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The endovascular treatment of large, wide-necked basilar apex aneurysms (BAAs) remains challenging. Although horizontal stent deployment across both P1 segments of the posterior cerebral arteries (PCAs) would be an optimal strategy in coil embolization of wide-necked BAAs, this is only feasible in cases with anatomically favorable access. In rare circumstances, large-diameter conduits of extracranial-intracranial (EC-IC) bypass can also provide a good access route for endovascular treatment of complex intracranial aneurysms. We describe the technique of accessing the PCA via EC-IC bypass grafts and deploying a stent horizontally across the neck of BAA and its coil embolization. We provide a detailed technical review and describe some pitfalls of the procedure. Two patients underwent EC-IC bypass surgery prior to the treatment of a large, wide-necked BAA. The radial artery and saphenous vein were used as grafts, respectively. To facilitate coil embolization for a large BAA, a PCA-to-PCA horizontal stent was deployed via the bypass graft. Trans-cell and jailing techniques were used, respectively. Both aneurysms were completely occluded, and the patients were discharged without any neurological deficit. Horizontal stent deployment via EC-IC bypass grafts can be performed safely, providing proper closure of the aneurysmal neck and apposition to both PCAs, facilitating complete coil embolization.

A high flow arteriovenous shunts in newborns may require urgent endovascular treatment right immediately after delivery if high output cardiac failure is resistant to medical treatment. The umbilical approach is often the first choice of the access route for endovascular treatment in the newborn. It is, however, not infrequent that the patient has an extensive lesion, which necessitates a second session of treatment because of the limitation of the usable amount of the contrast material in one session. In such a case, re-puncturing the femoral artery is difficult and carries the risk of leg ischemia. On the other hand, leaving the umbilical sheath for the second procedure carries risks of infection, thrombosis, and vessel injury. Herein we introduce our umbilical vessel catheter (UVC) retro-exchange technique (U-RET) in which we replace the umbilical sheath to a 3.5Fr UVC at the end of the first endovascular procedure to preserve the umbilical artery access and prepare for the repeated use. We believe that this method minimizes the risks of infection and vessel injury.

OBJECTIVE Neurosurgical ablation is an effective treatment for medically refractory motor symptoms of Parkinson’s disease (PD). A limited number of studies have reported the effect of ablation of the pallidothalamic tract for PD. In this study, the authors evaluated the safety and efficacy of unilateral pallidothalamic tractotomy for akinetic-rigid (AR)–PD. METHODS Fourteen AR-PD patients, who were enrolled in this prospective open-label study, underwent unilateral pallidothalamic tractotomy. The Movement Disorder Society–Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) Part III and Part IV (dyskinesia and dystonia) scores and levodopa equivalent daily dose (LEDD) were evaluated at baseline and at 3 and 12 months postoperatively. RESULTS Of the 14 patients enrolled in the study, 4 were lost to follow-up and 10 were analyzed. The total MDS-UPDRS Part III score significantly improved from 45 ± 4.6 at baseline to 32.9 ± 4.8 at 12 months postoperatively (p = 0.005). Contralateral side rigidity and bradykinesia significantly improved from 4.4 ± 0.5 and 10.4 ± 1.5 at baseline to 1.7 ± 0.4 (p = 0.005) and 5.2 ± 1.4 (p = 0.011) at 12 months, respectively. While posture significantly improved with a 20% reduction in scores (p = 0.038), no significant improvement was found in gait (p = 0.066). Dyskinesia and dystonia were improved with a 79.2% (p = 0.0012) and 91.7% (p = 0.041) reduction in scores, respectively. No significant change was found in the LEDD. Hypophonia was noted in 2 patients, eyelid apraxia was noted in 1 patient, and a reduced response to levodopa, which resulted in an increase in the daily dose of levodopa, was noted in 3 patients. No serious permanent neurological deficits were observed. CONCLUSIONS Unilateral pallidothalamic tractotomy improved contralateral side rigidity and bradykinesia, dyskinesia, and dystonia in patients with AR-PD. Clinical trial registration no.: UMIN000031138 (umin.ac.jp)

Breath-holding spells (BHS) are commonly observed in children as a result of an autonomic nervous system disorder triggered by crying, emotional distress, or pain. There are several types of BHS and cyanotic type is one of them. We encountered a case of 3-year-old girl who presented with a delayed adaptation period and BHS 2 weeks after an endoscopic third ventriculostomy (ETV). She experienced severe headache due to increased intracranial pressure (ICP) during the delayed adaptation period, which may have contributed to the onset of BHS. Management of BHS warrants treatment of the symptoms and removal of the causative factors; in our case, intensive pain control and resolution of the increased ICP after the adaptation period were effective. While BHS are usually described as a benign condition that improves spontaneously, we highlighted the importance of recognizing and monitoring atypical symptoms such as BHS in pediatric cases.

