Posterior cerebral artery stenosis related to implanted intracranial electrodes for temporal lobe epilepsy: A case report.

INTRODUCTION: Intracranial electroencephalography is a crucial diagnostic technique for epilepsy surgery, though it is associated with a range of complications, including infection, intracranial hemorrhage, increased intracranial pressure, and cerebral infarction. This case study presents an uncommon occurrence of stenosis of the left posterior cerebral artery (PCA) following intracranial electrode implantation. CASE PRESENTATION: A woman in her thirties with drug-resistant focal impaired awareness seizures underwent implantation of subdural and depth electrodes on the bilateral temporal lobes to lateralize seizure onset. A left anterior-temporal lobectomy was performed based on the evaluation results. Following the resection of the hippocampus, stenosis of the left PCA, with a pinched appearance, was observed. Postoperatively, extensive cerebral edema in the bilateral temporal lobes and a defect in the left PCA were detected on magnetic resonance (MR) imaging. MR imaging performed the day after surgery showed cerebral infarction in the left medial temporal lobe and left lateral thalamus. A video review indicated that surgical manipulation was not the cause of vascular stenosis. MR angiography one week later confirmed the recanalization of the PCA. DISCUSSION: We surmised that the subdural electrodes inserted along the middle skull base might have induced the PCA stenosis or spasms. The patient did not experience any significant sequelae, with no episodes of seizures for more than five years after surgery. CONCLUSION: It is essential to note that subdural grid electrodes placed in the medial temporal lobe can cause vascular stenosis, albeit with an extremely rare occurrence.

The collagen matrix dural substitute graft prevents postoperative cerebrospinal fluid leakage after ventriculoperitoneal shunt surgery in patients aged <1 year.

Background: Cerebrospinal fluid (CSF) leakage is a common complication of ventriculoperitoneal shunt (VPS) and has the potential to induce shunt infection. Especially in infants and children, these are serious complications. DuraGen is a collagen matrix dural substitute used to reduce the risk of CSF leakage in various neurosurgeries. We report our VPS procedure with DuraGen for preventing postoperative CSF leakage in patients aged <1 year. Methods: We used DuraGen to prevent postoperative CSF leakage in six VPS surgeries. Antibiotic-impregnated shunt catheters and programmable valves with anti-siphon devices were also used in all cases. DuraGen was placed inside and atop the burr hole. All cases had an initial shunt pressure of 5 cmH2O. Fibrin glue was not used. Results: The patients underwent follow-up for a year after VPS surgery. There was no postoperative subcutaneous CSF collection or leakage after all six VPS surgeries. Furthermore, no postoperative shunt infections or DuraGen-induced adverse events were noted. Conclusion: We speculate that DuraGen has a preventive effect on postoperative CSF leakage in VPS cases aged <1 year.

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.

Effects of Vagus Nerve Stimulation on Sustained Seizure Clusters: A Case Report.

Seizure clusters (SCs) are acute repetitive seizures with acute episodes of deterioration during seizure control. SCs can be defined as a series of grouped seizures with short interictal periods. Vagus nerve stimulation (VNS) is a treatment option for drug-resistant epilepsy. We present a case where VNS suppressed epileptic SCs, which had persisted for several months. A 13-year-old boy with congenital cerebral palsy and mental retardation had drug-resistant epilepsy with daily jerking movements and spasms in both sides of his body. The seizures were often clustered, and he experienced two sustained SC episodes that persisted for a few months even with prolonged use of continuous intravenous midazolam (IV-MDZ). The patient underwent VNS device placement at the second sustained SC and rapid induction of VNS. Because the tapering of IV-MDZ did not exacerbate the SC, midazolam was discontinued 4 weeks after VNS initiation. Non-refractory SCs also disappeared 10 months after VNS. The seizure severity was improved, and the frequency of seizures reduced from daily to once every few months. The epileptic activity on electroencephalography (EEG) significantly decreased. This case highlights VNS as an additional treatment option for SC. VNS may be a therapeutic option if SC resists the drugs and sustains. Additional studies are necessary to confirm our findings and to investigate how device implantation and stimulation parameters affect the efficacy of VNS.

Surgical Treatment for Mesial Temporal Lobe Epilepsy Accompanied with Neuro-Behçet's Disease: A Case Report.

