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PURPOSE: Coherence analysis in electroencephalography (EEG) allows measurement of the degree of consistency of amplitude between pairs of electrodes. Theoretically, disconnective epilepsy surgery should decrease coherence between corresponding areas. The study aimed to evaluate postoperative changes in interhemispheric coherence values after corpus callosotomy (CC). METHODS: Non-lesional, drug-resistant, generalized epilepsy patients who underwent total CC were retrospectively collected. To evaluate coherence, we divided the scalp interictal EEG into "baseline" and "discharge" states after excluding periods with artifacts. Interhemispheric coherence values were obtained between eight pairs of symmetrically opposite scalp electrodes in six different frequency bands. We analyzed both pre- and postoperative EEG sessions and calculated the percentage of difference (POD) in coherence values. RESULTS: We collected 13 patients and analyzed 2496 interhemispheric coherence values. Preoperative coherence values differed significantly between baseline and discharge states (p = 0.0003), but postoperative values did not (p = 0.11). For baseline state, coherence values were decreased after CC and median POD was - 22.3% (p < 0.0001). Delta frequency showed the most decreased POD (-44.3%, p = 0.0009). Median POD was lowest in the Fp1-Fp2 pair of electrodes. For discharge state, coherence values were decreased after CC and median POD was - 24.7% (p < 0.0001). Delta frequency again showed the most decreased POD (-55.9%, p = 0.0016). Median POD was lowest in the F7-F8 pair. CONCLUSION: After total CC, interhemispheric coherence decreased significantly in both baseline and discharge states. The most decreased frequency band was the delta band, which may be used as a representative frequency band in future studies.
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Corpo Caloso , Eletroencefalografia , Epilepsia Generalizada , Couro Cabeludo , Humanos , Feminino , Eletroencefalografia/métodos , Masculino , Corpo Caloso/cirurgia , Corpo Caloso/fisiopatologia , Criança , Adolescente , Estudos Retrospectivos , Pré-Escolar , Epilepsia Generalizada/cirurgia , Epilepsia Generalizada/fisiopatologia , Epilepsia Resistente a Medicamentos/cirurgia , Epilepsia Resistente a Medicamentos/fisiopatologiaRESUMO
BACKGROUND: The objective of frontal lobe disconnection (FD) is to isolate the frontal lobe without removing brain parenchyma, to achieve epilepsy outcomes comparable to those for frontal lobectomy but with fewer complications, since a large post-operative cavity is not created. METHOD: Main surgical steps include "Identification of the central sulcus" to ensure the preservation of motor function, and "Frontal lobe disconnection" comprising: 1) fronto-parietal disconnection; 2) fronto-basal disconnection; 3) anterior corpus callosotomy; and 4) insula and basal ganglia disconnection. CONCLUSION: In performing FD, consideration of the disconnection boundary based on an anatomical understanding of white matter tracts is essential.
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Lobo Frontal , Humanos , Lobo Frontal/cirurgia , Lobo Frontal/anatomia & histologia , Procedimentos Neurocirúrgicos/métodos , Corpo Caloso/cirurgia , Corpo Caloso/anatomia & histologia , Corpo Caloso/diagnóstico por imagem , Epilepsia do Lobo Frontal/cirurgiaRESUMO
Background Carbon 11 (11C)-methionine is a useful PET radiotracer for the management of patients with glioma, but radiation exposure and lack of molecular imaging facilities limit its use. Purpose To generate synthetic methionine PET images from contrast-enhanced (CE) MRI through an artificial intelligence (AI)-based image-to-image translation model and to compare its performance for grading and prognosis of gliomas with that of real PET. Materials and Methods An AI-based model to generate synthetic methionine PET images from CE MRI was developed and validated from patients who underwent both methionine PET and CE MRI at a university hospital from January 2007 to December 2018 (institutional data set). Pearson correlation coefficients for the maximum and mean tumor to background ratio (TBRmax and TBRmean, respectively) of methionine uptake and the lesion volume between synthetic and real PET were calculated. Two additional open-source glioma databases of preoperative CE MRI without methionine PET were used as the external test set. Using the TBRs, the area under the receiver operating characteristic curve (AUC) for classifying high-grade and low-grade gliomas and overall survival were evaluated. Results The institutional data set included 362 patients (mean age, 49 years ± 19 [SD]; 195 female, 167 male; training, n = 294; validation, n = 34; test, n = 34). In the internal test set, Pearson correlation coefficients were 0.68 (95% CI: 0.47, 0.81), 0.76 (95% CI: 0.59, 0.86), and 0.92 (95% CI: 0.85, 0.95) for TBRmax, TBRmean, and lesion volume, respectively. The external test set included 344 patients with gliomas (mean age, 53 years ± 15; 192 male, 152 female; high grade, n = 269). The AUC for TBRmax was 0.81 (95% CI: 0.75, 0.86) and the overall survival analysis showed a significant difference between the high (2-year survival rate, 27%) and low (2-year survival rate, 71%; P < .001) TBRmax groups. Conclusion The AI-based model-generated synthetic methionine PET images strongly correlated with real PET images and showed good performance for glioma grading and prognostication. Published under a CC BY 4.0 license. Supplemental material is available for this article.