Background One of the greatest concerns associated with radiofrequency ablation is intracerebral hemorrhage (ICH). However, the majority of previous studies have mainly evaluated Parkinson disease patients with ablation of the globus pallidus internus (GPi). Objective To investigate the hemorrhagic risk associated with radiofrequency ablation using ventro-oral (Vo) nucleus, ventral intermediate (Vim) nucleus, GPi, and pallidothalamic tract. Methods Radiofrequency ablations for movement disorders from 2012 to 2019 at our institution were retrospectively analyzed. Multivariate analyses were performed to evaluate associations between potential risk factors and ICH. Results A total of 558 patients underwent 721 stereotactic radiofrequency ablations for movement disorders. Among 558 patients, 356 had dystonia, 111 had essential tremor, and 51 had Parkinson disease. Among 721 procedures, the stereotactic targets used in this study were as follows: Vo: 230; Vim: 199; GPi: 172; pallidothalamic tract: 102; Vim/Vo: 18. ICH occurred in 37 patients (5.1%, 33 with dystonia and 4 with essential tremor). Symptomatic ICH developed in 3 Vo nuclei (1.3%), 3 Vim nuclei (1.5%), and 2 GPi (1.2%). Hypertension (odds ratio = 2.69, P = .0013), higher number of lesions (odds ratio = 1.23, P = .0221), and younger age (odds ratio = 1.04, P = .0055) were significant risk factors for ICH associated with radiofrequency ablation. Conclusion The present study revealed that younger age, higher number of lesions, and history of hypertension were independent risk factors for ICH associated with stereotactic radiofrequency ablation.

OBJECTIVE Identification of the motor area during awake craniotomy is crucial for preservation of motor function when resecting gliomas located within or close to the motor area or the pyramidal tract. Nevertheless, sometimes the surgeon cannot identify the motor area during awake craniotomy. However, the factors that influence failure to identify the motor area have not been elucidated. The aim of this study was to assess whether tumor localization was correlated with a negative cortical response in motor mapping during awake craniotomy in patients with gliomas located within or close to the motor area or pyramidal tract. METHODS Between April 2000 and May 2019 at Tokyo Women’s Medical University, awake craniotomy was performed to preserve motor function in 137 patients with supratentorial glioma. Ninety-one of these patients underwent intraoperative cortical motor mapping for a primary glioma located within or close to the motor area or pyramidal tract and were enrolled in the study. MRI was used to evaluate whether or not the tumors were localized to or involved the precentral gyrus. The authors performed motor functional mapping with electrical stimulation during awake craniotomy and evaluated the correlation between identification of the motor area and various clinical characteristics, including localization to the precentral gyrus. RESULTS Thirty-four of the 91 patients had tumors that were localized to the precentral gyrus. The mean extent of resection was 89.4%. Univariate analyses revealed that identification of the motor area correlated significantly with age and localization to the precentral gyrus. Multivariate analyses showed that older age (≥ 45 years), larger tumor volume (> 35.5 cm3), and localization to the precentral gyrus were significantly correlated with failure to identify the motor area (p = 0.0021, 0.0484, and 0.0015, respectively). Localization to the precentral gyrus showed the highest odds ratio (14.135) of all regressors. CONCLUSIONS Identification of the motor area can be difficult when a supratentorial glioma is localized to the precentral gyrus. The authors’ findings are important when performing awake craniotomy for glioma located within or close to the motor area or the pyramidal tract. A combination of transcortical motor evoked potential monitoring and awake craniotomy including subcortical motor mapping may be needed for removal of gliomas showing negative responses in the motor area to preserve the motor-related subcortical fibers.

Objective Ablation of the globus pallidus internus (pallidotomy) is an effective surgical intervention for dystonia. However, the current literature on the efficacy and safety of pallidotomy for dystonia is derived only from single‐case reports and small cohort studies. Methods We retrospectively analyzed patients with primary dystonia who underwent pallidotomy at our institution between 2014 and 2019. Neurological conditions were evaluated using the Burke‐Fahn‐Marsden Dystonia Rating Scale (BFMDRS, range: 0–120). We evaluated the total BFMDRS score and each subitem score (nine body regions) in the patients who underwent unilateral and bilateral pallidotomy before surgery and at last available follow‐up. Moreover, postoperative complications were analyzed. Results We found that 69 and 20 patients underwent unilateral and bilateral pallidotomy respectively. The mean age at dystonia onset was 40.4 ± 15.2 years. The mean clinical follow‐up period was 17.2 ± 11.6 months. Unilateral pallidotomy significantly improved the total BFMDRS score from 11.2 ± 14.7 preoperatively to 5.4 ± 7.6 at last available follow‐up (51.8% improvement, p

Forel‐H‐tomy for intractable epilepsy was introduced by Dennosuke Jinnai in the 1960s. Recently, Forel‐H‐tomy was renamed to “pallidothalamic tractotomy” and revived for the treatment of Parkinson's disease and dystonia. Two of our patients with movement disorders and comorbid epilepsy experienced significant seizure reduction after pallidothalamic tractotomy, demonstrating the efficacy of this method. The first was a 29‐year‐old woman who had temporal lobe epilepsy with focal impaired awareness seizure once every three months and an aura 10‐20 times daily, even with four antiseizure medicines. For the treatment of hand dyskinesia, she underwent left pallidothalamic tractotomy and her right‐hand dyskinesia significantly improved. Fourteen months later, she had experienced no focal impaired awareness seizure and the aura decreased to one to three times per month. The second case was that of a 15‐year‐old boy diagnosed with progressive myoclonic epilepsy, who developed generalized tonic‐clonic seizure, which manifested once every month, despite treatment with five antiseizure medicines. After surgery, myoclonic movements in his right hand slightly improved. A one‐year follow‐up revealed that he had not experienced a generalized tonic‐clonic seizure. The lesion locations in the two cases were close to the vicinity of Jinnai's Forel‐H‐tomy. Forel's field H deserves reconsideration as a treatment target for intractable epilepsy.

