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We evaluated the effect of early surgical intervention on the change in memory performance of patients with low-grade brain tumors associated with epilepsy. Twenty-three adult patients with low-grade brain tumors and epilepsy who underwent surgery at our institution between 2010 and 2019 were included. The Wechsler Memory Scale-Revised (WMS-R) was used to assess cognitive memory performance. Memory performance before and after surgery was retrospectively evaluated. In addition, the relationships among preoperative memory function, postoperative seizure outcome, preoperative seizure control, temporal lobe lesion, and change in memory function were examined. There were statistically significant improvements from median preoperative to postoperative WMS-R subscale scores for verbal memory, general memory, and delayed recall (p0.001, p0.001, and p=0.0055, respectively) regardless of preoperative sores and tumor location. Good postsurgical seizure control was associated with significant improvements in postoperative WMS-R performance. Our results indicated that early surgical intervention might improve postoperative memory function in patients with low-grade brain tumors and epilepsy.

Background The physiological and pathological significance of the arachnoid membrane (AM) is still unknown. In this study, we investigated various characteristics of the AM, focusing on the influence of inflammation and fibrosis. Methods Small pieces of AM sample were obtained during neurosurgical procedures from 74 cases. The clinical and pathological characteristics of the hyperplastic AM group (≥ 50 μm) and the non-hyperplastic AM group ( Results The median age of the patients in hyperplastic AM group was significantly older than that of the non-hyperplastic AM group. Moreover, the number of fibroblasts, CD68+ cells, CD86+ cells, and CD206+ cells in the hyperplastic AM group was significantly higher than that in the non-hyperplastic AM group. The AM thickness was significantly correlated to age and number of fibroblasts, CD68+ cells, CD86+ cells, and CD206+ cells. The thickness of the AM was significantly correlated to the messenger RNA expression levels of VEGFα (ρ = 0.337), and the VEGFα expression levels were significantly correlated with TGFβ and TNFα. Conclusions The AM hyperplasia was influenced by aging and could be a result of inflammation and fibrosis through cytokine secretion from the inflammatory cells and fibroblasts in the AM.

This study investigated the networks originating from frontal eye fields (FEFs) using electric cortical stimulation and diffusion tensor imaging (DTI). Seven patients with intractable focal epilepsy, in which FEFs were identified by electrical cortical stimulation, were enrolled in this study. Electric stimulation at 50 Hz was applied to the electrodes for functional mapping. DTI was used to identify the subcortical fibers originating from the FEFs with two regions of interests (ROIs) in the FEF and contralateral paramedian pontine reticular formation (PPRF). FEFs were found in the superior precentral sulcus (pre-CS) in six patients and superior frontal gyrus (SFG) in three patients. DTI detected fibers connecting FEFs and contralateral PPRFs, passing within the internal capsule. The fibers were located close to the lateral antero-superior border of the subthalamic nucleus (STN) and medial posterior border of the globus pallidus internus (GPi). This study found the characteristic subcortical networks of the FEF. These tracts should be noted to prevent complications of deep brain stimulation (DBS) of the STN or GPi.

Objective The aim of the present study was to compare localization of the language cortex using electrical cortical stimulation (ECS) and functional magnetic resonance imaging (fMRI) to establish the relevance of fMRI language mapping. Methods Language mapping with fMRI and functional ECS mapping were retrospectively compared in ten patients with refractory epilepsy who underwent fMRI language mapping and functional ECS mapping between June 2012 and April 2019. A shiritori task, a popular Japanese word chain game, was used for fMRI language mapping. Results BOLD signal activation was observed in the left inferior frontal gyrus (including the pars opecularis and the pars triangularis), and superior temporal gyrus, which is a language-related area, as well as in the left superior and middle frontal gyri, the intraparietal sulcus, and fusiform gyrus. These results were compared with ECS to elucidate the functional role of the activated areas during fMRI language tasks. These activated areas included language areas, negative motor areas, supplementary motor areas (SMAs), and non-functional areas. Conclusion The activated areas of fMRI language mapping include language-related areas, the negative motor area, and SMAs. These findings suggest the involvement of language and higher order motor networks in verbal expression.

The purpose of this study was to investigate whether and how vagus nerve stimulation (VNS) reduces the epileptogenic activity in the bilateral cerebral cortex in patients with intractable epilepsy. We analyzed the electrocorticograms (ECoGs) of five patients who underwent callosotomy due to intractable epilepsy even after VNS implantation. We recorded ECoGs and analyzed power spectrum in both VNS OFF and ON phases. We counted the number of spikes and electrodes with epileptic spikes, distinguishing unilaterally and bilaterally hemispherically spread spikes as synchronousness of the epileptic spikes in both VNS OFF and ON phases. There were 24.80 ± 35.55 and 7.20 ± 9.93 unilaterally spread spikes in the VNS OFF and ON phases, respectively (P = 0.157), and 35.8 ± 29.21 and 10.6 ± 13.50 bilaterally spread spikes in the VNS OFF and ON phases, respectively (P = 0.027). The number of electrodes with unilaterally and bilaterally spread spikes in the VNS OFF and ON phases was 3.84 ± 2.13 and 3.59 ± 1.82 (P = 0.415), and 8.20 ± 3.56 and 6.89 ± 2.89 (P = 0.026), respectively. The ECoG background power spectra recordings in the VNS OFF and ON phases were also analyzed. The spectral power tended to be greater in the high-frequency band at VNS ON phase than OFF phase. This study showed the reduction of epileptogenic spikes and spread areas of the spikes by VNS as immediate effects, electrophysiologically.

