Infantile Epileptic Spasms Syndrome Complicated by Leigh Syndrome and Leigh-Like Syndrome: A Retrospective, Nationwide, Multicenter Case Series.

BACKGROUND: Six percent of patients with Leigh syndrome (LS) present with infantile epileptic spasms syndrome (IESS). However, treatment strategies for IESS with LS remain unclear. This retrospective study aimed to evaluate the efficacy and safety of treatment strategies in patients with IESS complicated by LS and Leigh-like syndrome (LLS). METHODS: We distributed questionnaires to 750 facilities in Japan, and the clinical data of 21 patients from 15 hospitals were collected. The data comprised treatment strategies, including adrenocorticotropic hormone (ACTH) therapy, ketogenic diet (KD) therapy, and antiseizure medications (ASMs); effectiveness of each treatment; and the adverse events. RESULTS: The median age at LS and LLS diagnosis was 7 months (range: 0 to 50), whereas that at the onset of epileptic spasms was 7 (range: 3 to 20). LS was diagnosed in 17 patients and LLS in four patients. Seven, two, five, and seven patients received ACTH + ASMs, ACTH + KD + ASMs, KD + ASMs, and ASMs only, respectively. Four (44%) of nine patients treated with ACTH and one (14%) of seven patients treated with KD achieved electroclinical remission within one month of treatment. No patients treated with only ASMs achieved electroclinical remission. Seven patients (33%) achieved electroclinical remission by the last follow-up. Adverse events were reported in four patients treated with ACTH, none treated with KD therapy, and eight treated with ASMs. CONCLUSION: ACTH therapy shows the best efficacy and rapid action in patients with IESS complicated by LS and LLS. The effectiveness of KD therapy and ASMs in this study was insufficient.

Analysis of Cell Immunity for Children Infected with SARS-CoV-2 and Those Vaccinated against SARS-CoV-2 Using T-SPOT®.COVID.

Cellular immunity is critical for the regulation of viral diseases, including coronavirus disease 2019 (COVID-19), and is generally considered immature in childhood. However, the details of cellular immunity against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection among children are unclear. We assessed cellular immunity in eight children post-vaccination against SARS-CoV-2 and 11 children after SARS-CoV-2 infection using the T-SPOT®.COVID assay for the spike (S) and nucleocapsid (N) proteins. In the vaccinated group, the T-SPOT®.COVID assay for the S protein yielded positive results in seven children. In the post-infection group, the assay for the N protein was positive for 5 of 11 children, with 3 of these 5 children requiring hospitalization, including 2 who needed mechanical ventilation. The T-SPOT®.COVID assay is thus valuable for assessing cellular immunity against SARS-CoV-2, and most children infected with SARS-CoV-2 may not develop such immunity unless the disease severity is significant.

Non-1st seizure was less severe than 1st seizure with non-urgent level among suspected seizures transferred by ambulance.

BACKGROUND: To prioritize emergency medical calls for ambulance transport for patients with suspected seizures, information about whether the event is their 1st or non-1st seizure is important. However, little is known about the difference between 1st and non-1st seizures in terms of severity. We hypothesized that patients transferred multiple times (≥2 times) would represent a milder scenario than patients on their first transfer. The purpose of this study was to compare patients with suspected seizures on 1st transfer by ambulance and patients who had been transferred ≥2 times. METHODS: We statistically compared severity of suspected seizures between two groups of patients with suspected seizures transferred between December 2014 and November 2019 (before the coronavirus disease 2019 pandemic) to our facility by ambulance for either the first time (1st Group) or at least the second time (Non-1st Group). Severity categories were defined as: Level 1 = life-threatening; Level 2 = emergent, needing admission to the intensive care unit; Level 3 = urgent, needing admission to a hospital general ward; Level 4 = less urgent, needing intervention but not hospitalization; and Level 5 = non-urgent, not needing intervention. RESULTS: Among 5996 patients with suspected seizures conveyed to the emergency department by ambulance a total of 14,263 times during the study period, 1222 times (8.6%) and 636 patients (11%) met the criteria. Severity grade of suspected seizures ranged from 1 to 5 (median, 4; interquartile range, 3-4) for the 1st Group and from 1 to 5 (median, 5; interquartile range, 4-5) for the Non-1st Group. Most severe grade ranged from 1 to 5 (median, 4; interquartile range, 4-5) for the Non-1st Group. Severity grade differed significantly between groups (p < 0.001, Mann-Whitney U-test). Uni- and multivariate logistic regression tests also suggested a significant difference (p < 0.001) in severity grades. CONCLUSION: In direct comparisons, grade of suspected seizure severity was lower in the Non-1st Group than in the 1st Group.