The disadvantages of biopsy for lesions in the basal ganglia and thalamus include a risk of various complications, difficulty in selecting the target tissue in some cases due to indistinct neuroimaging findings and limited availability of sample tissue. Placental alkaline phosphatase (PLAP) plays a decisive role in the diagnosis and management of intracranial germ cell tumors (IGCTs) in the basal ganglia and thalamus. The present study aimed to demonstrate the ability, specificity, and optimal use of PLAP values obtained from cerebrospinal fluid (CSF). Twenty patients with lesions in the basal ganglia and thalamus were enrolled in this study: 11 had IGCTs and 9 had non-IGCTs. The values of PLAP and other established tumor markers in the CSF were measured in all patients before treatment. The mean follow-up period was 76.0 months (range, 3–168) for all lesions. PLAP was elevated in all 11 patients with IGCTs in the basal ganglia or thalamus, whereas none of the patients with non-IGCT exhibited elevated PLAP. Thus, the sensitivity and specificity of PLAP were both 100%. Our data demonstrated that the PLAP value can specifically identify the germinomatous component even in cases of IGCTs in the basal ganglia or thalamus with high sensitivity and specificity. PLAP is undoubtedly beneficial for the safe and timely detection of the germinomatous component of IGCTs in the basal ganglia and thalamus, because reliance on PLAP measurement enables us to avoid invasive surgical procedures and facilitates the prompt initiation of chemoradiation therapy.

Glioma patients were frequently associated with mucosal thickening of the maxillary sinus (MTMS), which reflects mucosal inflammation. We suspected that MTMS is associated with impaired mucosal immune response and correlated with dysfunction in the anti-tumor immune response in diffuse glioma patients. Therefore, the aim of this study was to determine whether the occurrence of diffuse glioma is correlated with MTMS compared to meningioma and control groups. Furthermore, we investigated whether MTMS is associated with overall survival (OS) in glioblastoma (GBM) patients. This study included 343 patients with newly diagnosed diffuse gliomas and 218 patients with meningioma treated at our institution between 2015 and 2018. As control, 201 patients with headache who did not have an intracranial organic lesion were included. Using three-axis MR images, we evaluated the incidence of MTMS in all patients. Additionally, we investigated the relationship between MTMS and OS. The incidence of MTMS in patients with diffuse glioma was significantly higher than that in the meningioma (p < .0001) and control groups (p < .0001). In 128 patients with GBM, MTMS status correlated significantly with OS (p = .0064). We revealed that the incidence of MTMS is significantly associated with patients with diffuse glioma. This suggests that MTMS is indirectly involved in the occurrence of diffuse gliomas. Furthermore, the presence of MTMS correlated significantly with shorter OS in GBM patients, indicating that MTMS is involved in suppression of anti-tumor immune response. Preoperative recognition of MTMS might be useful for improving the clinical management of GBM patients.

Extracranial carotid artery aneurysms (ECAAs) are rare, with the etiology mainly classified as degeneration or dissection. Pseudoaneurysms in the region are even rarer and are seen following trauma, iatrogenic injury, or infection. We report a case of extracranial carotid artery pseudoaneurysm (pseudo-ECAA) with a rare clinical course and pathological features. A 58-year-old man presented with swelling and purpura on the left side of his neck after sneezing. Radiological examinations suggested a ruptured left common carotid artery aneurysm. The operative findings were consistent with a pseudoaneurysm. Pathological examination revealed disarrangement and degeneration of smooth muscle fibers in the media, in addition to scattered foci of mucoid accumulation and irregular-shaped cavitation in the medial extracellular matrix, raising the possibility of an intrinsic dysfunction of the vascular wall in the pathological process of pseudoaneurysm formation.

NSAIDs (nonsteroidal anti-inflammatory drugs) were administered to patients with ischemic onset-type moyamoya disease who experience headaches, but their therapeutic effect was very poor and resulted in a drop in quality of life (QOL). On the other hand, patients who were administered aspirin initially to prevent transient ischemic attacks (TIA) were observed to have a better QOL with the absence of headaches. Here, we report on patients with ischemic onset-type moyamoya disease experiencing headaches who received aspirin in order to verify its safety and effectiveness. From October 2012 to July 2014, 35 patients (male: 19, female: 16 average age: 10.5 ± 3.9) with ischemic onset-type pediatric moyamoya disease and who were admitted or commuted to hospital and had surgical treatment were evaluated for background, moyamoya staging (Suzuki), presence/absence of TIA, and platelet aggregation activity by adenosine diphosphate (ADP)/collagen turbidity test. The patients were divided into four groups depending on the intensity of headache prior to being administered aspirin, and the Kruskal-Wallis test was carried out for platelet aggregation activity and moyamoya staging. Also, the 4 × 2 χ2 test was carried out for the presence/absence of TIA. Next, the items which were significant in these tests were used as independent variables to analyze the risk of headache onset, using logistic regression analysis. One item with statistical significance was the platelet aggregation test(PAT) value (on collagen) (P