Diffuse astrocytic and oligodendroglial tumors are frequently associated with symptomatic epilepsy, and predictive seizure control is important for the improvement of patient quality of life. To elucidate the factors related to drug resistance of brain tumor-associated epilepsy from a pathological perspective. From January 2012 to October 2017, 36 patients diagnosed with diffuse astrocytic or oligodendroglial tumors were included. Assessment for seizure control was performed according to the Engel classification of seizures. Patient clinical, radiological, and pathological data were stratified based on the following 16 variables: age, sex, location of tumor, existence of the preoperative seizure, extent of resection, administration of temozolomide, radiation therapy, recurrence, Karnofsky performance scale, isocitrate dehydrogenase 1, 1p/19q co-deletion, Olig2, platelet-derived growth factor receptor alpha, p53, ATRX, and Ki67. These factors were compared between the well-controlled group and drug-resistant seizure group. Twenty-seven patients experienced seizures; of these, 14 cases were well-controlled, and 13 cases were drug-resistant. Neither clinical nor radiological characteristics were significantly different between these two groups, though p53 immunodetection levels were significantly higher, and the frequency of 1p/19q co-deletion was significantly lower in the group with drug-resistant seizures than in the well-controlled group. In the multivariate analysis, only one item was selected according to stepwise methods, and a significant difference was observed for p53 (OR, 21.600; 95% CI, 2.135-218.579; P = 0.009). Upregulation of p53 may be a molecular mechanism underlying drug resistant epilepsy associated with diffuse astrocytic and oligodendroglial tumors.

Numerous non-epileptic physiological electroencephalographic (EEG) patterns morphologically mimic epileptiform activity. However, misleading non-epileptic findings of electrocorticography (ECoG) have not yet been examined in detail. The aim of the present study was to identify non-epileptic epileptiform ECoG findings. We retrospectively reviewed the intracranial recordings of 21 patients with intractable focal epilepsy who became seizure-free after a presurgical evaluation with subdural electrodes following resective surgeries at Sapporo Medical University between January 2014 and December 2018. Morphological epileptiform findings outside epileptogenic areas were judged as non-epileptic and analyzed. Seventeen areas in nine patients exhibited non-epileptic epileptiform activities. These areas were identified in the lateral temporal cortices, basal temporal areas, rolandic areas, and frontal lobe. Morphological patterns were classified into three types: 1) spiky oscillations, 2) isolated spiky activity, and 3) isolated fast activity. The normal cortex may exhibit non-epileptic epileptiform activities. These activities need to be carefully differentiated from real epileptic abnormalities to prevent the mislocalization of epileptogenic areas.

Advances in the understanding of the pathogenesis of arteriosclerosis, abdominal aorta aneurysms and dissections, and carotid artery plaques have focused on chronic inflammation. In this study, we report that inflammatory changes of thrombi contribute to the enlargement and growth of giant intracranial thrombotic aneurysms. Surgical and postmortem samples were collected from 12 cases of large or giant intracranial thrombotic aneurysms diagnosed via pathological investigations. Degeneration of the aneurysmal wall and the infiltration of inflammatory cells in the thrombi were assessed. The number of blood cells and immunohistochemical stain-positive cells was enumerated, and the inflammation and neovascularization in the thrombi were assessed. In all cases, the appearance of inflammatory cells (CD68+ cells, CD206+ cells, lymphocytes, and neutrophils) was apparent in the thrombi. The number of CD34+ cells was moderately correlated with the number of CD68+ cells, and CD34+ cells significantly and strongly correlated with the number of CD206+ cells. Based on the number of neutrophils per CD68+ cells, we classified the cases into 2 groups: a macrophage inflammation-dominant group and a neutrophilic inflammation-dominant group. The neutrophilic inflammation-dominant group had significantly more cases with previous treatments and neurological symptoms due to mass effect than the macrophage inflammation-dominant group. Chronic inflammation due to macrophages in thrombi is a fundamental mechanism in the enlargement of an intracranial thrombotic aneurysm, and neutrophilic inflammation can accelerate this process. Microvascularization in thrombi is linked to inflammation and might promote thickening of the intima and repeated intimal microbleeds.

An electrical cortical stimulation provides important information for functional brain mapping. However, subjective responses (i.e. sensory, visual, and auditory symptoms) are purely detected by patients' descriptions, and may be affected by patients' awareness and intelligence levels. We experienced psychogenic responses in the electrical cortical stimulation of two patients with intractable epilepsy. A sham stimulation was useful for differentiating pseudo-responses from real responses in the electrical cortical stimulation. Inductive questions, long testing durations, and clear cues of stimulation onsets need to be avoided to prevent psychogenic pseudo-responses in the electrical cortical stimulation. Furthermore, a sham stimulation is applicable for detecting pseudo-responses the moment patients show atypical or inexplicable symptoms.

We report the first case of pseudoaneurysm associated with polytetrafluoroethylene fiber used in microvascular decompression (MVD). A 62-year-old female who had undergone MVD for hemifacial spasm 30 years ago presented with a 4-month history of progressive facial palsy. Computed tomography angiography revealed a large thrombosed aneurysm originating from the right posterior inferior cerebellar artery and having a mass effect upon the pons. The aneurysm was treated by trapping and bypass procedure. Intraoperatively, the pseudoaneurysm adhered to the dura mater, and the thrombus contained a large amount of polytetrafluoroethylene fiber. The cause and management of pseudoaneurysm after MVD is discussed.

