Characteristics of malignant brain tumor-associated epileptic spasms.

Although epilepsy is the most common comorbidity of brain tumors, epileptic spasms rarely occur. Brain tumors associated with epileptic spasms are mostly low-grade gliomas. To date, few studies in the literature have reported on malignant (Grades 3-4) brain tumors associated with epileptic spasms. Thus, we aimed to investigate the characteristics of malignant brain tumor-associated epileptic spasms. We retrospectively reviewed patients with malignant brain tumors and epileptic spasms in our institution. Data on demographics, tumor histology, magnetic resonance imaging, epileptic spasm characteristics, electroencephalography, and treatment responsiveness were also collected. Six patients were included. In all cases, the brain tumors occurred in infancy in the supratentorial region and epileptic spasm onset occurred after the completion of brain tumor treatment. Anti-seizure medication did not control epileptic spasms; two patients were seizure-free after epileptic surgery. Although all patients had developmental delays caused by malignant brain tumors and their treatment, developmental regression proceeded after epileptic spasm onset. Two patients who achieved seizure-free status showed improved developmental outcomes after cessation of epileptic spasms. This is the first report of the characteristics of malignant brain tumor-associated epileptic spasms. Our report highlights a difficulties of seizure control and possibillity of efficacy of epileptic surgery in this condition. In malignant brain tumor-associated epileptic spasms, it is important to proceed with presurgical evaluation from an early stage, bearing in mind that epileptic spasms may become drug-resistant.

Total Corpus Callosotomy via Posterior Approach with Endoscopic-Alone Technique.

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).

Successful Hemispherotomy in a Patient With 22q11.2 Deletion Syndrome Who Had Developmental and Epileptic Encephalopathy With Spike-and-Wave Activation During Sleep.

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.

Total callosotomy ameliorates epileptic activity and improves cognitive function in a patient with Miller-Dieker syndrome.

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.

Changes in interhemispheric coherence after total corpus callosotomy: a scalp EEG study in children with non-lesional generalized epilepsy.

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.

A scoping review of seizure onset pattern in SEEG and a proposal for morphological classification.

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.

Prediction of MGMT promotor methylation status in glioblastoma by contrast-enhanced T1-weighted intensity image.

Background: The study aims to explore MRI phenotypes that predict glioblastoma's (GBM) methylation status of the promoter region of MGMT gene (pMGMT) by qualitatively assessing contrast-enhanced T1-weighted intensity images. Methods: A total of 193 histologically and molecularly confirmed GBMs at the Kansai Network for Molecular Diagnosis of Central Nervous Tumors (KANSAI) were used as an exploratory cohort. From the Cancer Imaging Archive/Cancer Genome Atlas (TCGA) 93 patients were used as validation cohorts. "Thickened structure" was defined as the solid tumor component presenting circumferential extension or occupying >50% of the tumor volume. "Methylated contrast phenotype" was defined as indistinct enhancing circumferential border, heterogenous enhancement, or nodular enhancement. Inter-rater agreement was assessed, followed by an investigation of the relationship between radiological findings and pMGMT methylation status. Results: Fleiss's Kappa coefficient for "Thickened structure" was 0.68 for the exploratory and 0.55 for the validation cohort, and for "Methylated contrast phenotype," 0.30 and 0.39, respectively. The imaging feature, the presence of "Thickened structure" and absence of "Methylated contrast phenotype," was significantly predictive of pMGMT unmethylation both for the exploratory (p = .015, odds ratio = 2.44) and for the validation cohort (p = .006, odds ratio = 7.83). The sensitivities and specificities of the imaging feature, the presence of "Thickened structure," and the absence of "Methylated contrast phenotype" for predicting pMGMT unmethylation were 0.29 and 0.86 for the exploratory and 0.25 and 0.96 for the validation cohort. Conclusions: The present study showed that qualitative assessment of contrast-enhanced T1-weighted intensity images helps predict GBM's pMGMT methylation status.

Neuroimaging of Brain Tumor Surgery and Epilepsy.

To make the best clinical judgements, surgeons need to integrate information acquired via multimodal imaging [...].