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Neoplasias Encefálicas , Glioma , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Metionina , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Inteligência Artificial , Tomografia por Emissão de Pósitrons/métodos , Gradação de Tumores , Glioma/diagnóstico por imagem , Glioma/patologia , Imageamento por Ressonância Magnética/métodos , RacemetioninaRESUMO
Surgical treatment is an effective option for medically intractable epilepsy. Amygdalohippocampectomy for mesial temporal lobe epilepsy is a surgically remediable epileptic syndrome. It is a well-established surgery and various approaches to the mesial temporal lobe have been reported. To reduce the complication rate, surgeons should have sufficient knowledge of anatomy in the mesial temporal region. Here, we summarize the surgical treatments for mesial temporal lobe epilepsy, focusing on anatomical understanding. We described in detail the surgical anatomy of amygdalohippocampectomy and various approaches to the mesial temporal region. In addition, we describe hippocampal transection aimed at preserving memory function, which is an alternative surgery in patients without hippocampal sclerosis. An anatomical understanding of the mesial temporal region helps surgeons not only in the field of epilepsy surgery, but also in other fields of neurosurgery, such as brain tumor and vascular surgery.
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Epilepsia Resistente a Medicamentos , Epilepsia do Lobo Temporal , Epilepsia , Humanos , Epilepsia do Lobo Temporal/cirurgia , Epilepsia do Lobo Temporal/patologia , Hipocampo/cirurgia , Hipocampo/patologia , Procedimentos Neurocirúrgicos , Epilepsia/cirurgia , Epilepsia Resistente a Medicamentos/cirurgia , Resultado do TratamentoRESUMO
PURPOSE: Subependymal giant cell astrocytomas (SEGAs) are tumors that usually arise in the wall of one or the other lateral ventricle near a foramen of Monro, most often on a background of tuberous sclerosis complex (TSC). TSC has a variety of clinical manifestations caused by germline mutations of the TSC complex subunit 1 or 2 (TSC1, TSC2) genes. SEGAs without clinical manifestations of TSC are termed solitary SEGAs, which are hypothesized to be caused by tumor-only TSC1/2 mutations, or "forme fruste" of TSC with somatic mosaic mutations. However, it is difficult to distinguish between the two. Here, we report three patients with genetically investigated solitary SEGAs and review this rare manifestation. METHODS: SEGA was completely removed in two patients and partially removed in one. Genetic analyses were performed on the tumor tissue and on peripheral blood via DNA microarray, reverse-transcriptase polymerase chain reaction, and next-generation sequencing with ultra-deep sequencing of mutation points. RESULTS: All three patients had tumors with TSC2 somatic mutations and loss of heterozygosity (LOH). In one patient, the same TSC2 mutation was also detected in 1% of leukocytes in his blood. The tumors did not recur, and clinical manifestations of TSC did not develop during the 4-year follow-up. CONCLUSIONS: The genetic cause of solitary SEGAs may be a TSC2 mutation with LOH. In patients with solitary SEGA, mosaic mutations may present in other organs, and TSC may clinically manifest later in life; therefore, patients should be followed up for prolonged periods.
