien) 2018 09 1;160(9):1875-1882. Epub 2018 Jun 1.

Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Nakaku, Hamamatsu, Shizuoka, 430-8558, Japan.

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-[F]fluoro-2-deoxy-D-glucose (FDG)-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 FDG-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 FDG-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 FDG-PET are complementary examinations in the assessment of localization-related epilepsy.
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http://dx.doi.org/10.1007/s00701-018-3573-1DOI Listing
September 2018

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

Brain Dev 2018 Sep 3;40(8):719-723. Epub 2018 Apr 3.

Comprehensive Epilepsy Center, Seirei-Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.

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.
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http://dx.doi.org/10.1016/j.braindev.2018.03.007DOI Listing
September 2018