AI-based Virtual Synthesis of Methionine PET from Contrast-enhanced MRI: Development and External Validation Study.

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.

Use of an Endoscope Reduces the Size of Craniotomy Without Increasing Operative Time Compared With Conventional Microscopic Corpus Callosotomy.

BACKGROUND AND OBJECTIVES: Corpus callosotomy (CC) is an epilepsy surgery that disconnects the commissural fibers at the corpus callosum, a structure that often plays a key role in propagating seizure activity. CC is particularly beneficial in patients with drop attacks. Less invasive endoscopic surgeries have recently been introduced to some fields of neurosurgery but have not yet become common in epilepsy surgery. Endoscopic surgeries offer better visualization and require a smaller corridor than conventional microscopic surgeries. Here, we presented a case series comparing endoscopic CC with microscopic CC. METHODS: This 2-center retrospective study involved patients who underwent all types of CC (anterior, total, or posterior CC [pCC]) between January 2014 and May 2022. We excluded patients who underwent additional craniotomy for electrocorticography rather than CC, prior craniotomy, or CC without craniotomy. The primary outcomes were comparing size of craniotomy, operative time, and surgical complications between endoscopic CC and microscopic CC. RESULTS: We included 14 CCs in 11 patients in the endoscopic group and 58 CCs in 55 patients in the microscopic group. No significant difference in age was seen between groups. Craniotomies were significantly smaller in the endoscopic group for anterior (13.36 ± 1.31 cm 2 vs 27.55 ± 3.78 cm 2 ; P = .001), total (14.07 ± 2.54 cm 2 vs 26.63 ± 6.97 cm 2 ; P = .001), and pCC (9.44 ± 1.18 cm 2 vs 30.23 ± 10.76 cm 2 ; P = .002). Moreover, no significant differences in operative time (anterior CC [261 ± 53.11 min vs 298.73 ± 81.08 min, P = .226], total CC [339.5 ± 48.2 min vs 321.39 ± 65.98 min, P = .452], pCC [198 ± 24.73 min vs 242.5 ± 59.12 min, P = .240]), or complication rate were seen. CONCLUSION: Endoscopic CC is a promising technique requiring a smaller craniotomy than microscopic CC, without significantly increasing operative time or complication rate compared with microscopic CC.

Electrode Tip Shift During the Stereotactic Electroencephalography Evaluation Period with Boltless Suture Fixation.

BACKGROUND: Electrodes for stereotactic electroencephalography (SEEG) are typically fixed to the skull with anchor bolts. When anchor bolts are unavailable, electrodes have to be fixed using other methods, carrying the possibility of electrode shift. This study, therefore, evaluated the characteristics of electrode tip shift during SEEG monitoring in patients with electrodes fixed using the suture technique. METHODS: We retrospectively included patients who underwent SEEG implantation with suture fixation and evaluated the tip shift distance (TSD) of electrodes. Possible influences evaluated included: 1) implantation period, 2) lobe of entry, 3) unilateral or bilateral implantation, 4) electrode length, 5) skull thickness, and 6) scalp thickness difference. RESULTS: A total of 50 electrodes in 7 patients were evaluated. TSD was 1.4 ± 2.0 mm (mean ± standard deviation). Implantation period was 8.1 ± 2.2 days. Entry lobe was frontal for 28 electrodes and temporal for 22 electrodes. Implantation was bilateral for 25 electrodes and unilateral for 25 electrodes. Electrode length was 45.4 ± 14.3 mm. Skull thickness was 6.0 ± 3.7 mm. Scalp thickness difference was -1.5 ± 2.1 mm, which was found greater in temporal lobe entry compared with frontal lobe entry. According to univariate analyses, neither implantation period nor electrode length correlated with TSD. Multivariate regression analysis showed that only greater scalp thickness difference correlated significantly with greater TSD (P = 0.0018). CONCLUSIONS: Greater scalp thickness difference correlated with greater TSD. Surgeons need to consider the degree of scalp thickness difference and electrode shift when using suture fixation, especially with temporal lobe entry.

Intraparenchymal and Subarachnoid Hemorrhage in Stereotactic Electroencephalography Caused by Indirect Adjacent Arterial Injury: Illustrative Case.