False lateralization of scalp EEG and semiology in cavernous malformation-associated temporal lobe epilepsy: A case report.

Background: Several cases of temporal lobe epilepsy (TLE) showing false lateralization of ictal scalp electroencephalography (EEG) have been reported. However, TLE with cavernous malformation indicating false lateralization of both ictal scalp EEG and semiology as in the present case is rare. The aim of this report is to call attention to avoiding overestimation of ictal scalp EEG findings in epilepsy patients with cavernous malformation. Case report: A 25-year-old man without any medical history suffered from seizures for a year despite appropriate anti-epileptic medication. Magnetic resonance imaging (MRI) revealed cavernous malformation in the left amygdala. The seizure type was brief impaired consciousness with left dystonic posturing, preceded by a sensation of blood rushing to the head. Long-term video EEG with scalp electrodes showed periodic sharp waves beginning from the right temporal area during seizures. Although both semiology and ictal scalp EEG indicated right TLE, intracranial EEG revealed the onset of low-voltage fast activity from the left hippocampus near the cavernous malformation. This patient therefore underwent removal of cavernous malformation and left amygdala, and achieved freedom from seizures postoperatively. Conclusion: We reinforce the importance of performing intracranial EEG for cavernous malformation-associated epilepsy when discrepancies between scalp EEG and MRI are evident.

Finger movement functions remain in the ipsilesional hemisphere and compensation by the contralesional hemisphere might not be expected after hemispherotomy -pre- and post-hemispherotomy evaluations in 8 cases.

BACKGROUND: We hypothesized that fine finger motor functions are controlled by the ipsilesional hemisphere, and that gross motor functions are compensated for by the contralesional hemisphere after brain injury in humans. The purpose of this study was to compare finger movements before and after hemispherotomy that defunctionated the ipsilesional hemisphere for patients with hemispherical lesions. METHODS: We statistically compared Brunnstrom stage of the fingers, arm (upper extremity), and leg (lower extremity) before and after hemispherotomy. Inclusion criteria for this study were: 1) hemispherotomy for hemispherical epilepsy; 2) a ≥ 6-month history of hemiparesis; 3) post-operative follow-up ≥ 6 months; 4) complete freedom from seizures without aura; and 5) application of our protocol for hemispherotomy. RESULTS: Among 36 patients who underwent multi-lobe disconnection surgeries, 8 patients (2 girls, 6 boys) met the study criteria. Mean age at surgery was 6.38 years (range, 2-12 years; median, 6 years; standard deviation, 3.5 years). Paresis of the fingers was significantly exacerbated (p = 0.011) compared to pre-operatively, whereas that of the upper limbs (p = 0.07) and lower limbs (p = 0.103) was not. CONCLUSION: Finger movement functions tend to remain in the ipsilesional hemisphere after brain injury, whereas gross motor movement functions such as those of the arms and legs are compensated for by the contralesional hemisphere in humans.

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.

Unexplained Progressive Neurological Deficits after Corpus Callosotomy May Be Caused by Autoimmune Encephalitis: A Case of Suspected Postoperative Anti-NMDAR Encephalitis.