Implantation of left ventricular assist device (LVAD) is widely performed in patients with end-stage chronic heart failure. Infection and stroke are major complications after LVAD implantation. However, the incidence of intracranial mycotic aneurysm after LVAD implantation is rare, and with no standard of care. In this study, we describe a case of an intracranial mycotic aneurysm after LVAD implantation that was successfully treated with trans-arterial embolization (TAE) with N-butyl 2-cyanoacrylate (NBCA) via the brachial artery. A 49-year-old man with a history of implantation of LVAD for ischemic cardiomyopathy was admitted to our institution. He had infectious endocarditis and was administered systemic antibiotics. At 3 weeks after admission, intracranial mycotic aneurysm of the left posterior parietal artery was detected during a diagnostic examination for asymptomatic intracranial hemorrhage. Anticoagulant therapy was administered to prevent thromboembolic complications of LVAD implantation. Under local anesthesia, TAE with NBCA was performed via the brachial artery because of the tortuous anatomy of the origin of the innominate artery and implant of the aortic arch. The aneurysm was completely obliterated. The patient was discharged without neurological deficits. TAE using NBCA could be an effective modality for the treatment of intracranial mycotic aneurysm after LVAD implantation.

OBJECTIVEThe objective of this study was to assess the efficacy of unilateral pallidotomy in patients with asymmetrical cervical dystonia.METHODSThis study retrospectively included 25 consecutive patients with asymmetrical cervical dystonia refractory to botulinum toxin injections, who underwent unilateral pallidotomy between January 2015 and April 2017. Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were evaluated preoperatively and 1 week, 3 months, and 6 months postoperatively. The clinical responses were defined as good responders, exhibiting > 50% improvement in the TWSTRS score at 6 months postsurgery, or poor responders, exhibiting < 50% improvement in TWSTRS scores at 6 months postsurgery.RESULTSTwelve and 9 patients showed right- and left-side rotation, respectively; 1 and 3 patients had right- and left-side laterocollis, respectively. The mean age of onset and duration of the disease were 40.2 ± 13.9 and 8.9 ± 10.9 years, respectively. Mean TWSTRS scores were 38.4 ± 12.6 (p < 0.001), 17.3 ± 12.4 (p < 0.001), 19.5 ± 13.4 (p < 0.001), and 20.0 ± 14.7 (p < 0.001), preoperatively and 1 week, 3 months, and 6 months postoperatively, respectively. Fourteen patients (56%) demonstrated > 50% improvement in their TWSTRS total score (mean improvement of TWSTRS total score = 70.5%) 6 months postsurgically. Furthermore, preoperative TWSTRS severity score was a prognostic factor (odds ratio 1.37, 95% confidence interval 1.06–1.78, p = 0.003).CONCLUSIONSThese results suggest that unilateral pallidotomy is an acceptable treatment option for asymmetrical cervical dystonia. Further investigations with a larger number of cases and longer follow-up period are required to confirm these data.

Head tremor in patients with dystonia is referred to as dystonic tremor. During surgical treatment, numerous targets may be selected, including the internal segment of the globus pallidus and the ventral intermediate (Vim) nucleus; however, there is no consensus concerning the most effective treatment target. We report herein a case of dystonic head tremor in which improvement persisted for 5 years after deep brain stimulation (DBS) of the bilateral thalamic Vim and ventro-oral internus (Voi) nuclei. The patient, a 67-year-old woman, has a horizontal head tremor associated with cervical dystonia that had been resistant to drug treatment over 3 years. Immediately following surgery, dystonia and tremor symptoms had completely improved. Voice volume declined and dysarthria occurred but improved upon adjusting the stimulation conditions. Over 5 years, both head tremor and cervical dystonia have been completely controlled, and no other obvious complications have been observed. As the Voi nucleus receives pallidothalamic projections involved in dystonia and the Vim nucleus receives cerebellothalamic projections involved in tremors, stimulating these 2 nuclei with the same electrode appears reasonable in the treatment of dystonic tremor. This case suggests that Vim-Voi DBS may be effective for treating dystonic head tremor.

OBJECTIVEPatients with pediatric moyamoya disease (PMMD) showing recurrent symptoms or decreased cerebral blood flow after initial revascularization therapy may require additional revascularization to improve their clinical condition. The authors evaluated the clinical and hemodynamic benefits of an occipital artery (OA)–middle cerebral artery (MCA) bypass for patients with PMMD who have undergone an initial revascularization procedure.METHODSThe authors retrospectively identified 9 patients with PMMD who had undergone OA-MCA bypass between March 2013 and December 2017, and who had received a previous superficial temporal artery–MCA bypass. The following clinical data were collected: initial revascularization procedure, symptoms (presence or recurrence), pre- and postoperative cerebral blood flow and cerebrovascular reactivity (CVR) changes, posterior cerebral artery (PCA) stenosis, PCA-related and nonrelated symptoms, and latest follow-up.RESULTSPreoperatively, all patients (n = 9) suffered non–PCA-related recurrent symptoms, and 4 had PCA-related symptoms. At 1-year follow-up, all patients with PCA-related symptoms showed complete recovery. Additionally, 8 (89%) patients with non-PCA symptoms experienced improvement. Only 1 (11%) patient showed no improvement after the surgical procedure. The mean pre- and postoperative CVR values of the MCA territory were 14.8% and 31.3%, respectively, whereas the respective mean CVR values of the PCA territory were 22.8% and 40.0%.CONCLUSIONSThe OA-MCA bypass is an effective rescue therapy to improve the clinical condition and hemodynamic changes caused by PMMD in patients who experience recurrent symptoms after initial revascularization.