Behçet's disease (BD) is a rare chronic inflammatory disease associated with systemic vasculitis. Involvement of the nervous system in BD is called neuro-BD (NBD). Epilepsy related to NBD is uncommon but responds well to anti-epileptic drugs. We present a case of NBD with drug-resistant mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS). The patient presented with headache, dizziness, disorientation, and generalized seizures. Magnetic resonance imaging (MRI) identified pontine lesions. Chronic inflammation was suspected, and steroid pulse therapy improved his symptoms. He relapsed 1 year after onset and was diagnosed with NBD. MRI revealed bilateral mesial temporal lesions, with the right being edematous and the left atrophic. NBD was controlled by steroid and immunosuppressive medication. Three years after the onset of NBD, the patient suffered MTLE, and MRI suggested left hippocampal atrophy. His seizures became drug-resistant and surgical therapy was considered 12 years after NBD onset. Pre-surgical MRI clearly showed left HS. After evaluations, the patient had left anterior temporal lobectomy (ATL) 13 years after NBD onset under stable NBD. The patient was seizure-free for > 2 years after surgery. Surgery will be an effective treatment for drug-resistant MTLE with HS even in patients with NBD, of course the effects of surgical intervention should be considered.

A tailored next-generation sequencing panel identified distinct subtypes of wildtype IDH and TERT promoter glioblastomas.

Central nervous system tumors are classified based on an integrated diagnosis combining histology and molecular characteristics, including IDH1/2 and H3-K27M mutations, as well as 1p/19q codeletion. Here, we aimed to develop and assess the feasibility of a glioma-tailored 48-gene next-generation sequencing (NGS) panel for integrated glioma diagnosis. We designed a glioma-tailored 48-gene NGS panel for detecting 1p/19q codeletion and mutations in IDH1/2, TP53, PTEN, PDGFRA, NF1, RB1, CDKN2A/B, CDK4, and the TERT promoter (TERTp). We analyzed 106 glioma patients (grade II: 19 cases, grade III: 23 cases, grade IV: 64 cases) using this system. The 1p/19q codeletion was detected precisely in oligodendroglial tumors using our NGS panel. In a cohort of 64 grade Ⅳ gliomas, we identified 56 IDH-wildtype glioblastomas. Within these IDH-wildtype glioblastomas, 33 samples (58.9%) showed a mutation in TERTp. Notably, PDGFRA mutations and their amplification were more commonly seen in TERTp-wildtype glioblastomas (43%) than in TERTp-mutant glioblastomas (6%) (P = .001). Hierarchical molecular classification of IDH-wildtype glioblastomas revealed 3 distinct groups of IDH-wildtype glioblastomas. One major cluster was characterized by mutations in PDGFRA, amplification of CDK4 and PDGFRA, homozygous deletion of CDKN2A/B, and absence of TERTp mutations. This cluster was significantly associated with older age (P = .021), higher Ki-67 score (P = .007), poor prognosis (P = .012), and a periventricular tumor location. We report the development of a glioma-tailored NGS panel for detecting 1p/19q codeletion and driver gene mutations on a single platform. Our panel identified distinct subtypes of IDH- and TERTp-wildtype glioblastomas with frequent PDGFRA alterations.

[Anti-diabetic effect of ethanol extract of Cyclolepis genistoides D. Don (Palo azul), made in Paraguay].

Extract of Cyclolepis genistoides D. Don (vernacular name Palo azul; Palo) are traditionally consumed in the Republic of Paraguay in South America for the treatment of diabetes and kidney disease, and is sold in Japan as dietary supplement. This study aimed to elucidate the mechanism of anti-diabetes activity of Palo, especially focused on insulin resistance. Palo promoted adipocytes differentiation and regulated adipokine profiles in 3T3-L1 adipocytes by modulation of PPARγ, a major regulator of adipose differentiation. Human adipocyte showed almost similar profile with 3T3-L1 against Palo treatment. Furthermore, Palo treatment (250 or 1000 mg/kg) was performed with C57BL/6J mice for 14 weeks, being fed high-fat-diet (HFD60) simultaneously. Palo 250 mg/kg exhibited a tendency to decrease subcutaneous adipose volume along with increase of PPARγ and its target, adiponectin mRNA expression. In addition, as the other insulin targeted cell, effect on muscle differentiation was examined. Palo increased differentiation of C2C12 mouse muscle myoblasts by increase of IGF-1, myogenin, and myosine heavy chain (MHC) as well as 5'-AMP-activated protein kinase (AMPK) activation. Palo subsequently promoted myotube formation under differentiation condition. From the above, it was clarified that Palo acts variously on the differentiation and maturation of both adipocytes and muscle cells, and from the viewpoint of the regulatory mechanism for adipocytes, PPARγ-inducing action was shown to be a mechanism that acts across species.