The complication rate of stereotactic electroencephalography (SEEG) is generally low, but various types of postoperative hemorrhage have been reported. We presented an unusual hemorrhagic complication after SEEG placement. A 20-year-old man presented with suspected frontal lobe epilepsy. We implanted 11 SEEG electrodes in the bilateral frontal lobes and the left insula. Computed tomography after implantation showed intraparenchymal hemorrhage in the left temporal lobe and insula and subarachnoid hemorrhage in the left Sylvian cistern. Later, the point of vessel injury was revealed from the identification of a pseudoaneurysm, but this location was not along the planned or actual electrode trajectory. The cause of hemorrhage was suggested to be indirect injury from stretching of the arachnoid trabeculae by the puncture needle.

Frontal Encephalocele Plus Epilepsy: A Case Report and Review of the Literature.

An encephalocele is a pathological brain herniation caused by osseous dural defects. Encephaloceles are known to be regions of epileptogenic foci. We describe the case of a 44-year-old woman with refractory epilepsy associated with a frontal skull base encephalocele. Epilepsy surgery for encephalocele resection was performed; however, the epilepsy was refractory. A second epilepsy surgery for frontal lobectomy using intraoperative electroencephalography was required to achieve adequate seizure control. Previous reports have shown that only encephalocele resection can result in good seizure control, and refractory epilepsy due to frontal lobe encephalocele has rarely been reported. To the best of our knowledge, this is the first report of frontal encephalocele plus epilepsy in which good seizure control using only encephalocele resection was difficult to achieve. Herein, we describe the possible mechanisms of encephalocele plus epilepsy and the surgical strategy for refractory epilepsy with encephalocele, including a literature review.

[Surgical Treatment of Mesial Temporal Lobe Epilepsy Focusing on Anatomical Understanding].

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.

Comparing late-onset epileptic spasm outcomes after corpus callosotomy and subsequent disconnection surgery between post-encephalitis/encephalopathy and non-encephalitis/encephalopathy.

OBJECTIVE: We aimed to analyze the efficiency of corpus callosotomy (CC) and subsequent disconnection surgeries in patients with late-onset epileptic spasms (LOES) by comparing post-encephalitis/encephalopathy (PE) and non-encephalitis/encephalopathy (NE). We hypothesized these surgeries can control potential focal onset epileptic spasms (ES) in the NE group but not in the PE group. METHODS: We retrospectively included 23 patients (12 with PE and 11 with NE) who initially underwent CC and subsequent disconnection surgeries (five NE). We compared the clinical courses, seizure types, MRI, video-EEG, epilepsy surgery, and seizure outcomes between the two groups. RESULTS: The median age of LOES onset in the PE group was 2.8 (range 1.0-10.1 years) and 2.9 years (range 1.1-12.6) in the NE group. Bilateral MRI abnormalities were observed in both groups (PE, n = 12; NE, n = 3; P < 0.05). The PE group presented ES alone (n = 2), ES + focal seizures (FS) (n = 3), ES + generalized seizures (GS) (n = 3), and ES + FS + GS (n = 4) in addition to stimulus-induced startle seizures (SS) (n = 8) (mean 3.1 seizure types/patient). The NE group presented ES alone (n = 1), ES + FS (n = 2), and ES + FS + GS (n = 8) (mean 2.7 seizure types/patient). In the PE group, CC stopped ES (n = 1) and SS (n = 1) and achieved <50% SS (n = 3). In the NE group, CC achieved immediate ES-free status (n = 2) and < 50% ES (n = 1), and additional disconnection surgeries subsided all seizure types (n = 3) based on lateralized interictal/ictal EEG findings. LOES was significantly remitted by surgery in the NE group (6/11 [55%]) compared with the PE group (1/12 [8%]) (P < 0.05). SIGNIFICANCE: LOES is a drug-resistant, focal/generalized/unknown onset ES. Lateralization of ES in NE could be achieved after CC and eliminated by further disconnection surgeries because of potential focal onset ES. LOES in PE had little benefit from CC for generalized onset ES. However, CC might reduce SS in patients in the PE group with multiple seizure types.