Each year, the Japan Neurosurgical Society (JNS) reports up-to-date statistics from the Japan Neurosurgical Database regarding case volume, patient demographics, and in-hospital outcomes of the overall cohort and neurosurgical subgroup according to the major classifications of main diagnosis. We hereby report patient demographics, in-hospital mortality, length of hospital stay, purpose of admission, number of medical management, direct surgery, endovascular treatment, and radiosurgery of the patients based on the major classifications and/or main diagnosis registered in 2018 and 2019 in the overall cohort (523283 and 571143 patients, respectively) and neurosurgical subgroup (177184 and 191595 patients, respectively). The patient demographics, disease severity, proportion of purpose of admission (e.g., operation, 33.9-33.5%) and emergent admission (68.4-67.8%), and in-hospital mortality (e.g., cerebrovascular diseases, 6.3-6.5%; brain tumor, 3.1-3%; and neurotrauma, 4.3%) in the overall cohort were comparable between 2018 and 2019. In total, 207783 and 225217 neurosurgical procedures were performed in the neurosurgical subgroup in 2018 and 2019, respectively, of which endovascular treatment comprised 19.1% and 20.3%, respectively. Neurosurgical management of chronic subdural hematoma (19.4-18.9%) and cerebral aneurysm (15.4-14.8%) was most common. Notably, the proportion of management of ischemic stroke/transient ischemic attack, including recombinant tissue plasminogen activator infusion and endovascular acute reperfusion therapy, increased from 7.5% in 2018 to 8.8% in 2019. The JNS statistical update represents a critical resource for the lay public, policy makers, media professionals, neurosurgeons, healthcare administrators, researchers, health advocates, and others seeking the best available data on neurosurgical practice.

Mutations in the lysine methyltransferase 2B (KMT2B) gene have recently been reported to be associated with childhood‐onset generalized dystonia. There have been no studies investigating ablative treatments for the management of this disorder. Three patients underwent either a staged unilateral pallidotomy and contralateral pallidothalamic tractotomy (19‐year‐old man, 2‐year follow‐up), a unilateral pallidothalamic tractotomy (34‐year‐old man, 6‐month follow‐up) or a simultaneous unilateral pallidothalamic tractotomy and ventro‐oral thalamotomy (29‐year‐old man, 6‐month follow‐up). The average total patient score on the Burke‐Fahn‐Marsden Dystonia Rating Scale‐Movement Scale improved from 39.5 to 13.2 (66.6%) after the procedures. No significant complications were identified. Ablative treatments appear to be a promising alternative surgical option for generalized dystonia with KMT2B mutation.

OBJECTIVESubarachnoid hemorrhage (SAH) has a poor outcome despite modern advancements in medical care. The development of a novel therapeutic strategy to prevent rupture of intracranial aneurysms (IAs) or a novel diagnostic marker to predict rupture-prone lesions is thus mandatory. Therefore, in the present study, the authors established a rat model in which IAs spontaneously rupture and examined this model to clarify histopathological features associated with rupture of lesions.METHODSFemale Sprague Dawley rats were subjected to bilateral ovariectomy; the ligation of the left common carotid, the right external carotid, and the right pterygopalatine arteries; induced systemic hypertension; and the administration of a lysyl oxidase inhibitor.RESULTSAneurysmal SAH occurred in one-third of manipulated animals and the locations of ruptured IAs were exclusively at a posterior or anterior communicating artery (PCoA/ACoA). Histopathological examination using ruptured IAs, rupture-prone IAs induced at a PCoA or ACoA, and IAs induced at an anterior cerebral artery–olfactory artery bifurcation that never ruptured revealed the formation of vasa vasorum as an event associated with rupture of IAs.CONCLUSIONSThe authors propose the contribution of a structural change in an adventitia, i.e., vasa vasorum formation, to the rupture of IAs. Findings from this study provide important insights about the pathogenesis of IAs.