Microvascular Decompression in Patients Aged 30 Years or Younger.

OBJECTIVE: The aim of this study was to identify the etiology of hemifacial spasm (HFS) and trigeminal neuralgia (TN) in patients aged 30 years or younger and to examine the efficacy of microvascular decompression (MVD). PATIENTS AND METHODS: Between 1996 and 2012, 228 HFS and 190 TN patients underwent MVD at Atsuchi Neurosurgical Hospital. Of these, 7 patients were 30 years of age or younger at the time of treatment (HFS: n = 6, TN: n = 1). Assessments were based on their medical history and on magnetic resonance imaging, magnetic resonance angiography, surgical, and follow-up findings. RESULTS: The age of the 6 HFS patients ranged from 23 to 30 years (mean 27.8 ± 1.8 [standard deviation] years) at the time of surgery; the earliest symptom onset was in an 11-year-old boy. We noted vascular variations in 5 patients, a duplicate posterior inferior cerebellar artery in 2 patients, a short basilar artery in 1 patient, and an aberrant arterial course in 2 patients. At the latest follow-up, 1-69 months after MVD, 5 of the HFS patients were asymptomatic and the 6th had mild residual symptoms. A 23-year-old TN female underwent straightening of the trigeminal nerve by separation of a thickened arachnoid membrane from the nerve and dislocation of a small branch of the superior cerebellar artery from the distal end of the root exit zone. While she continued to experience occasional facial pain 48 months after the operation, she required no medication because surgery yielded significant pain amelioration. CONCLUSION: Although the pathogenesis of early-onset HFS and TN remains unclear, our findings suggest that vascular variations may be related to the etiology of vascular compression symptoms in patients with HFS or TN. MVD was useful for the treatment of neurovascular compression symptoms in young patients.

Postoperative Changes in Metabolic Parameters of Patients with Surgically Controlled Acromegaly: Assessment of New Stringent Cure Criteria.

The criteria for surgical cure of acromegaly have become more stringent during the past decades and a change from Cortina to new consensus criteria has recently been proposed. However, the superiority of the new consensus over Cortina criteria with respect to postoperative metabolic parameters remains to be ascertained. We retrospectively assessed metabolic parameters, the body habitus, and other health-related parameters of 48 patients with surgically controlled acromegaly who met the Cortina criteria [normalized insulin-like growth factor-1 (IGF-1) level and nadir growth hormone (GH) level <1.0 ng/ml during postoperative oral glucose tolerance test]. The 48 patients were divided into two groups. Group A (n = 33) met the new consensus criteria (normalized IGF-1 and nadir GH level <0.4 ng/ml). Group B (n = 15) met Cortina criteria, but their nadir GH ranged from 0.4 to 1.0 ng/ml. In both groups, the level of triglyceride and homeostasis model assessment-insulin resistance (HOMA-IR) was significantly decreased 1 year after the operation (P < 0.05). High-density lipoprotein cholesterol showed a significant increase only in group B (P = 0.02). However, the two groups did not differ with respect to the postoperative improvement rate of these parameters and the other health-related parameters including body mass index, blood pressure, anterior pituitary function, and self-estimated quality of life scale. In conclusion, our findings show that with respect to changes in metabolic parameters and the body habitus assessed 1 year after surgery, the stricter consensus criteria seemed not to be superior to Cortina criteria.

Application of Rubber Band with Hooks on Both Ends for Vagus Nerve Stimulator Implantation.