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Astrocitoma , Proteína 2 do Complexo Esclerose Tuberosa/genética , Esclerose Tuberosa , Astrocitoma/diagnóstico por imagem , Astrocitoma/genética , Humanos , Mutação/genética , Recidiva Local de Neoplasia , Esclerose Tuberosa/complicações , Esclerose Tuberosa/genéticaRESUMO
The Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) update 3 recommends that histologic grade II and III IDH-wildtype diffuse astrocytic gliomas that harbor EGFR amplification, the combination of whole chromosome 7 gain and whole chromosome 10 loss (7 + /10 -), or TERT promoter (pTERT) mutations should be considered as glioblastomas (GBM), World Health Organization grade IV. In this retrospective study, we examined the utility of molecular classification based on pTERT status and copy-number alterations (CNAs) in IDH-wildtype lower grade gliomas (LGGs, grade II, and III). The impact on survival was evaluated for the pTERT mutation and CNAs, including EGFR gain/amplification, PTEN loss, CDKN2A homozygous deletion, and PDGFRA gain/amplification. We analyzed 46 patients with IDH-wildtype/pTERT-mutant (mut) LGGs and 85 with IDH-wildtype/pTERT-wildtype LGGs. EGFR amplification and a combination of EGFR gain and PTEN loss (EGFR + /PTEN -) were significantly more frequent in pTERT-mut patients (p < 0.0001). Cox regression analysis showed that the pTERT mutation was a significant predictor of poor prognosis (hazard ratio [HR] 2.79, 95% confidence interval [CI] 1.55-4.89, p = 0.0008), but neither EGFR amplification nor EGFR + /PTEN - was an independent prognostic factor in IDH-wildtype LGGs. PDGFRA gain/amplification was a significant poor prognostic factor in IDH-wildtype/pTERT-wildtype LGGs (HR 2.44, 95% CI 1.09-5.27, p = 0.03, Cox regression analysis). The IDH-wildtype LGGs with either pTERT-mut or PDGFRA amplification were mostly clustered with GBM by DNA methylation analysis. Thus, our study suggests that analysis of pTERT mutation status is necessary and sufficient to diagnose IDH-wildtype diffuse astrocytic gliomas with molecular features of glioblastoma. The PDGFRA status may help further delineate IDH-wildtype/pTERT-wildtype LGGs. Methylation profiling showed that IDH-wildtype LGGs without molecular features of GBM were a heterogeneous group of tumors. Some of them did not fall into existing categories and had significantly better prognoses than those clustered with GBM.
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Neoplasias Encefálicas/genética , Glioma/diagnóstico , Glioma/genética , Mutação/genética , Telomerase/genética , Adulto , Neoplasias Encefálicas/diagnóstico , Variações do Número de Cópias de DNA/genética , Feminino , Glioma/patologia , Homozigoto , Humanos , Isocitrato Desidrogenase/genética , Masculino , Pessoa de Meia-Idade , PTEN Fosfo-Hidrolase/genética , Deleção de Sequência/genéticaRESUMO
OBJECTIVE: Sudden death in the bathtub occurs relatively frequently in Japan, particularly among elderly people. We hypothesize that sudden death in epilepsy occurring in the bathtub (SDEPB) can be distinguished from sudden death in nonepilepsy occurring in the bathtub (SDnonEPB), but is identical to sudden unexpected death in epilepsy (SUDEP). METHODS: Tokyo Medical Examiner's Office conducts postmortem examinations for all sudden and unexpected deaths in Tokyo. Clinical, social, and autopsy findings of 43 SDEPB were compared with 76 SDnonEPB, 50 SUDEP outside the bathtub, and Japanese forensic autopsy data as controls. RESULTS: Extension of the leg(s) outside the bathtub was seen in 33% of SDEPB, but none of SDnonEPB. Sitting position was seen less frequently in SDEPB (37%) than in SDnonEPB (64%). Lung weight and pleural effusion volume were significantly lower in SDEPB than in SDnonEPB. Age at death in SDEPB was significantly younger than that in SDnonEPB. Sudden death in epilepsy occurring in the bathtub showed no differences in lung weight and pleural effusion volume from SUDEP. Living with family was more frequent in SDEPB (73%) than in SUDEP (48%). Few antiepileptic drugs, infrequent seizures, and low rate of mental retardation were identical between SDEPB and SUDEP. Lung weight was significantly heavier in all three groups than in age- and sex- matched autopsy controls. CONCLUSIONS: Leg extension outside the bathtub, lower lung weight, and absence of pleural effusion distinguish SDEPB from SDnonEPB in elderly people. Sudden death in epilepsy occurring in the bathtub may represent a form of SUDEP occurring in the bathtub, rather than drowning despite submergence in the bathtub at discovery. Conditions for bathing require careful attention from physicians and relatives, even for patients with epilepsy with few medications and infrequent seizures, and without mental retardation.