Introduction: Moyamoya disease (MMD) is a rare cerebrovascular disease associated with cerebral infarction or hemorrhage. Hyperperfusion is the most significant complication of direct bypass surgery. Previous research has shown that an increase in cerebral blood flow (CBF) is strongly related to symptomatic hyperperfusion and highlighted the importance of postoperative assessment of CBF. Objective: The principal aims of this study were to quantitatively analyze the relationship between intraoperative graft flow and increase in CBF and to evaluate the effectiveness of intraoperative graft flow measurement during bypass surgery for patients with MMD. Methods: This study included 91 surgeries in 67 consecutive adult patients with MMD who underwent direct revascularization surgery at our institution between November 2013 and September 2018. Intraoperative graft flow of the branches and main trunk was measured in all patients, after anastomosis had been established. Postoperative CBF measurements were performed under sedation, immediately after surgery. Radiological hyperperfusion was defined as focal high uptake, as determined by CBF imaging immediately after surgery. Patients were divided into two groups (radiological hyperperfusion and nonradiological hyperperfusion groups), and the relationship between intraoperative graft flow and radiological hyperperfusion was analyzed. Results: Significant differences were observed between the radiological hyperperfusion and nonradiological hyperperfusion groups in terms of intraoperative graft flow of both the branch (median 72 vs. 42 mL/min, respectively; p < 0.01) and main trunk (median 113 vs. 68 mL/min, respectively; p < 0.01). A receiver-operating characteristic analysis was performed to test the utility of intraoperative flow as a quantitative measure. We set the cutoff values for the intraoperative branch and main trunk flow at 57 mL/min (sensitivity: 0.707, specificity: 0.702; area under the curve [AUC]: 0.773; 95% confidence interval [CI]: 0.675–0.871) and 84 mL/min (sensitivity: 0.667, specificity: 0.771; AUC: 0.78; 95% CI: 0.685–0.875), respectively. Conclusions: Measuring intraoperative graft flow during bypass surgery may be an effective means of predicting hyperperfusion and could serve to facilitate early therapeutic intervention such as strict blood pressure control.

Background Scalp arteriovenous malformation is a rare disease. In terms of treatment, surgical removal is often effective and performed. With the development of endovascular treatments, a combination of surgical removal and embolization is now often performed. Case Description A 44-year-old man presented with a mass in his left occipital region. Cerebral angiography led to a diagnosis of scalp arteriovenous malformation. Although he had no neurologic deficits, perfusion computed tomography (CT) scan showed a slight decrease in blood flow in the left cerebral hemisphere, which was presumed to have been caused by the scalp arteriovenous malformation. He suffered from a sleep disorder caused by tinnitus, and a discomfort with the lesion itself; therefore, we decided to surgically remove the lesion. To suppress intraoperative bleeding and safely perform the surgery, preoperative embolization was also planned. After treatment, he had no neurologic deficits and the sleep disorder improved. Perfusion CT scan performed after the surgery showed an improvement in cerebral blood flow in the left cerebral hemisphere. Conclusions Because cerebral blood flow may decrease depending on the progression of the lesion, the cerebral blood flow should be evaluated. Considering the treatment modalities depending on the lesion can provide treatment with less recurrence and higher patient satisfaction.

OBJECTIVEResection of gliomas in the precentral gyrus carries a risk of severe motor dysfunction. To prevent permanent, severe postoperative motor dysfunction, reliable intraoperative predictors of postoperative function are required. Since 2005, the authors have removed gliomas in the precentral gyrus with combined functional mapping and estimation of intraoperative voluntary movement (IVM) during awake craniotomy and transcortical motor evoked potentials (MEPs). The purpose of the current study was to evaluate whether intraoperative findings of combined monitoring of IVM during awake craniotomy and transcortical MEP monitoring were useful for predicting postoperative motor function of patients with gliomas in the precentral gyrus.METHODSThe current study included 30 patients who underwent resection of precentral gyrus gliomas during awake craniotomy from April 2000 to January 2018. All tumors were removed with monitoring of IVM during awake craniotomy and transcortical MEPs. Postoperative motor function was classified as stable or declined, with the extent of decline categorized as mild, moderate, or severe. We defined moderate and severe deficits were those that hindered daily life.RESULTSIn 28 of 30 cases, available waveforms were obtained with transcortical MEPs. The mean extent of resection (EOR) was 93%. Relative to preoperative status, motor function 6 months after surgery was considered stable in 20 patients and was considered to show mild decline in 7, moderate decline in 2, and severe decline in 1. Motor function 6 months after surgery was significantly correlated with IVM (p = 0.0096), changes in transcortical MEPs (decline ≤ or > 50%) (p = 0.0163), EOR, and ischemic lesions on postoperative MRI. Six patients with no change in IVM showed stable motor function 6 months after surgery. Only 2 patients with a decline in IVM and a decline in MEPs ≤ 50% had a decline in motor function 6 months after surgery (18%; 2/11 patients), whereas 11 patients with a decline in IVM and a decline in MEPs > 50% had such a decline in motor function (73%; 8/11 patients) including 2 patients with moderate and 1 with severe deficits. Three patients with moderate or severe motor deficits showed the lowest MEP values (< 100 µV).CONCLUSIONSCombined judgment from monitoring of IVM during awake craniotomy and transcortical MEPs is useful for predicting postoperative motor function during removal of gliomas in the precentral gyrus. Maximum resection was achieved with an acceptable morbidity rate. Thus, these tumors should not be considered unresectable.