Scalp tissue shrinkage and volume contraction is a major problem in cranioplasty, and sometimes a tissue expander must be set before cranioplasty. The procedure for placing scalp expanders is cumbersome. In this study, we present a method for flattening the curvature of synthetic materials to relieve scalp skin tension and discuss the feasibility and limitations of the method.A total of 25 cranioplasty patients were included in this study. The optimal degree of curvature flattening for each piece of bone substitute material was determined based on cosmetic considerations and the extent of encephalomalacia or atrophy due to primary disease. In this series, the correlation between the degree of curvature flattening and the size or location of the bone flap was considered, and the amount of scalp surface area that could be obtained through curvature flattening was estimated.The median degree of curvature flattening was 5.0 mm. The degree of curvature flattening showed moderate correlation with the rate of change in the area of synthetic material achieved through curvature flattening (p 0.001). The 21 cases of fronto-temporal craniectomy were divided into two groups according to the distance from the midline. There was a statistically significant difference between these two groups in degree of flattening curvature.In the present cranioplasty series using synthetic materials, curvature flattening was a non-invasive and convenient method for skin closure. This method can be beneficial especially in patients requiring a larger craniotomy including convexity regions.

Previous studies have reported the usefulness of intraoperative corticocortical evoked potentials (CCEPs) for preserving language function during brain surgery.This study aimed to assess the influence of depth of anesthesia on CCEP to establish its clinical utility.Twenty patients with brain tumors or epilepsy who underwent awake craniotomy were included in this study. Before resection, the electrode plates were placed on the frontal and temporoparietal cortices, and 1-Hz alternating electrical stimuli were delivered to the pars opercularis/pars triangularis in a bipolar fashion. Electrocorticograms from the temporoparietal cortices time-locked to stimuli were averaged to obtain CCEP responses from a state of deep anesthesia until the awake state. The correlation between CCEP waveforms and bispectral index (BIS) was evaluated.CCEP amplitude increased with the increase in the BIS level. CCEP latency decreased in 5 patients and increased in 15 patients under anesthesia compared with the awake state. CCEP amplitudes decreased by 11.3% to 75.2% (median 31.3%) under anesthesia with65 BIS level. These differences were statistically significant (P0.01, Wilcoxon signed-rank test). With respect to CCEP latencies, there was no significant difference between the awake and anesthetic states.CCEP amplitudes were correlated with depth of anesthesia, whereas CCEP latencies were not affected by anesthesia. The influence of anesthesia should be considered when applying this technique to intraoperative monitoring.

Adult orbital xanthogranuloma is rare and usually associated with systemic disease. There are several options available to treat this disorder. Periorbital lesions are treated with steroids, chemotherapy, radiotherapy, or local excision; however, there is still no consensus regarding optimal treatment. Here, we report a rare case of orbital xanthogranuloma that was not associated with systemic disease and was treated by transcranial surgery. The patient was a 52-year-old man who presented with a 2-year history of unilateral eye symptoms. A computed tomography scan revealed a well-defined mass in the right orbit. The mass was completely removed via a transcranial orbital approach. The histopathologic diagnosis was xanthogranuloma. No recurrence was observed during 15 months of postoperative follow-up. Complete surgical resection might be an effective treatment option for locally growing sporadic adult xanthogranulomatous disease of the orbit, and allows systemic steroids, chemotherapy, and irradiation to be avoided.

Moyamoya disease is a unique cerebrovascular disease that is characterized by progressive bilateral stenotic alteration at the terminal portion of the internal carotid arteries. These changes induce the formation of an abnormal vascular network composed of collateral pathways known as moyamoya vessels. In quasi-moyamoya disease, a similar stenotic vascular abnormality is associated with an underlying disease, which is sometimes an inflammatory disease. Recent advances in moyamoya disease research implicate genetic background and immunological mediators, and postulate an association with inflammatory disease as a cause of, or progressive factor in, quasi-moyamoya disease. Although this disease has well-defined clinical and radiological characteristics, the role of inflammation has not been rigorously explored. Herein, we focused on reviewing two main themes: (1) molecular biology of inflammation in moyamoya disease, and (2) clinical significance of inflammation in quasi-moyamoya disease. We have summarized the findings of the former theme according to the following topics: (1) inflammatory biomarkers, (2) genetic background of inflammatory response, (3) endothelial progenitor cells, and (4) noncoding ribonucleic acids. Under the latter theme, we summarized the findings according to the following topics: (1) influence of inflammatory disease, (2) vascular remodeling, and (3) mechanisms gleaned from clinical cases. This review includes articles published up to February 2019 and provides novel insights for the treatment of the moyamoya disease and quasi-moyamoya disease.

To describe the far-anterior interhemispheric transcallosal approach for the treatment of a central neurocytoma at the roof of the lateral ventricle. In comparison to the view obtained during the usual anterior transcallosal approach, the far-anterior approach allowed for a higher view of the lateral ventricle to be obtained without further injury or retraction of the corpus callous. Two patients with central neurocytoma in the lateral ventricle were treated with the far-anterior interhemispheric transcallosal approach. Gross-total resections were achieved in both the patients without any postoperative neurological impairments by only 2-3 cm incisions of the corpus callosum. With the anterior transcallosal approach, which was usually used for the intraventricular tumors, the surgical view was relatively downward into the lateral ventricle and suitable for the resection of the tumors located at the base of the lateral ventricle or even in the third ventricle through the foramen of Monro. However, it was relatively difficult to reach the roof of the lateral ventricle using this approach. In contrast, the surgical corridor of the far-anterior transcallosal approach reaches upward to the roof of the lateral ventricle. The far-anterior transcallosal approach provides an alternative to reach the lesions, especially those located in the upper region of the lateral ventricle near important structures, such as the pyramidal tracts.