The main causes of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are ovarian teratoma and herpes simplex virus (HSV) encephalitis. We present a rare case of suspected anti-NMDAR encephalitis caused by corpus callosotomy (CC). An 18-year-old woman with Lennox-Gastaut syndrome underwent CC. Although left hemiplegic due to cerebral hemorrhage and impaired consciousness due to cerebral venous sinus thrombosis (CVST) appeared postoperatively, anticoagulant therapy quickly improved CVST and impaired consciousness. However, various unexplained symptoms such as insomnia, hallucination, impulsivity, impaired consciousness, and a new type of drug-resistant cluster seizures gradually developed over a 2-month period. Magnetic resonance imaging revealed the gradual extension of a hyperintense area from the right frontal lobe on fluid-attenuated inversion recovery images. Intravenous methylprednisolone pulse was initiated from postoperative day (POD) 74, followed by intravenous immunoglobulin (IVIg) therapy, although white blood cell counts were normal in all three cerebrospinal fluid (CSF) examinations. After IVIg therapy, the above unexplained symptoms promptly improved. On POD 103, antibodies against NMDAR were revealed in both the serum and CSF collected before these immunotherapies. The patient was transferred to a rehabilitation hospital due to residual left hemiplegia. Psychiatric symptoms and a new onset of drug-resistant seizures may be suggestive of postoperative anti-NMDAR encephalitis, even if CSF findings are mild.

Replacement of Valproic Acid with New Anti-Seizure Medications in Idiopathic Generalized Epilepsy.

Background: Little is known regarding the non-inferiority of new anti-seizure medications (ASMs) in terms of replacing valproic acid (VPA) in patients with idiopathic generalized epilepsy (IGE). We hypothesized that replacement of VPA with new ASMs would offer non-inferior or better control of seizure frequency. The purpose of this study was to compare epileptic seizure frequency between the subset of patients with IGE who were on VPA and the subset of patients with IGE who replaced VPA with new ASMs. Methods: Patients with IGE who were on or had been on VPA between January 2016 and March 2022 were divided into a group that replaced VPA with new ASMs (VPA-replace group) and a group that remained on VPA (VPA-continue group). We then compared the groups in terms of seizure frequency and myoclonus. Results: Of the 606 patients on VPA between January 2016 and March 2022, 156 patients with IGE were enrolled to this study (VPA-replace group, n = 68; VPA-continue group, n = 88). The VPA-replace group included significantly more females than the VPA-continue group (p < 0.001). The VPA-replace group also showed significantly higher seizure frequency before replacement (p < 0.001), but not after replacement (p = 0.074). Patients on monotherapy displayed improved seizure frequency with new ASMs (p < 0.001). Among the new ASMs, perampanel (PER) significantly improved seizure frequency (p = 0.002). Forty-two patients in the VPA-replace group who had myoclonus achieved significant improvements (p < 0.001). Among these, patients on PER monotherapy (p < 0.001) or PER + lamotrigine (0.016) showed significantly improved myoclonus scale scores. Conclusions: This study shows the non-inferiority of new ASMs compared to VPA, with better seizure control using new ASMs in subsets of patients with IGE on monotherapy.

Omitting Hyperventilation in Electroencephalogram during the COVID-19 Pandemic May Reduce Interictal Epileptiform Discharges in Patients with Juvenile Myoclonic Epilepsy.

BACKGROUND: To prevent the spread of coronavirus disease 2019 (COVID-19), hyperventilation (HV) activation has been avoided in electroencephalograms (EEGs) since April 2020. The influence of omitting HV in EEG on epilepsy diagnosis remains uncertain for patients with epilepsies other than child absence epilepsy. We hypothesized that EEGs with HV would show more interictal epileptiform discharges (IEDs) than EEGs without HV in patients with juvenile myoclonic epilepsy (JME). METHODS: We reviewed the EEGs of seizure-free patients with JME who underwent EEG, both with and without HV, from January 2019 to October 2021, in our institution, and compared IEDs between EEG with and without HV. RESULTS: This study analyzed 23 JME patients. The IED-positive rate was significantly higher in EEG with HV (65.2%) than in EEG without HV (34.8%, p = 0.016). The mean ± standard deviation number of IEDs per minute was significantly larger during HV (1.61 ± 2.25 × 10-1) than during non-activation of both first EEG (0.57 ± 0.93 × 10-1, p = 0.039) and second EEG (0.39 ± 0.76 × 10-1, p = 0.009). CONCLUSIONS: In JME patients, performing HV during EEG may increase IEDs and appears to facilitate the accurate diagnosis of epilepsy.

Impact of COVID-19 pandemic on epilepsy care in Japan: A national-level multicenter retrospective cohort study.