Pineal region germ cell tumors are a heterogenous group of tumors; of these, pure germinoma shows high sensitivity to adjuvant therapy, and the timing and sequence of surgical intervention and adjuvant/neoadjuvant therapy are important for devising a treatment strategy for intracranial germ cell tumors (IGCT). Biopsy is diagnostically useful, but is often insufficient because only a limited amount of specimen can be obtained. In the present study, we aimed to determine the value of cerebrospinal fluid placental alkaline phosphatase (PLAP) levels, reflecting the presence of germinoma, as a reliable indicator to determine treatment strategies for pineal germ cell tumors. To assess the relationship between elevated PLAP levels and the presence of germinoma, we retrospectively reviewed histopathological findings of 25 surgical cases of IGCT in the pineal region. The PLAP value reflects the existence of a germinoma component within a total tumor volume; consequently, tumor volume could be reduced in cases with elevated PLAP, while tumors negative for PLAP did not decrease in size. Therefore, PLAP levels may help neurosurgeons optimize surgical intervention timing for teratomas in the pineal region.

Background: Paroxysmal kinesigenic dyskinesia (PKD) is a movement disorder characterized by transient dyskinetic movements, including dystonia, chorea, or both, triggered by sudden voluntary movements. Carbamazepine and other antiepileptic drugs (AEDs) are widely used in the treatment of PKD, and they provide complete remission in 80–90% of medically treated patients. However, the adverse effects of AEDs include drowsiness and dizziness, which interfere with patients' daily lives. For those with poor compatibility with AEDs, other treatment approaches are warranted.Case Report: A 19-year-old man presented to our institute with right hand and foot dyskinesia. He had a significant family history of PKD; his uncle, grandfather, and grandfather's brother had PKD. The patient first experienced paroxysmal involuntary left hand and toe flexion with left forearm pronation triggered by sudden voluntary movements at the age of 14. Carbamazepine (100 mg/day) was prescribed, which led to a significant reduction in the frequency of attacks. However, carbamazepine induced drowsiness, which significantly interfered with his daily life, especially school life. He underwent right-sided ventro-oral (Vo) thalamotomy at the age of 15, which resulted in complete resolution of PKD attacks immediately after the surgery. Four months after the thalamotomy, he developed right elbow, hand, and toe flexion. He underwent left-sided Vo thalamotomy at the age of 19. Immediately after the surgery, the PKD attacks resolved completely. However, mild dysarthria developed, which spontaneously resolved within three months. Left-sided PKD attacks never developed six years after the right Vo thalamotomy, and right-sided PKD attacks never developed two years after the left Vo thalamotomy without medication.Conclusion: The present case showed long-term suppression of bilateral PKDs after bilateral thalamotomy, which led to drug-free conditions.

Langerhans cell histiocytosis (LCH) is a multi-organ disorder that rarely involves the hypothalamic-pituitary region (HPR). HPR-LCH presents with severe progressive pituitary dysfunction and its prognosis is poor. The definitive diagnosis of LCH is considerably difficult and complicated owing to the occurrence of several diseases with similar manifestations in the HPR and its location in the deepest portion of the anterior skull base, in close proximity to important normal structures, severely limiting the size of the biopsy specimen. Chemotherapy is the established treatment modality for LCH; hence, timely and accurate diagnosis of LCH is essential for early therapeutic intervention. We retrospectively reviewed clinical features and biopsy procedures in four patients with HPR-LCH (all female, 28-44 years old) from 2009 to 2020. Maximum diameter of supra-sellar lesions was 23-35 mm and 2 cases had skip lesions. All patients demonstrated central diabetes insipidus, hyper-prolactinemia, and severe anterior pituitary dysfunction. Two of the patients had progressive disease. Furthermore, four patients presented body weight gain, two visual disturbance, and two impaired consciousness. The duration from onset to diagnosis of LCH was 3 to 10 (average 7.25) years. In total, eight operations were performed until final diagnosis. The percentage of correct diagnosis by biopsy was 50% (4/8). Clinical features of HPR-LCH are very similar to those of other HPR diseases, and their symptoms are progressive and irreversible. Clinicians should consider repeated biopsy with a more aggressive approach if the lesion is refractory to steroid therapy, in order to ensure accurate diagnosis and appropriate treatment.

BACKGROUND Trapping an aneurysm after the establishment of an extracranial to intracranial high-flow bypass is considered the optimal surgical strategy for ruptured blood blister–like aneurysms (BBAs) of the internal carotid artery (ICA). For high-flow bypass surgeries, a radial artery graft is generally preferred over a saphenous vein graft (SVG). However, SVGs can be advantageous in acute-phase surgeries because of their greater length, easy manipulability, ability to act as high-flow conduits, and reduced risk of vasospasms. In this study, the authors presented five cases of ruptured BBAs treated with high-flow bypass using an SVG followed by BBA trapping, and they reported on surgical outcomes and operative nuances that may help avoid potential pitfalls. OBSERVATIONS After the surgeries, there were no ischemic or hemorrhagic complications, including symptomatic vasospasms. In three of the five cases, postoperative modified Rankin scale scores were between 0 and 2 at the 3-month follow-up. In one case, the SVG spontaneously occluded after surgery while the protective superficial temporal artery (STA) to middle cerebral artery (MCA) bypass became dominant, and the patient experienced no ischemic symptoms. LESSONS High-flow bypass using an SVG with a protective STA-MCA bypass followed by BBA trapping is a safe and effective treatment strategy.