ObjectivesTo evaluate the effects of nonadherence to antiseizure medications (ASMs) and clinical characteristics on seizure control, we employed a prospective cohort cross-sectional study using self-reports and medical records of patients with epilepsy (PWEs).MethodsEight hundred and fifty-five PWEs taking ASMs were enrolled from fourteen collaborative outpatient clinics from January 2018 to March 2019. Questions from the Morisky Medication Adherence Scale were used as adherence self-reports. If a PWE's questionnaire indicated that they had missed doses of their ASMs, outpatient physicians asked them directly about the details of their compliance, including the timing of intentionally or unintentionally missed doses. The association between lack of seizure control and utilization outcomes, such as missed doses, demographics, and clinical characteristics of the PWEs, were assessed by univariate and multivariate analyses.ResultsMultivariate analysis revealed that forgetting to take ASMs was associated with lack of seizure control and the existence of focal to bilateral tonic-clonic seizures. Dementia, younger age, use of three or more antiepileptic agents, and living in a one-person household were associated with the risk of forgetting to take ASMs.SignificanceFor PWEs with poor drug management or a high incidence of missed doses of ASMs, efforts to improve adherence could facilitate better seizure control and decrease focal to bilateral tonic-clonic propagation.

We report a case of moyamoya disease (MMD), which developed after non-herpetic acute limbic encephalitis (NHALE) associated with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody. The patient's mother had a history of MMD. No vascular lesions were identified at the time of the NHALE. Nine years later, the patient visited our hospital due to memory disturbances and repeated transient ischemic attacks affecting the right limb. Diffusion-weighted magnetic resonance imaging revealed scattered areas of signal hyperintensity, and the patient was ultimately diagnosed with MMD based on angiography. Revascularization surgery was performed on the left side, where cerebral blood flow was impaired on 123I-N-isopropyl-p-iodoamphetamine single photon emission computed tomography. Postoperatively, the patient was discharged with a normal neurological examination. NHALE associated with LGI1 antibodies is an autoimmune disease. Although autoimmune disease is the most frequent finding other than atherosclerosis in quasi-MMD, this is the first report of NHALE associated with anti-LGI1 antibodies mimicking quasi-MMD. Inflammation and angiogenesis may contribute to the development of MMD, in addition to genetic background.

Extracranial-to-intracranial (EC-IC) bypass surgery may be necessary in patients with moyamoya disease and other ischemic conditions. However, there is a potential risk of wound-related complications in some cases. In this study, we report our approach to the prevention of wound-related complications in EC-IC bypass. Technical considerations and pitfalls of surgery are also discussed. This study included 89 patients with ischemic-onset moyamoya disease and atherosclerotic disease who underwent 108 superficial temporal artery (STA)-to-middle cerebral artery bypass procedures. Our study emphasized 3 major features. First, 3-dimensional simulation imaging was used to confirm STA anatomy. Second, the STA was meticulously dissected on the epigaleal layer to protect the galeal layer. Third, scalp skin ischemia was taken into consideration at each step until skin closure. There was no neurologic morbidity or mortality in this series. There were 2 cases of major wound-related complications requiring plastic surgical intervention, and 4 cases of minor complications that were treated conservatively. In major complication cases, the scalp defect was treated with pedicle flap reconstruction. In EC-IC bypass surgery, interdisciplinary management involving neurosurgery, plastic surgery, and radiology should reduce wound-related complications and achieve safe surgery.

In neurosurgery, extracranial-to-intracranial (EC-IC) bypass surgery is necessary for patients who have undergone surgery in which the superficial temporal artery (STA) was already used for a different bypass procedure or was damaged. Here we report our experience with EC-IC bypass using the occipital artery (OA) in patients in whom the STA was unavailable, and discuss the technical considerations and pitfalls. Five patients with ischemic-onset moyamoya disease and atherosclerotic disease were included. Two patterns of skin incisions were planned according to the OA pathway and recipient artery location. In one of these methods, a skin incision is made above the OA, and a craniotomy is performed under this incision after OA dissection. In the other method, a skin incision is made above the OA to enable its dissection, and a craniotomy is performed via a separate skin incision. No major perioperative complications developed in any of the 5 cases, and bypass patency was confirmed in all patients. There was a significant difference between the preoperative and postoperative asymmetry ratios of the mean transit time values. Our findings suggest that OA-to-middle cerebral artery (MCA) bypass is a simple and effective technique in patients in whom the STA was already used or was damaged by previous intracranial revascularization or craniotomy. This procedure could be an alternative to STA-MCA bypass in patients without an available STA.

In specific cases of moyamoya disease (MMD), posterior cerebral artery (PCA) stenosis can develop after treatment of the anterior circulation and require additional revascularization. Here, we report two cases that underwent additional posterior indirect revascularization with multiple burr holes for PCA involvement after bilateral revascularization treatment of the anterior circulation. They presented with transient ischemic attack even after bilateral superficial temporal artery-middle cerebral artery bypass, and magnetic resonance angiography (MRA) showed that PCA stenosis had worsened. Indirect revascularization with multiple burr holes using Benz-marked skin incisions was performed. After surgery, the symptoms improved without perioperative complications, and cerebral angiography showed collateral circulation via the burr hole. Indirect revascularization for MMD is often combined with direct revascularization, and there are only a few reports on the use of multiple burr hole surgery alone. In addition, there are few reports of posterior circulation, despite the emphasis on the importance of PCA involvement in MMD. Indirect revascularization with multiple burr holes alone can be performed in multiple areas and applied to patients who cannot undergo direct revascularization using the occipital artery. The procedure is simple and less invasive than traditional direct revascularization procedures. Therefore, it can be effective, especially in pediatric cases of MMD with PCA involvement.