OBJECTIVE: The impact of the coronavirus disease 2019 (COVID-19) pandemic on epilepsy care across Japan was investigated by conducting a multicenter retrospective cohort study. METHODS: This study included monthly data on the frequency of (1) visits by outpatients with epilepsy, (2) outpatient electroencephalography (EEG) studies, (3) telemedicine for epilepsy, (4) admissions for epilepsy, (5) EEG monitoring, and (6) epilepsy surgery in epilepsy centers and clinics across Japan between January 2019 and December 2020. We defined the primary outcome as epilepsy-center-specific monthly data divided by the 12-month average in 2019 for each facility. We determined whether the COVID-19 pandemic-related factors (such as year [2019 or 2020], COVID-19 cases in each prefecture in the previous month, and the state of emergency) were independently associated with these outcomes. RESULTS: In 2020, the frequency of outpatient EEG studies (-10.7%, p<0.001) and cases with telemedicine (+2,608%, p=0.031) were affected. The number of COVID-19 cases was an independent associated factor for epilepsy admission (-3.75*10-3 % per case, p<0.001) and EEG monitoring (-3.81*10-3 % per case, p = 0.004). Further, the state of emergency was an independent factor associated with outpatient with epilepsy (-11.9%, p<0.001), outpatient EEG (-32.3%, p<0.001), telemedicine for epilepsy (+12,915%, p<0.001), epilepsy admissions (-35.3%; p<0.001), EEG monitoring (-24.7%: p<0.001), and epilepsy surgery (-50.3%, p<0.001). SIGNIFICANCE: We demonstrated the significant impact that the COVID-19 pandemic had on epilepsy care. These results support those of previous studies and clarify the effect size of each pandemic-related factor on epilepsy care.

Lacosamide monotherapy for the treatment of childhood epilepsy with centrotemporal spikes.

OBJECTIVE: Childhood epilepsy with centrotemporal spikes (CECTS) is known as age-limited focal epilepsy syndrome in childhood. Lacosamide is a third-generation antiepileptic drug. This study aimed to evaluate the efficacy of lacosamide monotherapy for the treatment of CECTS. METHODS: We enrolled 18 patients (6 girls and 12 boys) who met the following criteria: 1) the age of onset of the seizures was between 3 and 13 years of age; 2) showing at least hemifacial and/or oropharyngeal seizures; 3) interictal discharges in central and/or middle temporal electrodes; 4) no intellectual disability; 5) treatment duration of lacosamide monotherapy over 6 months. We retrospectively collected and analyzed clinical data and treatment information. We evaluated the seizure occurrences during 0-3, 4-6, and 7-12 months from the treatment initiation and the last 6 months of the follow-up. We also evaluated the outcomes as seizure-free if the patients developed no seizures both over 6 months and 3 times of pretreatment mean seizure interval at the last follow-up. RESULTS: Of the patients, 39%, 67% and 72% were seizure-free during 0-3, 4-6, and 7-12 months from treatment initiation, respectively. Finally, 83% of the patients achieved seizure freedom. Seizure freedom was achieved in 72% during the first 4 months of treatment. All patients continued lacosamide monotherapy during the study, although four patients showed transient fatigue or somnolence. CONCLUSIONS: Lacosamide showed good efficacy for controlling seizures with fewer adverse effects, and therefore may be a good candidate as a first-line medication for the treatment of new-onset CECTS.

Earlier Age at Surgery for Brain Cavernous Angioma-Related Epilepsy May Achieve Complete Seizure Freedom without Aid of Anti-Seizure Medication.