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Banhos/efeitos adversos , Epilepsia/diagnóstico , Epilepsia/mortalidade , Morte Súbita Inesperada na Epilepsia/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticonvulsivantes/uso terapêutico , Banhos/tendências , Morte Súbita/epidemiologia , Morte Súbita/prevenção & controle , Epilepsia/tratamento farmacológico , Feminino , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Convulsões/diagnóstico , Convulsões/tratamento farmacológico , Convulsões/mortalidade , Morte Súbita Inesperada na Epilepsia/prevenção & controle , Adulto JovemRESUMO
INTRODUCTION: This study investigates the current state of clinical practice and molecular analysis for elderly patients with diffuse gliomas and aims to elucidate treatment outcomes and prognostic factors of patients with glioblastomas. METHODS: We collected elderly cases (≥ 70 years) diagnosed with primary diffuse gliomas and enrolled in Kansai Molecular Diagnosis Network for CNS Tumors. Clinical and pathological characteristics were analyzed retrospectively. Various factors were evaluated in univariate and multivariate models to examine their effects on overall survival. RESULTS: Included in the study were 140 elderly patients (WHO grade II: 7, III: 19, IV: 114), median age was 75 years. Sixty-seven patients (47.9%) had preoperative Karnofsky Performance Status score of ≥ 80. All patients underwent resection (gross-total: 20.0%, subtotal: 14.3%, partial: 39.3%, biopsy: 26.4%). Ninety-six of the patients (68.6%) received adjuvant treatment consisting of radiotherapy (RT) with temozolomide (TMZ). Seventy-eight of the patients (75.0%) received radiation dose of ≥ 50 Gy. MGMT promoter was methylated in 68 tumors (48.6%), IDH1/2 was wild-type in 129 tumors (92.1%), and TERT promoter was mutated in 78 of 128 tumors (60.9%). Median progression-free and overall survival of grade IV cases was 8.2 and 13.6 months, respectively. Higher age (≥ 80 years) and TERT promoter mutated were associated with shorter survival. Resection and adjuvant RT + TMZ were identified as independent factors for good prognosis. CONCLUSIONS: This community-based study reveals characteristics and outcomes of elderly glioma patients in a real-world setting. Elderly patients have several potential factors for poor prognosis, but resection followed by RT + TMZ could lengthen duration of survival.