Background Dilatation of the microvascular diameter is recognized in moyamoya disease and referred to as microvascularization. The purpose of this study was to characterize the cortical microvascularization in moyamoya disease using imaging analysis, and to explore the developmental mechanism of the collateral network around the cortical surface. Methods A total of 20 hemispheric sides of 14 patients with moyamoya disease were included in this study. From the intraoperative images, cortical surface images were extracted, and binary images were subsequently created. Then the ratio of the microvessels of the brain surface (vascular fraction; VF) and the box-counting fractal dimension (Db) values were calculated. The VF and Db values in the moyamoya disease group were then compared with those in atherosclerotic disease and nonischemic disease groups, and assessed in terms of clinical and radiologic factors. Results VF was significantly higher in the moyamoya disease group compared with the atherosclerotic disease group, and Db was significantly higher in the moyamoya disease group compared with the atherosclerotic disease and nonischemic disease groups. In the moyamoya disease group, VF showed a moderate correlation with magnetic resonance angiography (MRA) score. Moreover, Db was significantly higher in the pediatric patients, in the presence of ischemic symptoms, and in the presence of ivy sign, and Db showed a moderate correlation with MRA score and cerebral blood flow in moyamoya disease. Conclusions In the patients with moyamoya disease, the cortical microvascularization exhibited increased Db and dilatation of the pial arteries. In moyamoya disease, cortical microvascularization is associated with clinical and radiologic factors. This microvascularization might be a compensatory mechanism in the ischemic condition in moyamoya disease.

Reflex seizures are epileptic events triggered by specific external stimuli, or less commonly, internal mental stimuli. Understanding the characteristics of reflex seizures is important to elucidate the mechanisms underlying network abnormalities in epileptic conditions. This report details a patient with medically intractable reflex seizures provoked by sensory stimuli to the patient's right foot. Single-photon emission computed tomography (SPECT) during the seizure induced by sensory stimulation showed hyperperfusion in broad sensory-motor networks (dorsal column-medial lemniscus pathway, left thalamus, bilateral postcentral gyri and posterior parietal cortices, left supplementary motor area (SMA), and left paracentral lobule) and left caudateputamen. The irritative zones and ictal onset zone were localized to the left medial frontoparietal (SMA, anterior and middle cingulate gyrus, and paracentral lobule) and lateral posterior parietal cortices, as evidenced by amelioration of reflex seizures following intracranial electroencephalography and surgical resection of these areas. The neuroradiological and electrophysiological findings in our case study illustrate that the mechanism of reflex seizures may be associated with hyperexcitability of the broad sensory-motor networks, including the basal ganglia. Disconnection of these networks is necessary to treat reflex seizures.

OBJECTIVEMedullary streaks detected on fluid-attenuated inversion recovery (FLAIR) imaging have been considered to be reflected ischemic regions in pediatric moyamoya disease. The purpose of this study was to evaluate these medullary streaks both clinically and radiologically and to discuss associated pathophysiological concerns.METHODSThe authors retrospectively reviewed data from 14 consecutive pediatric patients with moyamoya disease treated between April 2009 and June 2016. Clinical and radiological features and postoperative imaging changes were analyzed. In 4 patients, hyperintense medullary streaks on FLAIR imaging (HMSF) at the level of the centrum semiovale were detected.RESULTSThe HMSF were coincident with hyperintense medullary streaks on a T2-weighted image, though they were not completely coincident with the vasculature on either a T2*-weighted image or contrast-enhanced CT. Analysis revealed significantly higher values in terms of MR angiography scores, number of flow voids of the basal ganglia, and the presence of the medullary artery in the group with HMSF than in those without. In contrast, the presence of white matter damage was significantly less frequent in the HMSF group. All HMSF disappeared after surgery, and the mean apparent diffusion coefficient at the same level was significantly reduced postoperatively.CONCLUSIONSAlthough HMSF should be associated with collateral circulation in moyamoya disease, other factors may be involved, including stagnated cerebrospinal fluid or vasogenic edema that is relevant to the impaired state of the white matter. Findings in this study provide insight into the pathophysiological basis of the perivascular space in moyamoya disease.

Introduction This study investigated the networks of Negative motor areas (NMAs) using electric cortical stimulation and diffusion tensor imaging (DTI). Methods Twelve patients with intractable focal epilepsy, in which NMAs were identified by electrical cortical stimulation, were enrolled in this study. Electric stimulation at 50 Hz was applied to the electrodes during motor tasks to identify the NMAs. DTI was used to identify the subcortical fibers originating from the NMAs found by electrical stimulation. Results NMAs were found in lateral frontal areas (premotor area (PM) and precentral gyrus) in all 12 patients, in pre-supplementary motor areas (pre-SMAs) in four patients, and in posterior parietal cortices (PPCs) in four. DTI detected fibers connecting to the ipsilateral PMs, PPCs and temporal regions via U-fibers, superior longitudinal fasciculus (SLF), and arcuate fasciculus (AF) from the lateral frontal NMAs. Pre-SMA-NMAs had connections with ipsilateral PMs and contralateral pre-SMAs via the frontal aslant tract and transcallosal commissural fibers, and PPC-NMAs with ipsilateral PMs via SLF and AF. Conclusion This study found the characteristic cortical network of each NMA, and especially revealed new insight of pre-SMA-NMA and PPC NMA. These NMAs might be associated with different mechanism of negative motor response.