BACKGROUND: The present study hypothesized that some factors may distinguish between patients with a brain cavernous angioma (BCA), who were free from anti-seizure medication (ASM), and patients who still required ASMs postoperatively. The purpose of the study was thus to identify factors associated with ceasing ASMs for patients with drug-resistant epilepsy secondary to BCA, who underwent BCA removal surgery. METHODS: We divided patients into those with drug-resistant epilepsy secondary to BCA who achieved complete seizure freedom without ASMs a year after surgery (No-ASM group) (International League Against Epilepsy (ILAE) classification class I with no epileptiform discharges), and others (ASM group) (ILAE classification ≤ II and/or epileptiform discharges). We statistically compared groups in terms of: (1) age at operation; (2) history of epilepsy; (3) size of BCA; and (4) location of BCA. RESULTS: Overall, a year after the surgery, the No-ASM group comprised 12 patients (48%), and the ASM group comprised 13 patients (52%). In both multi- and univariate logistic regression analyses, age at BCA removal surgery correlated significantly with the No-ASM group (p = 0.043, p = 0.019), but history of epilepsy did not (p = 0.581, p = 0.585). CONCLUSIONS: Earlier age at surgery for patients with drug-resistant epilepsy is encouraged to achieve complete seizure freedom without the need for ASMs when the cause of epilepsy is BCA.

Effects of Vagus Nerve Stimulation following Corpus Callosotomy for Patients with Drug-Resistant Epilepsy.

OBJECTIVE: The effectiveness of vagus nerve stimulation (VNS) for residual seizures after corpus callosotomy (CC) has not yet been fully investigated. We hypothesized that seizure control would be improved by VNS after CC. The purpose of this study was to compare seizure frequency between patients with implantation of a VNS generator (post-VNS group) or without VNS (non-post-VNS group) following CC. METHODS: We retrospectively reviewed patients who underwent CC between January 2009 and May 2019 in our institution. We evaluated proportions of ≥50% reduction in seizure frequency (responders) and seizure reduction rate 1 and 2 years after VNS. To investigate factors related to responders, uni- and multivariate logistic regression analyses were performed regarding age, number of anti-seizure medications (ASMs), addition of novel ASMs (levetiracetam, lacosamide or perampanel), and post-VNS or non-post-VNS status. RESULTS: Thirteen post-VNS patients and 24 non-post-VNS patients were analyzed in this study. Responder rate at 1 year after VNS differed significantly between the post-VNS group (53.9%) and non-post-VNS group (12.5%, p = 0.017). Number of ASMs at the time of CC and post-VNS were significantly associated with responders in univariate analyses (odds ratio [OR] 0.34, 95% confidence interval [CI] 0.13-0.88, p = 0.025 and OR 8.2, 95%CI 1.6-41.6, p = 0.011, respectively), whereas age, sex, seizure frequency, and addition of novel ASMs were not. In multivariate analysis, the presence of VNS procedures after CC was the only factor favorably associated with responder status (OR 82.2, 95%CI 1.55-4355.7, p = 0.03). CONCLUSIONS: VNS therapy after CC may increase the proportion of responders independent of the addition of novel ASMs.

Non-Ruptured Temporal Lobe Dermoid Cyst Concomitant with Focal Cortical Dysplasia Causing Temporal Lobe Epilepsy-A Case Report and Literature Review.

BACKGROUND: Intracranial dermoid cyst is a rare, benign, nonneoplastic tumor-like lesion that could cause seizures, headache, and hydrocephalus. We hypothesized that the temporal lobe dermoid cyst in combination with other factors were causing the epileptic seizure. METHODS: We encountered a 17-year-old girl with anti-seizure medication-resistant epilepsy secondary to dermoid cyst located in the temporal region depicted on magnetic resonance imaging (MRI). She showed neither symptoms of meningitis nor rupture of the cyst according to serial MRI. We hypothesized that temporal lobe dermoid cyst in combination with other factors, such as focal cortical dysplasia (FCD), etc., was causing epileptic seizures in this case. She underwent dermoid cyst removal surgery with resection of the tip of the antero-inferior temporal lobe. RESULTS: Histopathological study showed multiple small intramedullary dermoid cysts in the left antero-inferior temporal lobe in addition to MRI lesions and FCD. CONCLUSION: A patient with medically intractable epilepsy secondary to left temporal lobe dermoid cyst showed multiple intramedullary dermoid cysts and focal cortical dysplasia that might have interacted to create epileptogenicity. To our knowledge, this is the first case report of dermoid cyst concomitant with FCD.

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.

Postoperative Pneumocephalus on Computed Tomography Might Predict Post-Corpus Callosotomy Chemical Meningitis.