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Neoplasias Encefálicas/metabolismo , Neoplasias Encefálicas/terapia , Glioma/metabolismo , Glioma/terapia , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/mortalidade , Metilação de DNA , Metilases de Modificação do DNA/genética , Metilases de Modificação do DNA/metabolismo , Enzimas Reparadoras do DNA/genética , Enzimas Reparadoras do DNA/metabolismo , Feminino , Glioma/genética , Glioma/mortalidade , Humanos , Isocitrato Desidrogenase/genética , Japão , Masculino , Mutação , Gradação de Tumores , Prognóstico , Estudos Retrospectivos , Telomerase/genética , Proteínas Supressoras de Tumor/genética , Proteínas Supressoras de Tumor/metabolismoRESUMO
BACKGROUND: Electrocorticogram (ECoG) spike activity is enhanced under general anesthesia with 1.5 minimum alveolar concentration (MAC) sevoflurane compared with lower concentrations in adult patients with epilepsy. However, the effect of concentration of sevoflurane on ECoG in children with epilepsy is less known. AIMS: The primary endpoint was to investigate the effects of sevoflurane on ECoG spike activity in pediatric patients undergoing epilepsy surgery. The secondary endpoint was to examine its effects on baseline ECoG including burst suppression. METHODS: Children of age 3-18 years with medically intractable epilepsy undergoing corpus callosotomy or resection of the epileptic foci (n = 11) were enrolled. Electrodes were placed on the brain surface and ECoG was recorded under anesthesia with endtidal carbon dioxide tension at 30 mmHg and sevoflurane at 2.5%, followed by age-adjusted 1.5 MAC (3.1-3.4%) for 10 min. The number of leads with spikes, the average number of spikes per lead per minute, median frequency of ECoG, and duration of suppression of ECoG ≥ 1 s were compared between 2.5% and 1.5 MAC sevoflurane. RESULTS: The number of leads with spikes increased [11 vs 14, P = 0.003, difference in mean (95% CI) is 3 (2-5)], and the average number of spikes increased [9 vs 14·lead-1 ·min-1 , P = 0.003, difference in mean (95% CI) is 5 (2-8) lead-1 ·min-1 ] under anesthesia with 1.5 MAC compared with 2.5% sevoflurane. Median frequency was decreased [2.8 Hz vs 2.0 Hz, P = 0.003, difference in mean (95% CI) is 0.8 (0.4-1.2) Hz], and the duration of suppression was increased [105 s vs 262 s, P < 0.001, difference in mean (95% CI) is 156 (90-223) s] with 1.5 MAC compared with 2.5% sevoflurane. CONCLUSIONS: Sevoflurane at 1.5 MAC significantly increased the extent and the number of spikes, prolonged the duration of suppression, and decreased median frequency of ECoG compared with those at 2.5% sevoflurane.
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Anestésicos Inalatórios/farmacologia , Encéfalo/efeitos dos fármacos , Eletroencefalografia/efeitos dos fármacos , Epilepsia/cirurgia , Éteres Metílicos/farmacologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , SevofluranoRESUMO
OBJECTIVE: Amygdala enlargement (AE) has been suggested to be a subtype of mesial temporal lobe epilepsy (MTLE). However, most reports related to AE have referred to imaging studies, and there have been few reports regarding surgical and pathological findings. The present study was performed to clarify the surgical outcomes and pathology of AE. METHODS: Eighty patients with drug-resistant MTLE were treated surgically at the Tokyo Metropolitan Neurological Hospital between April 2010 and July 2013. Of these patients, 11 were diagnosed as AE based on presurgical MRI. Nine patients with AE underwent selective amygdalohippocampectomy, while the remaining two patients underwent selective amygdalotomy with hippocampal transection. Intraoperative EEG was routinely performed. The histopathology of the resected amygdala tissue was evaluated and compared with the amygdala tissue of patients with hippocampal sclerosis. RESULTS: Pathological findings indicated that 10 of 11 specimens had closely clustering hypertrophic neurons with vacuolisation of the background matrix. Slight gliosis was seen in nine specimens, while the remaining two showed no gliotic changes. Intraoperative EEG showed abnormal sharp waves that seemed to originate not from the amygdala but from the hippocampus in all cases. Ten patients became seizure-free during the postoperative follow-up period. CONCLUSIONS: Histopathologically, clustering hypertrophic neurons and vacuolation with slight gliosis or without gliosis were considered to be pathological characteristics of AE. Amygdalohippocampectomy or hippocampal transection with amygdalotomy is effective for seizure control in patients with AE.