Epileptic nystagmus is a quick, repetitive, jerky movement of the eyeball caused by seizure activity, which is unaccompanied by other ictal phenomena. We report a case of moyamoya disease with epileptic nystagmus. A 23-year-old woman presented with a headache and transient hemiparesis on her left side. Magnetic resonance imaging showed no ischemic or hemorrhagic stroke lesions. Digital subtraction angiography confirmed stenosis of the terminal portion of the right internal carotid artery and the formation of moyamoya vessels on the right side. 123I-N-isopropyl-iodoamphetamine (123I-IMP) single photon emission computed tomography (SPECT) showed decreased uptake in the right basal ganglia, frontal, and parietal regions. After electroencephalography (EEG) and a hyperventilation test were performed, nystagmus appeared and was accompanied with a declining level of consciousness. Ictal EEG during an attack showed no epileptiform discharge. Moreover, the patient sometimes experienced simultaneous upper limb-shaking and gelastic attacks. After superficial temporal artery to middle cerebral artery bypass surgery was performed on the right side, symptom frequency and duration gradually decreased. Decreased 123I-IMP SPECT blood flow in the right frontal region is considered a mechanism that causes the onset of epileptic nystagmus. It is presumed that the attack was caused by an ischemic abnormality in the saccade region of the frontal eye field. Moreover, revascularization can effectively treat the symptoms of moyamoya disease.

Background Vagus nerve stimulation is a palliative treatment for patients with refractory epilepsy; however, the misplacement of electrodes may cause complications and thus needs to be avoided. Methods We herein report an intraoperative monitoring technique to prevent the misplacement of electrodes. Endotracheal tube electrodes were inserted to record electromyographic activity from the vocal cords and identify the vagus nerve. Electromyography electrodes were placed on the sternomastoid muscle, sternohyoid muscle, geniohyoid muscle, and trapezius muscle to record muscle activities innervated by the ansa cervicalis. The vagus nerve and ansa cervicalis were electrically stimulated during surgery, and electromyography of the vocal cords and muscles innervated by the ansa cervicalis was recorded. The threshold of vagus nerve activation ranged between 0.05 and 0.75 mA. Results The vagus nerve was successfully identified and differentiated from the nerve root of the ansa cervicalis using this technique. Conclusions Intraoperative monitoring of the vagus nerve and ansa cervicalis is useful for safe and effective vagus nerve stimulation.

Background Although superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis is a beneficial treatment for hemorrhagic moyamoya disease, indeterminate mechanisms can cause rebleeding even after successful bypass surgery. We describe a case with a prominent collateral from the lenticulostriate artery (LSA) causing multiple recurrent hemorrhages after successful STA-MCA anastomosis. Case Description A 49-year-old Japanese woman with moyamoya disease was referred to our institution after suffering intracranial hemorrhage on 2 occasions. Angiography revealed multiple anastomotic vessels branching from a prominent LSA to connect medullary arteries in the periventricular area corresponding to the location of the hemorrhage. She underwent STA-MCA anastomoses and the bypass widely perfused the MCA territory; however, intracranial hemorrhage recurred in the same hemisphere. Angiography revealed sustained dilatation of the medial anastomotic branches from the LSA with de novo pseudoaneurysm, whereas the lateral branches showed shrinkage. Subsequent surgical treatment included direct bypass targeting the medial hemispheric surface in which the medial branches were distributed; marked shrinkage of the branches and disappearance of the aneurysm were obtained without infarct. Conclusions Lenticulostriate anastomotic branches distributed medially to the anterior cerebral artery territory should be given more attention as an intractable cause of hemorrhage after successful STA-MCA anastomosis. Although challenging, direct bypass targeting the area reached by collateral vessels could be considered an alternative treatment strategy for this pathologic condition.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. It is characterized by multiple xanthogranulomatous masses throughout the body, predominantly in the tibia. One of the characteristic radiological findings of the lesions associated with ECD is a "coated artery," which is often observed in the aorta. Although approximately one-fourth of ECD cases involve the central nervous system (CNS), an intracranial-coated artery has only been reported in four cases. We report a case of ECD that involves the CNS and has the unique appearance of a coated vertebral artery (VA). These tumors entirely encase the bilateral VAs without stenosis and are attached to the dura. Cranial magnetic resonance imaging also showed multiple extra-axial tumors in the cavernous sinus, the frontal convexity, and the orbital cavity. Further investigation revealed additional extracranial lesions around the cervical carotid artery, at the bilateral tibia, and at the elbow joint. A biopsy of the cervical and tibial lesions confirmed ECD. Steroid therapy resulted in a month-long improvement of preoperative symptoms. However, the patient's condition gradually progressed and he died of pneumonia 1 year after ECD diagnosis. The encasement of the intracranial artery by the tumor without stenosis and the dural attachment suggest ECD, which requires whole body investigation.

Giant thrombosed middle cerebral artery (MCA) aneurysms are difficult to treat and sometimes require complex revascularization using allografts. We describe a technical method using revascularization with a natural Y-shaped graft that provides a normal variation for a complex MCA aneurysm. A 65-year-old man with a giant thrombosed MCA aneurysm presented with right hemiparesis and aphasia. The patient had a history of clipping surgery for the ipsilateral side of the MCA aneurysm 25 years before, and a de novo aneurysm developed over the previous 18 years. For the giant thrombosed aneurysm, trapping and revascularization were performed. A natural radial artery Y-graft was used as the graft and anastomosed to both M2 trunks. The symptoms improved after surgery, and the patient was discharged 3 weeks later. This is the first report of a double-barrel bypass using a natural Y-graft. This method attained a normal variation, and the flow of the Y-graft was physiological. For the radical cure of giant thrombosed MCA aneurysms, multiple revascularizations might be required. With this natural Y-graft, complex transpositions could be avoided.