BACKGROUND: A corpus callosotomy (CC) is a procedure in which the corpus callosum, the largest collection of commissural fibers in the brain, is disconnected to treat epileptic seizures. The occurrence of chemical meningitis has been reported in association with this procedure. We hypothesized that intraventricular pneumocephalus after CC surgery represents a risk factor for postoperative chemical meningitis. The purpose of this study was to analyze the potential risk factors for postoperative chemical meningitis in patients with medically intractable epilepsy who underwent a CC. METHODS: Among the patients who underwent an anterior/total CC for medically intractable epilepsy between January 2009 and March 2021, participants were comprised of those who underwent a computed tomography scan on postoperative day 0. We statistically compared the groups with (c-Group) or without chemical meningitis (nc-Group) to determine the risk factors. RESULTS: Of the 80 patients who underwent a CC, 65 patients (25 females and 40 males) met the inclusion criteria. Their age at the time of their CC procedure was 0-57 years. The c-Group (17%) was comprised of seven females and four males (age at the time of their CC procedure, 1-43 years), and the nc-Group (83%) was comprised of 18 females and 36 males (age at the time of their CC procedure, 0-57 years). Mann-Whitney U-tests (p = 0.002) and univariate logistic regression analysis (p = 0.001) showed a significant difference in pneumocephalus between the groups. CONCLUSION: Postoperative pneumocephalus identified on a computed tomography scan is a risk factor for post-CC chemical meningitis.

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.

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.

Speech Analysis Using Artificial Intelligence as a Peri-Operative Evaluation: A Case Report of a Patient with Temporal Lobe Epilepsy Secondary to Tuberous Sclerosis Complex Who Underwent Epilepsy Surgery.

BACKGROUND: Improved conversational fluency is sometimes identified postoperatively in patients with epilepsy, but improvements can be difficult to assess using tests such as the intelligence quotient (IQ) test. Evaluation of pre- and postoperative differences might be considered subjective at present because of the lack of objective criteria. Artificial intelligence (AI) could possibly be used to make the evaluations more objective. The aim of this case report is thus to analyze the speech of a young female patient with epilepsy before and after surgery. METHOD: The speech of a nine-year-old girl with epilepsy secondary to tuberous sclerosis complex is recorded during interviews one month before and two months after surgery. The recorded speech is then manually transcribed and annotated, and subsequently automatically analyzed using AI software. IQ testing is also conducted on both occasions. The patient remains seizure-free for at least 13 months postoperatively. RESULTS: There are decreases in total interview time and subjective case markers per second, whereas there are increases in morphemes and objective case markers per second. Postoperatively, IQ scores improve, except for the Perceptual Reasoning Index. CONCLUSIONS: AI analysis is able to identify differences in speech before and after epilepsy surgery upon an epilepsy patient with tuberous sclerosis complex.

Patients With Epilepsy Who Underwent Epilepsy Surgery During the COVID-19 Pandemic Showed Less Depressive Tendencies.

Introduction: Our hypothesis in this study was that differences might exist between patients with epilepsy (PWE) who underwent epilepsy surgery before and within the period of the coronavirus disease 2019 (COVID-19) pandemic. The purpose of this study was to compare results of the Zung Self-Rating Depression Scale (SDS) between PWE who underwent epilepsy surgery before and during the pandemic period. Methods: Participants were PWE who underwent open cranial epilepsy surgery between February 2019 and February 2021 in our hospital. Patients who underwent surgery in the first half of this period, between February 2019 and January 2020, were defined as the pre-pandemic period group (pre-Group) and those treated in the second half, between February 2020 and February 2021, were categorized as the pandemic period group (within-Group). All patients completed the SDS before surgery, and scores were compared between groups. Results: SDS score was significantly higher in the pre-Group than in the within-Group (p = 0.037). Other factors, including age (p = 0.51), sex (p = 0.558), epilepsy duration from onset to SDS score evaluation (p = 0.190), seizure frequency (p = 0.794), number of anti-seizure medications (p = 0.787), and intelligence quotient (p = 0.871) did not differ significantly between groups. Conclusion: SDS score was higher in the pre-pandemic group than in the within-pandemic group, which may indicate that PWE with less-positive outlooks may be less likely to seek medical attention during stressful periods.