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Tonsila do Cerebelo/cirurgia , Epilepsia do Lobo Temporal/cirurgia , Adolescente , Adulto , Tonsila do Cerebelo/patologia , Eletroencefalografia , Epilepsia do Lobo Temporal/patologia , Feminino , Humanos , Hipertrofia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuroimagem , Resultado do Tratamento , Adulto JovemRESUMO
We report a case of developmental and epileptic encephalopathy with spike-and-wave activation during sleep with 22q11.2 deletion syndrome in a patient who had undergone hemispherotomy and achieved developmental improvement. A four-year-old male child with paralysis on the left side of his body since birth had a mild developmental delay. An MRI of the brain revealed polymicrogyria diffusely throughout the right hemisphere. He was diagnosed with the 22q11.2 deletion syndrome at one year of age. Focal impaired awareness seizure in the right hemisphere origin and focal to bilateral tonic-clonic seizure appeared by two years of age. At three years of age, myoclonic seizures occurred, which induced frequent falls. Simultaneously, developmental and epileptic encephalopathy with spike-and-wave activation during sleep were observed. At four years and seven months of age, the patient underwent a right hemispherotomy. Epileptic seizures and spike-and-wave activation during sleep disappeared, and cognitive improvement was observed one year after surgery. In spite of chromosomal abnormalities being present, drug-resistant epilepsy with localized regions on MRI should be evaluated to determine surgical options to improve cognitive function and development.
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Minimally invasive surgery is gaining increasing interest in epilepsy surgery. In this video, the authors present the endoscopic transorbital approach for an epileptogenic lesion located at the temporal tip. The patient was a man in his 40s who has had intractable focal impaired awareness seizures and focal to bilateral tonic-clonic seizures since he was 31 years of age. According to the preoperative examination, including stereotactic electroencephalography, a cavernous angioma located at the tip of the right temporal lobe was diagnosed as an epileptogenic lesion. Lesionectomy for this lesion was performed using the endoscopic transorbital approach as minimally invasive surgery and a favorable outcome was achieved. The video can be found here: https://stream.cadmore.media/r10.3171/2024.4.FOCVID2414.
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Two main surgical techniques are available for corpus callosotomy (CC): conventional microscopic CC and endoscopic CC.1 Microscopic CC is more familiar to neurosurgeons and allows three-dimensional visualization, but it requires a larger craniotomy and has a narrower visual angle in the deep part. Endoscopic CC has only recently been introduced to epilepsy surgery, but it is gaining increasing interest among epilepsy surgeons. The endoscope provides two-dimensional visualization and requires a camera as an additional instrument inserted into the surgical corridor. The merits of endoscopic CC include the smaller craniotomy and smaller skin incision, potentially reducing invasiveness.2 Bridging veins to the superior sagittal sinus are also less problematic because of the reduced need for brain retraction. The lack of need of arachnoid dissection is another advantage. Generally, an anterior approach is applied for CC, but this approach makes interhemispheric fissure dissection mandatory, especially at the cingulate gyri. In some cases, this procedure can take a long time. On the other hand, a posterior approach requires less interhemispheric arachnoid dissection, or sometimes none at all, due to the anatomy of the falx cerebri. These reasons have driven the development of a posterior approach for an endoscopic-alone technique.3 Here, we present a 5-year-old girl with medically intractable epileptic spasms that were diagnosed as infantile epileptic spasms syndrome, who underwent endoscopic total CC via a posterior approach to control her seizures (Video 1).
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Corpo Caloso , Neuroendoscopia , Humanos , Feminino , Neuroendoscopia/métodos , Corpo Caloso/cirurgia , Pré-Escolar , Epilepsia Resistente a Medicamentos/cirurgiaRESUMO
Hemispherotomy is a surgical procedure aimed at the treatment of hemispheric epilepsy. Hemispherotomy disconnects the commissural fibers, projecting fibers, and limbic system while preserving most of the brain parenchyma, unlike conventional hemispherectomy.1 Hemispherotomy is one of most complicated operations currently used in epilepsy surgery and requires a high-level understanding of the intrinsic neuroanatomy. Generally, 2 main techniques are applied in hemispherotomy: lateral hemispherotomy and vertical hemispherotomy.2,3 Vertical hemispherotomy was developed after the lateral technique. Despite a deeper, narrower surgical corridor, vertical hemispherotomy requires a smaller craniotomy and a shorter disconnection line and allows easier disconnection of the insula than the lateral approach.1 In performing vertical hemispherotomy, 2 options are available: the interhemispheric approach4 and the parasagittal approach.3 With the parasagittal approach, the lateral ventricle is opened and entered via superior frontal gyrus. To ease the surgery and minimize the operation time, we apply "partial superior frontal gyrus removal." This procedure is useful to widen a shallower surgical field while skipping the need for interhemispheric dissection. Total callosotomy is then performed via the lateral ventricle. To disconnect limbic system, we used the technique following the falx and tentorial edge as a landmark.5 Here, we present our surgical steps for parasagittal hemispherotomy in a 5-month-old girl diagnosed as hemimegalencephaly (Figures 1-8).