BACKGROUND: Vagus nerve stimulation (VNS) is a valuable therapeutic option for many types of drug-resistant epilepsy. Muscle hooks and carotid endarterectomy rings have been used for cervical delamination preceding the implantation of stimulation electrodes. The attachment on both sides of a rubber band of Kamiyama-style hanging needles, as are used for scalp and dural retraction during craniotomy, yields a useful tool for VNS implantation. Here we report our experience with this method. METHODS: We present our method using a rubber band plus hooks and a review of 21 consecutive patients who underwent VNS implantation using our rubber band-plus-hooks method. RESULTS: None of the 21 patients experienced intraoperative or perioperative complications. Hooks placed in connective tissue around the common carotid artery and jugular vein raised the vagus nerve by elevating the carotid sheath. A single surgeon was able to perform all cervical manipulations under a surgical microscope. The average operation time in this series of 21 patients was 137 minutes. CONCLUSIONS: The use of hooks attached to both sides of a rubber band rendered VNS implantation safer by lifting the vagus nerve and standardizing the procedure.

Long-term outcomes of epilepsy surgery in 85 pediatric patients followed up for over 10 years: a retrospective survey.

OBJECTIVE The aim of this study was to investigate the treatment outcomes and social engagement of patients who had undergone pediatric epilepsy surgery more than 10 years earlier. METHODS Between 1983 and 2005, 110 patients younger than 16 years underwent epilepsy surgery at the National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders. The authors sent a questionnaire to 103 patients who had undergone follow-up for more than 10 years after surgery; 85 patients (82.5%) responded. The survey contained 4 categories: seizure outcome, use of antiepileptic drugs, social participation, and general satisfaction with the surgical treatment (resection of the epileptic focus, including 4 hemispherectomies). The mean patient age at the time of surgery was 9.8 ± 4.2 (SD) years, and the mean duration of postoperative follow-up was 15.4 ± 5.0 years. Of the 85 patients, 79 (92.9%) presented with a lesional pathology, such as medial temporal sclerosis, developmental/neoplastic lesions, focal cortical dysplasia, and gliosis in a single lobe. RESULTS For 65 of the 85 responders (76.5%), the outcome was recorded as Engel Class I (including 15 [93.8%] of 16 patients with medial temporal sclerosis, 20 [80.0%] of 25 with developmental/neoplastic lesions, and 27 [73.0%] of 37 with focal cortical dysplasia). Of these, 29 (44.6%) were not taking antiepileptic drugs at the time of our survey, 29 (44.6%) held full-time jobs, and 33 of 59 patients (55.9%) eligible to drive had a driver's license. Among 73 patients who reported their degree of satisfaction, 58 (79.5%) were very satisfied with the treatment outcome. CONCLUSIONS The seizure outcome in patients who underwent resective surgery in childhood and underwent followup for more than 10 years was good. Of 85 respondents, 65 (76.5%) were classified in Engel Class I. The degree of social engagement was relatively high, and the satisfaction level with the treatment outcome was also high. From the perspective of seizure control and social adaptation, resective surgery yielded longitudinal benefits in children with intractable epilepsy, especially those with a lesional pathology in a single lobe.

Vagus nerve stimulation for genetic epilepsy with febrile seizures plus (GEFS+) accompanying seizures with impaired consciousness.

Genetic epilepsy with febrile seizures plus (GEFS+) is characterized by childhood-onset epilepsy syndrome. It involves febrile seizures and a variety of afebrile epileptic seizure types within the same pedigree with autosomal-dominant inheritance. Approximately 10% of individuals with GEFS+ harbor SCN1A, a gene mutation in one of the voltage-gated sodium channel subunits. Considerably less common are focal epilepsies including complex partial seizures. We report vagus nerve stimulation (VNS) in a 6-year-old girl with GEFS+ who exhibited refractory generalized tonic-clonic seizures and complex partial seizures.

Radiologic and histologic features of the T2 hyperintensity rim of meningiomas on magnetic resonance images.