Visualization of Resected Area in Endonasal Endoscopic Approach versus Transcranial Approach for Skull Base Meningiomas by Voxel-Based-Lesion Mapping.

BACKGROUND: We aimed to evaluate the resected area of endonasal endoscopic approach (EEA) and transcranial approach (TCA) for skull base meningiomas (SBMs) using voxel-based-lesion mapping and visualized the appropriate tumor location in each approach. METHODS: We retrospectively examined 182 patients with SBMs who underwent tumor resection in our hospital between 2014 and 2019. Pre- and post-operative SBMs were manually delineated on MRI to create the voxels-of-interest (VOIpre and VOIpost) and were registered onto the normalized brain (normalized VOIpre and normalized VOIpost). The resected map was created by subtracting normalized VOIpost from the normalized VOIpre divided by the number of cases. The resected maps of TCA and EEA were compared by subtracting them. RESULTS: Twenty patients underwent EEA and 135 patients underwent TCA. The tumor resected map demonstrated that the resected area of EEA frequently accumulated on the central skull base, while that of TCA accumulated near the central skull base. The border of both approaches matched the circle that connects neural foramens at the skull base. CONCLUSIONS: The resected area of SBMs by EEA and TCA was well visualized by voxel-based-lesion mapping. The circle connecting the neural foramens was the border of EEA and TCA.

Primary Experiences with Robot-assisted Navigation-based Frameless Stereo-electroencephalography: Higher Accuracy than Neuronavigation-guided Manual Adjustment.

The use of robot-assisted frameless stereotactic electroencephalography (SEEG) is becoming more common. Among available robotic arms, Stealth Autoguide (SA) (Medtronic, Minneapolis, MN, USA) functions as an optional instrument of the neuronavigation system. The aims of this study were to present our primary experiences with SEEG using SA and to compare the accuracy of implantation between SA and navigation-guided manual adjustment (MA). Seventeen electrodes from two patients who underwent SEEG with SA and 18 electrodes from four patients with MA were retrospectively reviewed. We measured the distance between the planned location and the actual location at entry (De) and the target (Dt) in each electrode. The length of the trajectory did not show a strong correlation with Dt in SA (Pearson's correlation coefficient [r] = 0.099, p = 0.706) or MA (r = 0.233, p = 0.351). De and Dt in SA were shorter than those in MA (1.99 ± 0.90 vs 4.29 ± 1.92 mm, p = 0.0002; 3.59 ± 2.22 vs 5.12 ± 1.40 mm, p = 0.0065, respectively). SA offered higher accuracy than MA both at entry and target. Surgical times per electrode were 38.9 and 32 min in the two patients with SA and ranged from 51.6 to 88.5 min in the four patients with MA. During the implantation period of 10.3 ± 3.6 days, no patients experienced any complications.

A case of focal cortical dysplasia type IIa with pathologically suspected bilateral Rasmussen syndrome.

BACKGROUND: Rasmussen syndrome (RS) is a rare neurological disorder characterized by unilateral chronic inflammation, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. There has been no detailed pathological evaluation or finding, including focal cortical dysplasia, for bilateral RS. CASE REPORT: A 13-year-old boy presented with status epilepticus with focal to bilateral tonic clonic seizure starting from the left upper limb. At the age of 15, epilepsia partialis continua of the right face and upper extremities appeared, and MRI showed hemispheric abnormal signal intensities with left frontal lobe predominance. Three months later, MRI showed extensive abnormal signal intensities in the right occipitoparietal and left temporal lobes. Tacrolimus was useful in preventing recurrence. Because the seizures were intractable, a corpus callosotomy was performed at 16 years along with a concurrent brain biopsy from the bilateral lateral frontal cortices. We detected dysmorphic neurons in addition to inflammatory changes suspicious for RS, leading to a diagnosis of focal cortical dysplasia (FCD) type Ⅱa and suspected bilateral RS. Total callosotomy and vagus nerve stimulation were not sufficiently effective. CONCLUSIONS: In bilateral RS, FCD may be present in both cerebral hemispheres. In the current case, an autoimmune response to dysmorphic neurons may have contributed to the pathogenesis of intense inflammation.