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Hemisferectomia , Humanos , Hemisferectomia/métodos , Feminino , Epilepsia/cirurgia , Corpo Caloso/cirurgia , Corpo Caloso/diagnóstico por imagemRESUMO
Miller-Dieker syndrome (MDS) is characterized by facial abnormalities and lissencephaly and is caused by a microdeletion in the region containing the LIS1 gene at chromosome 17p13.3. We report a case in which postnatal neuroimaging revealed severe lissencephaly. A 9-month-old boy presented with infantile spasms syndrome. Because of the refractory course of seizures and continued poor vitality, total corpus callosotomy was performed at 28 months of age. Intraoperative electroencephalogram (EEG) showed that the bilateral synchronous epileptiform discharges disappeared immediately after the disconnection. Postoperatively, the epileptic spasms (ES) in clusters disappeared, and single ES followed by focal seizures became the main symptom. The patient smiled more and became more responsive to stimuli. Postoperative scalp interictal EEG showed desynchronized multifocal spike and wave discharges with a marked decrease in the bilateral synchronous spike and wave discharges. Our findings suggest that the corpus callosum is involved in the mechanism ES in clusters in MDS-associated lissencephaly, and total callosotomy could be a therapeutic option.
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BACKGROUND: Seizure onset pattern (SOP) represents an alteration of electroencephalography (EEG) morphology at the beginning of seizure activity in epilepsy. With stereotactic electroencephalography (SEEG), a method for intracranial EEG evaluation, many morphological SOP classifications have been reported without established consensus. These inconsistent classifications with ambiguous terminology present difficulties to communication among epileptologists. METHODS: We reviewed SOP in SEEG by searching the PubMed database. Reported morphological classifications and the ambiguous terminology used were collected. After thoroughly reviewing all reports, we reconsidered the definitions of these terms and explored a more consistent and simpler morphological SOP classification. RESULTS: Of the 536 studies initially found, 14 studies were finally included after screening and excluding irrelevant studies. We reconsidered the definitions of EEG onset, period for determining type of SOP, core electrode and other terms in SEEG. We proposed a more consistent and simpler morphological SOP classification comprising five major types with two special subtypes. CONCLUSIONS: A scoping review of SOP in SEEG was performed. Our classification may be suitable for describing SOP morphology.
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Eletroencefalografia , Convulsões , Técnicas Estereotáxicas , Humanos , Convulsões/classificação , Convulsões/fisiopatologia , Convulsões/diagnóstico , Convulsões/patologia , Eletroencefalografia/métodos , Eletrocorticografia/métodosRESUMO
OBJECTIVE: Awake craniotomy (AWC) allows intraoperative evaluation of functions involving the cortical surface and subcortical fibers. In epilepsy surgery, indications for and the role of AWC have not been established because evaluation with intracranial electrodes is considered the gold standard. We report herein our case series of patients who underwent AWC in epilepsy surgery and propose the scenarios for and roles of AWC. METHODS: Patients who underwent AWC in epilepsy surgery at our institutions between 2014 and 2023 were included. Information about age, sex, etiology, location of epileptogenicity, seizure type, use of intracranial electrode placement, surgical complications, neurological deficits, additional surgery, and seizure outcomes was reviewed. Following a diagnostic and treatment flow for epilepsy surgery, we clarified three different scenarios and roles for AWC. RESULTS: Ten patients underwent AWC. Three patients underwent AWC after non-invasive evaluations. Two patients underwent AWC after intracranial evaluation with stereotactic electroencephalography (SEEG). Five patients underwent AWC after intracranial evaluation with subdural grid electrodes (SDG). Among these, two patients were initially evaluated with SEEG and with SDG thereafter. One patient reported slight numbness in the hand, and one patient showed slight cognitive decline. Seizure outcomes according to the Engel outcome scale were class 1A in three patients, IIA in two patients, IIIA in four patients, and IVA in one patient. CONCLUSIONS: AWC can be used for purposes of epilepsy surgery in different situations, either immediately after non-invasive studies or as an additional invasive step after invasive monitoring with either SEEG or SDG. The application of AWC should be individualized according to each patient's specific characteristics.