Extracranial-to-intracranial (EC-IC) bypass surgery is an effective treatment for patients with moyamoya disease and other conditions. Some patients with moyamoya disease have a risk of acute thrombogenesis at the anastomotic site just after bypass surgery. The purpose of this study was to study risk factors of acute thrombogenesis and determine effective countermeasures. This study included 48 patients (66 EC-IC bypass procedures) with moyamoya disease and 52 controls (54 procedures) without moyamoya disease. The development of acute thrombogenesis was compared between the moyamoya disease and control groups. In the moyamoya disease group, clinical and radiological characteristics were assessed with respect to acute thrombogenesis. In the patients with acute thrombogenesis, causes of technical problems were retrospectively examined. The incidence of acute thrombogenesis was significantly higher in the moyamoya disease group than those in the control group. In the moyamoya disease group, acute thrombogenesis was observed in seven patients. In the moyamoya disease group, the magnetic resonance angiography (MRA) scores were significantly higher in patients with acute thrombogenesis than those in the patients without acute thrombogenesis. In the multivariate analysis, the predictive factor of acute thrombogenesis in moyamoya disease was a high MRA score (odds ratio, 2.336; p = 0.009). During EC-IC bypass surgery for moyamoya disease, acute thrombogenesis should be considered to obtain a high patency rate, particularly in patients with high MRA scores. Acute thrombogenesis will not influence morbidity if proper countermeasures are followed; therefore, the prediction and recognition of white thrombus are important for a successful bypass surgery.

Enlarged perivascular spaces (EPVS) are often observed in small vessel disease on T2-weighted images. However, their role in ischemic conditions caused by cerebral large vessel disease remains unclear. We evaluated EPVS in patients with hemodynamic compromise associated with atherosclerotic large vessel disease and aimed to identify the pathophysiology of EPVS.We examined 28 adults with atherosclerotic large vessel disease. EPVS numbers in the basal ganglia and the centrum semiovale were assessed. For each affected hemisphere, the total numbers of EPVS were compared with those on the unaffected side. EPVS in the impaired hemodynamics group were compared with those in the unimpaired hemodynamics group. Moreover, EPVS were compared in the presence/absence of large stroke.The number of EPVS was significantly increased on the affected side in the centrum semiovale (p = 0.023), particularly in the impaired hemodynamics group (p = 0.006). Moreover, in the small stroke subgroup of the impaired hemodynamics group, the number of EPVS was significantly increased on the affected side (p = 0.002), although this number was insignificant in the large ischemic stroke subgroup.The number of EPVS was increased in patients with atherosclerotic large vessel disease with hemodynamic compromise and decreased in the presence of a large stroke. EPVS might act as fluid absorbers in a hemodynamically compromised state until the occurrence of an ischemic stroke.

Background Microvascular anastomosis is an essential procedure in neurosurgery, but the opportunity to perform the surgery has gradually decreased for neurosurgeons. Therefore, training is necessary for obtaining and maintaining the skills required for the procedure. We describe the detailed anatomy of cervical and femoral regions in rats and discuss the advantages for practicing microvascular anastomosis. Methods Cervical regions of Sprague-Dawley rats were dissected under intraperitoneal anesthesia. The step-by-step anatomic description was documented using a high-resolution charge-coupled device image sensor and recording systems. Using this model, temporal occlusion time and patency were measured, and these measures were compared between the trainee and trainer groups. The number of times the training needs to be completed to attain competency in the bypass procedure was estimated. Results After exposing the carotid triangle, a half-ring was created by end-to-side anastomosis. Anastomosis was performed at the common carotid artery using the contralateral side of the carotid artery as a graft. The cutoff value for the temporal occlusion time was 79.3 minutes in the receiver operating characteristic curve based on a target temporal occlusion time for beginners determined during the training. Conclusions Using a living animal model, a trainee has the opportunity to learn not only anastomotic techniques but also hemostatic control as well as overcoming mental strain during surgery. Living animal models are important in training because the fidelity of a living animal model is superior to nonliving models. Applying training using a half-ring model contributes to safe and efficient surgery.

Objective The present study aimed to investigate the threshold and distribution of afterdischarges (ADs) with cortical electrical stimulation for functional brain mapping. Method We retrospectively analyzed data from 11 patients with medically intractable epilepsy who underwent 50-Hz cortical electrical stimulation for functional mapping followed by resection. These patients became seizure free for more than six months. The threshold and distribution of ADs induced by the stimulation were evaluated. Results The median threshold was 6 mA (range: 2–15 mA) for the frontal lobe, 8 mA (3–15 mA) for the temporal lobe, 6 mA (2–15 mA) for the parietal lobe, and 6 mA (4–12 mA) for the occipital lobe. No significant interlobar differences were observed in AD thresholds. No significant differences were noted between within and outside epileptogenic zones. The distribution of ADs, remote spread was observed in all patients, reflecting fronto-parieto-temporal connections, as well as contiguous spread. The stimulation of premotor areas, the inferior parietal lobule, supplementary motor area, and basal temporal areas appeared to induce ADs in remote cortices. Conclusion While no locational differences were observed in AD thresholds, each brain region showed a characteristic pattern for AD spread. Remote AD spread needs to be considered for safe functional mapping.

Recent advances in computed tomography angiography (CTA) enable repeated imaging follow up for post-clipping surgery. The purpose of this study was to clarify the critical volume and configuration of the aneurysmal clip in the postoperative evaluation using volume rendering (VR) imaging, and present four-dimensional (4D)-CTA for these larger metal artifacts. A total of 44 patients with cerebral aneurysm, treated using clipping surgery, were included in this study. The metal artifact volume was assessed using CTA and the association between the type of clips and its metal artifact volume was analyzed. A VR image and a 4D-CTA were then produced, and the diagnostic accuracy of arteries around the clip or residual aneurysm on these images was evaluated. In the receiver operating characteristic (ROC) curve analysis, the cutoff value for metal artifacts was 2.32 mm3 as determined through a VR image. Patients were divided into two groups. Group 1 included patients with a simple and small clip, and group 2 included patients with multiple, large or fenestrated clips. The metal artifact volume was significantly larger in group 2, and the group incorporated the cutoff value. Post-clipping status on the VR image was significantly superior in group 1 compared with group 2. In group 2, the imaging quality of post-clipping status on 4D-CTA was superior in 92.9% of patients. The metal artifact volume was dependent on the number, size, or configuration of the clip used. In group 2, evaluation using a 4D-CTA eliminated the effect of the metal artifacts.

