Treatment odyssey to epilepsy surgery in children with focal cortical dysplasia: Risk factors for delayed surgical intervention.

OBJECTIVE: To elucidate the patient's journey to epilepsy surgery and identify the risk factors contributing to surgical delay in pediatric patients with drug-resistant epilepsy (DRE) due to focal cortical dysplasia (FCD). METHODS: A retrospective review was conducted of 93 pediatric patients who underwent curative epilepsy surgery for FCD between January 2012 and March 2023 at a tertiary epilepsy center. The Odyssey plot demonstrated the treatment process before epilepsy surgery, including key milestones of epilepsy onset, first hospital visit, epilepsy diagnosis, MRI diagnosis, DRE diagnosis, and surgery. The primary outcome was surgical delay; the duration from DRE to surgery. Multivariate linear regression models were used to examine the association between surgical delay and clinical, investigative, and treatment characteristics. RESULTS: The median age at seizure onset was 1.3 years (interquartile range [IQR] 0.14-3.1), and at the time of surgery, it was 6 years (range 1-11). Notably, 46% experienced surgical delays exceeding two years. The Odyssey plot visually highlighted that surgical delay comprised a significant portion of the patient journey. Although most patients underwent MRI before referral, MRI abnormalities were identified before referral only in 39% of the prolonged group, compared to 70% of the non-prolonged group. Multivariate analyses showed that delayed notification of MRI abnormalities, longer duration from epilepsy onset to DRE, older age at onset, number of antiseizure medications tried, and moderate to severe intellectual disability were significantly associated with prolonged surgical delay. CONCLUSION: Pediatric DRE patients with FCD experienced a long journey until surgery. Early and accurate identification of MRI abnormalities is important to minimize surgical delays.

Intraventricular hemorrhage volume and younger age at surgery may be risk factors for postoperative hydrocephalus after hemispherotomy in children.

OBJECTIVE: Hemispherotomy is an effective treatment for intractable hemispheric epilepsy; however, hydrocephalus remains a common complication of the procedure. The causes of hydrocephalus following hemispherotomy have not been fully elucidated; therefore, the purpose of this study was to identify the risk factors associated with the condition. METHODS: The authors investigated the records of all patients aged < 18 years who underwent hemispherotomy at their institution between 2003 and 2020 and were monitored for hydrocephalus for at least 1 year after the procedure. To identify the risk factors for hydrocephalus, the following information about each patient was collected: sex, corrected age at surgery, body weight at surgery, previous intracranial surgery, etiology of epilepsy, results of PET for hypermetabolism, side of surgery, type of operation (vertical or horizontal approach), operation time, blood loss during surgery, use of intraventricular drainage, occurrence of intraventricular hemorrhage (IVH) on the 1st postoperative day, duration of postoperative fever of > 38°C, and maximum C-reactive protein level after the operation. Multivariate logistic regression analyses were performed. RESULTS: This study included 51 children who underwent hemispherotomies for drug-resistant epilepsy at our hospital. Seven patients (13.7%) experienced hydrocephalus and were treated with ventricular or subdural peritoneal shunts or fenestration. Multivariate logistic analysis using the Bayesian information criterion revealed that 3 factors were associated with the occurrence of hydrocephalus: age at surgery, postoperative IVH volume, and duration of postoperative fever of > 38°C. CONCLUSIONS: This study showed that younger age at surgery, postoperative IVH volume, and duration of postoperative fever of > 38°C might be risk factors for hydrocephalus after hemispherotomy. The risk of hydrocephalus should be considered in cases of early surgical indication in children. Intraoperative hemostasis and postoperative use of anti-inflammatory measures may reduce the risk of hydrocephalus.

An integrated genetic analysis of epileptogenic brain malformed lesions.

Focal cortical dysplasia is the most common malformation during cortical development, sometimes excised by epilepsy surgery and often caused by somatic variants of the mTOR pathway genes. In this study, we performed a genetic analysis of epileptogenic brain malformed lesions from 64 patients with focal cortical dysplasia, hemimegalencephy, brain tumors, or hippocampal sclerosis. Targeted sequencing, whole-exome sequencing, and single nucleotide polymorphism microarray detected four germline and 35 somatic variants, comprising three copy number variants and 36 single nucleotide variants and indels in 37 patients. One of the somatic variants in focal cortical dysplasia type IIB was an in-frame deletion in MTOR, in which only gain-of-function missense variants have been reported. In focal cortical dysplasia type I, somatic variants of MAP2K1 and PTPN11 involved in the RAS/MAPK pathway were detected. The in-frame deletions of MTOR and MAP2K1 in this study resulted in the activation of the mTOR pathway in transiently transfected cells. In addition, the PTPN11 missense variant tended to elongate activation of the mTOR or RAS/MAPK pathway, depending on culture conditions. We demonstrate that epileptogenic brain malformed lesions except for focal cortical dysplasia type II arose from somatic variants of diverse genes but were eventually linked to the mTOR pathway.

Successful Hemispherotomy in a Patient with Encephalopathy with Continuous Spikes and Waves during Sleep Related to Neonatal Thalamic Hemorrhage: A Case Report with Intracranial Electroencephalogram Findings.

Neonatal thalamic hemorrhage is a strong risk factor for developing encephalopathy with continuous spikes and waves during sleep (ECSWS), even when not accompanied by widespread cortical destruction. The efficacy and indication of resective epilepsy surgery in such patients has not yet been reported. A 4-year-old boy was diagnosed with ECSWS based on strong epileptiform activation during sleep and neurocognitive deterioration. He had a history of left thalamic hemorrhage related to a straight sinus thrombosis during the newborn period. He presented with daily absence seizures that were refractory to medical treatment. At age 5, he underwent intracranial electroencephalogram (EEG) recording using depth and subdural strip electrodes placed in the left thalamus and over bilateral cortex, respectively. Interictal and ictal epileptiform discharges were observed in the thalamus, always preceded by discharges in the left or right parietal lobe. Left hemispherotomy successfully normalized the EEG of his unaffected hemisphere and extinguished his seizures. This is the first case report documenting resective epilepsy surgery in a patient with ECSWS due to neonatal thalamic injury without widespread cerebral destruction. Based on intracranial EEG findings, his injured thalamus did not directly generate the EEG abnormalities or absence seizures on its own. Patients with ipsilateral neonatal thalamic injury and even mild lateralized cortical changes may be candidates for resective or disconnective surgery for ECSWS.

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

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.

Nutritional Intervention Facilitates Food Intake after Epilepsy Surgery.

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 (p = 0.015) and lower total infusion (p = 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.

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

Introduction: 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. Methods: 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. Results: 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 (p = 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 (p < 0.05 each; paired t-test). Conclusion: 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.

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

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.

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

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.

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

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.

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

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.

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

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.

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.

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

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.

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

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.

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

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 × 10-6) 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.

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.

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.