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OBJECTIVE: Corpus callosotomy (CC) is one of the palliative epilepsy surgical procedures available for nonlesional generalized epilepsy, but it is more invasive than other palliative surgical procedures. The main challenge is proper selection of suitable patients for CC. Coherence analysis is a method for evaluating brain connectivity, but the correlation between preoperative coherence and surgical outcomes has not previously been clarified. The authors aimed to evaluate correlations between preoperative interhemispheric coherence and surgical outcome in patients with nonlesional generalized epilepsy. METHODS: This retrospective study investigated patients with nonlesional generalized epilepsy who underwent total CC. The authors collected data for patients with good seizure outcome (Oguni classification A or B) and bad seizure outcome (Oguni classification D). For coherence analysis, the authors selected a period without interictal discharges. Preoperative interhemispheric coherence values from 8 pairs of symmetrically opposite scalp electrodes were computed across 5 frequency bands. Then, the authors evaluated correlations between coherence and surgical outcomes. RESULTS: Forty patients were included (19 males and 21 females). The mean (range) age at the time of surgery was 5.1 (1-18) years. Seizure outcomes were good in 15 patients and bad in the other 25 patients. Age at onset of epilepsy, duration of epilepsy before surgery, age at time of surgery, and presence of epileptic spasm did not differ significantly between patients with good and bad seizure outcomes (p = 0.36, p = 0.14, p = 0.10, and p = 0.20, respectively). Significant correlations were identified between higher Fp1-Fp2 interhemispheric coherence values in the delta, theta, and alpha frequency bands and bad surgical outcomes (p = 0.0397, p = 0.0322, and p = 0.0476, respectively). The receiver operating characteristic curves of the Fp1-Fp2 coherence values in these frequency bands showed areas under the curve of 67%, 69%, and 67%, respectively. The optimal cutoff values for Fp1-Fp2 interhemispheric coherence to predict surgical outcomes were 55.6 for delta (66.7% sensitivity and 72.0% specificity), 55.9 for theta (60.0% sensitivity and 76.0% specificity), and 50.3 for alpha (53.3% sensitivity and 84.0% specificity). CONCLUSIONS: This is the first study to identify potential predictive factors for surgical outcomes based on preoperative interhemispheric coherence in nonlesional generalized epilepsy. Higher coherence between Fp1-Fp2 in the delta, theta, and alpha frequencies correlated with bad seizure outcome after CC.
RESUMO
Magnetoencephalography (MEG) provides crucial information in diagnosing focal epilepsy. However, dipole estimation, a commonly used analysis method for MEG, can be time-consuming since it necessitates neurophysiologists to manually identify epileptic spikes. To reduce this burden, we developed the automatic detection of spikes using deep learning in single center. In this study, we performed a multi-center study using six MEG centers to improve the performance of the automated detection of neuromagnetically recorded epileptic spikes, which we previously developed using deep learning. Data from four centers were used for training and evaluation (internal data), and the remaining two centers were used for evaluation only (external data). We used a five-fold subject-wise cross-validation technique to train and evaluate the models. A comparison showed that the multi-center model outperformed the single-center model in terms of performance. The multi-center model achieved an average ROC-AUC of 0.9929 and 0.9426 for the internal and external data, respectively. The median distance between the neurophysiologist-analyzed and automatically analyzed dipoles was 4.36 and 7.23 mm for the multi-center model for internal and external data, respectively, indicating accurate detection of epileptic spikes. By training data from multiple centers, automated analysis can improve spike detection and reduce the analysis workload for neurophysiologists. This study suggests that the multi-center model has the potential to detect spikes within 1 cm of a neurophysiologist's analysis. This multi-center model can significantly reduce the number of hours required by neurophysiologists to detect spikes.