A hyperintensity rim is often seen at the brain-tumor interface of meningiomas upon T2-weighted (T2WI) magnetic resonance imaging (MRI), and it is referred to as the cerebrospinal fluid (CSF) space; however, the true nature of the rim remains unclear. We surveyed the MRI findings and the histopathologic characteristics of such rims. Our study population consisted of 53 consecutive patients who underwent meningioma removal at our hospital. The intensity of the rim on MRI scans obtained with different imaging sequences was assessed in all patients. We used 22 tumors for histopathologic investigation: tissue samples were acquired from both the tumor surface and from a deep intratumoral site. Of the 53 meningiomas, 37 (69.8%) manifested a hyperintensity rim on T2WI (T2-rim). The other 16 showed neither a hyperintense nor a hypointense rim on their T2WI. An enhancement effect corresponding to the rim was observed in 28 of the 37 (75.7%) T2-rim positive tumors. While 9 among the 37 tumors with a T2-rim (24.3%) did not show rim enhancement, they showed low intensity on fluid-attenuated inversion recovery (FLAIR) images. The microvascular density in the tumor capsule was significantly greater in the 12 T2-rim and rim enhancement positive tumors than in 10 tumors that were T2-rim negative or T2-rim positive, but rim enhancement-negative ( p < 0.001, Mann-Whitney U test). We found that 75.7% of T2 hyperintense rims that were detected at the brain-meningioma interface reflected a microvascular-rich capsule layer, rather than the CSF space.

Determination of hemispheric language dominance using functional magnetic resonance imaging and the Shiritori (Japanese word chain) task in patients with epilepsy: Comparison with the Wada test.

PURPOSE: The Wada test has been the gold standard for determining hemispheric language dominance (HLD) in the presurgical evaluation of patients scheduled for neurosurgical procedures. As it poses inherent risks associated with intra-arterial catheter techniques and as it occasionally fails to indicate language dominance, an alternative reliable test is needed. We quantitatively assessed the results of functional magnetic resonance imaging (fMRI) using the Shiritori task, a Japanese word chain, to identify the threshold for correctly predicting HLD. METHODS: The subjects were 28 patients with intractable epilepsy scheduled to undergo the Wada test and focus resection. We set the region of interest (ROI) on the bilateral Brodmann areas 44 and 45 (BA 44 and 45). To compare the functional activity at both ROIs we calculated the language laterality index (LI) using the formula: [VL-VR]/[VL+VR]×100, where VL and VR indicated the number of activated voxels in the left and right ROIs, respectively. RESULTS: As 2 patients were excluded due to the lack of activation in either ROI, the final study population consisted of 26 patients. By the Wada test, HLD was left in 20, right in 3, and equivocal in 3. At a cut-off of LI+50, the predictive sensitivity and specificity for left HLD were 85% (17/20) and 100%; right HLD was predicted in a single patient (sensitivity 33.3%, specificity 100%). CONCLUSION: The fMRI using the Shiritori task showed good activation in ROI of BA 44 and 45. At a cut-off of LI+50, LI of BA 44 and 45 predicted HLD identified by the Wada test with high specificity.

Mesial temporal lobe epilepsy with no specific histological abnormality: a distinct surgically remediable syndrome.

PURPOSE: The purposes of the study were twofold: to clarify the clinical features and surgical outcome of mesial temporal lobe epilepsy (MTLE) with no specific histological abnormality and to determine the optimal surgical strategy. METHODS: Twelve patients who met the following criteria were included: (1) normal preoperative MRI; (2) intracranial EEG findings consistent with mesial temporal onset of seizures; (3) selective amygdalohippocampectomy (AHE) was performed, and the patient was followed for more than 2years postoperatively; and (4) hippocampal histopathology was nonspecific. Clinical characteristics, intracranial EEG findings, and postoperative seizure outcome were examined. These twelve patients were compared with twenty-one patients with MTLE with unilateral hippocampal sclerosis (HS) on MRI who underwent intracranial EEG before resection (control group). RESULTS: In patients with MTLE with no specific histological abnormality, the age at onset was significantly higher, the history of febrile seizures was significantly less frequent, and preoperative IQ score was significantly higher than that in the control group. The proportion of patients with bitemporal independent and/or nonlateralizing seizure onset on intracranial EEG was 50% in patients with MTLE with nonspecific histopathology and was significantly higher than that in the control group. Seizure outcome was classified as Engel class I in seven patients, class II in three, class III in one, and class IV in one. Seizure outcome was favorable even in three patients with seizures originating more frequently from the side contralateral to the resected side. CONCLUSIONS: Mesial temporal lobe epilepsy with no specific histological abnormality is a clinical entity distinctly different from MTLE with HS. Bitemporal independent and/or nonlateralizing seizure onset on intracranial EEG is very common. Although the presence of lateral temporal and/or extratemporal epileptogenicity should always be kept in mind, postoperative seizure outcome after AHE is favorable even in cases with bitemporal independent and/or nonlateralizing seizure onset.