Background Enlarged perivascular spaces (EPVS) are often observed with magnetic resonance imaging in patients with small vessel disease. However, the risk factors, radiological features, and clinical relevance of EPVS in patients with moyamoya disease are poorly understood. The purpose of this study was to evaluate EPVS, the risk factors of many EPVS, and the pathophysiology of EPVS in adult patients with moyamoya disease. Methods One hundred cerebral hemispheres of 50 adult patients with moyamoya disease were examined. The control group consisted of 50 age/sex-matched patients without ischemic disease. The numbers of EPVS at the level of the centrum semiovale per hemisphere were compared between the moyamoya disease and control groups. In each hemisphere, the total numbers of EPVS were categorized into five grades (0–4), and the clinical and radiological characteristics of the predictive factors in patients in the high EPVS grade group (EPVS grade = 4) were assessed. Results The EPVS counts and grades were significantly higher in the moyamoya disease group. Analyses of the background characteristics of the patients with moyamoya disease revealed that significantly higher prevalence of high EPVS grades were associated with the female sex, hypertension, high magnetic resonance angiography scores, high numbers of flow voids in the basal ganglia, high brain atrophy scores, ivy signs, and white matter lesions. A logistic multivariate analysis of the patients with high EPVS grades revealed significant associations with the female sex, hypertension, and flow voids in the basal ganglia. Conclusions Increased EPVS were confirmed in adult patients with moyamoya disease, and the associated clinical and radiological factors were identified. The presence of hypertension, the female sex, and flow voids in the basal ganglia were important for predicting high EPVS grades in patients with moyamoya disease. Reductions in arterial pulsations with steno-occlusive changes can inhibit the flow of interstitial fluid, which can increase the number of EPVS in patients with moyamoya disease. Other clinical factors, such as the female sex and hypertension, may promote secondary brain damage in patients with moyamoya disease. Further evaluations of EPVS in patients with moyamoya disease are needed to better understand their pathophysiological importance.

A 7-year-old girl presented with paroxysms of transient laughter accompanied by right limb weakness, followed by crying. She was diagnosed with moyamoya disease (figure 1). Interictal 123I-IMP-SPECT showed decreased cerebral blood flow in multiple areas including the frontal lobe and cingulate gyrus (figure 1). All the paroxysms of laughter occurred without unconsciousness and appeared only after hyperventilation during crying (video). EEG revealed no epileptiform discharge in the ictal state (figure 2). After bilateral revascularization, the paroxysms did not reappear. These attacks had behavioral features consistent with ischemia or epilepsy, the former being more likely.1,2

Computed tomography angiography (CTA) is often used to assess the vascular status in moyamoya disease. The purpose of the study is to identify the characteristics of cortical arteries (M4) of moyamoya disease on CTA; the clinical significance of which is also discussed. A total of 38 hemispheric sides of 27 patients with moyamoya disease were included in this study. The number of M4 was visualized on CTA using cortical surface imaging and compared between the moyamoya disease group and the non-moyamoya disease group or the control group. Then, the clinical and radiological factors associated with the number of M4, the distribution of M4, and collateral circulation were examined. The number of M4 was lower in the moyamoya disease group than in the non-moyamoya disease group and in the control group (p

Cases of intracranial germinoma with granulomatous reaction are rare, so a pathological diagnosis of this disease is difficult. In this report, we describe the case of a 13-year-old boy with a bilateral thalamic germinoma which initially mimicked tumefactive demyelinating disease with inflammation, based on the clinical symptoms, imaging results and histology of a biopsy specimen obtained endoscopically. Upon examination of the cerebrospinal fluid, oligoclonal bands were detected. Although his symptoms and radiological findings improved following steroid pulse treatment, they worsened dramatically almost 1 year after the first surgery and even after an additional steroid pulse treatment. Prompted by the clinical course, a second biopsy was performed, and a pathological examination of the specimen showed a two-cell pattern. The diagnosis was changed to intracranial germinoma. After chemotherapeutic treatment with etoposide and cisplatin, the patient's symptoms and radiological findings dramatically improved. We conclude that it is very challenging to distinguish germinomas with a granulomatous reaction due to other inflammatory diseases, especially when only small specimens can be obtained. Stereotactic or endoscopic biopsies should be performed using samples from several different points even if the lesions are associated with eloquent brain regions.

Breast milk has been found to contain high levels of biopterin compounds, whose physiological roles have attracted attention through newly discovered functions, particularly. as a cofactor for biosynthesis of nitric oxide. the biopterin level in serum and the volume of urinary excreted biopterin in neonatals. 1) The concentration of total biopterin in breast milk increased daily to a maximum value from the 7- 11 th day postpartum. then decreased to as low as one third of the peak value. 2) The daily ingestion of biopterin also increased. After a peak during the 7-9 th days. it then reduced to a constant level after 1 month. 3) The serum biopterin level on the 4 th day after birth showed a relatively constant value and had no correlation with the ingested amount of biopterin on the 3rd day. 4) No correlation existed between the level of urinary biopterin excreted and the ingested amount of breast milk. We concluded that orally ingested, concentrated biopterin may have no influence no the serum level of biopterin and urinary excreted biopterin .