The field of Forel (FF) is a subthalamic area through which the pallidothalamic tracts originating from the globus pallidus internus (GPi) traverse. The FF was used as a stereotactic surgical target (ablation and stimulation) to treat cervical dystonia in the 1960s and 1970s. Although recent studies have reappraised the ablation and stimulation of the pallidothalamic tract at FF for Parkinson’s disease, the efficacy of deep brain stimulation of FF (FF-DBS) for dystonia has not been well investigated. To confirm the efficacy and stimulation-induced adverse effects of FF-DBS, three consecutive patients with medically refractory dystonia who underwent FF-DBS were analyzed (tongue protrusion dystonia, cranio-cervico-axial dystonia, and hemidystonia). Compared to the Burke-Fahn-Marsden Dystonia Rating Scale-Movement Scale scores before surgery (23.3 ± 12.7), improvements were observed at 1 week (8.3 ± 5.9), 3 months (5.3 ± 5.9), and 6 months (4.7 ± 4.7, p = 0.0282) after surgery. Two patients had stimulation-induced complications, including bradykinesia and postural instability, all well controlled by stimulation adjustments.

Background Epidermoid cysts are rare, slow-growing, benign tumors. They commonly occur at the cerebellopontine angle or the suprasellar region, and they can also occur in the skull. Intradiploic epidermoid cysts are often detected incidentally, but their progression can cause various symptoms. Case Description The patient was a 49-year-old woman who was diagnosed with otitis media with effusion based on a reported sensation of left ear blockage. Thorough examination revealed a mass lesion in the left occipital bone. Computed tomography depicted a mass lesion on the left occipital bone, part of which reached the inner table of the skull and eroded the mastoid air cell. On magnetic resonance imaging, T1-weighted signal was low but T2-weighted signal and diffusion-weighted signal were high. An intradiploic epidermoid cyst was suspected on the basis of imaging results, and it was surmised that mastoid cellulitis by the mass lesion accompanied by bone destruction had progressed to otitis media with effusion. The pathologic diagnosis was epidermoid cysts. Six months after the operation the otitis media with effusion had improved, and there had been no recurrence of epidermoid cysts. Conclusions We experienced a case of occipital bone intradiploic epidermoid cysts diagnosed in conjunction with otitis media with effusion.

Cerebellar neuromodulation could influence the pathological abnormalities of movement disorders through several connections between the cerebellum and the basal ganglia or other cortices. In the present report, the authors demonstrate the effects of cerebellar deep brain stimulation (DBS) on a patient with severe generalized fixed dystonia (FD) that was refractory to bilateral pallidotomy and intrathecal baclofen therapy. A previously healthy 16-year-old girl presented with generalized FD. Bilateral pallidotomy and intrathecal baclofen therapy had failed to resolve her condition, following which she received DBS through the bilateral superior cerebellar peduncle (SCP) and dentate nucleus (DN). Ipsilateral stimulation of the SCP or DN improved the FD, and the ability of DBS administered via the SCP to relax muscles was better than that of DN DBS. A considerable improvement of generalized FD, from a bedridden state to a wheelchair-bound state, was observed in the patient following 6 months of chronic bilateral DBS via the SCP; moreover, the patient was able to move her arms and legs. The findings in the present case suggest that neuromodulation of deep cerebellar structures is a promising treatment for FD that is refractory to conventional treatments.

Background Meningeal melanocytoma is a rare benign lesion found in the central nervous system. Preoperative diagnosis of meningeal melanocytoma is often a diagnostic challenge, as the clinical and neurologic features are often nonspecific. Various characteristics, including the natural course of this tumor, remain poorly understood. We report a case of a rapidly growing dumbbell-shaped melanocytoma compressing the spinal cord that manifested 2 years after a tumor was identified at the right C2-C3 foramen. Case Description A 40-year-old, right-handed man presented with a 2-month history of right palm and left leg numbness. Magnetic resonance imaging of the cervical spine showed a dumbbell-shaped tumor at the right C2-C3 foramen with extension into the central canal. The lesion was hyperintense on T1-weighted images and hypointense to isointense on T2-weighted images. Contrast enhancement was not visualized clearly. Fluorodeoxyglucose–positron emission tomography with computed tomography showed intense uptake in the lesion. The patient's history included a small lesion that had been localized at the right C2-C3 foramen 2 years before admission. The pathologic findings were consistent with melanocytoma. Conclusions It is important to include meningeal melanocytoma in the differential diagnosis of dumbbell tumors, as meningeal melanocytomas may show rapid progression.

Primary motor cortex (M1) infarctions sometimes cause sensory impairment. Because sensory signals play a vital role in motor control, sensory impairment compromises the recovery and rehabilitation of motor disability. However, the neural mechanism of the sensory impairment is poorly understood. We show that sensory processing in mouse primary somatosensory cortex (S1) was impaired in the acute phase of M1 infarctions and recovered in a layer-specific manner in the subacute phase. This layer-dependent recovery process and the anatomical connection pattern from M1 to S1 suggested that functional connectivity from M1 to S1 plays a key role in the sensory processing impairment. A simulation study demonstrated that the loss of inhibition from M1 to S1 in the acute phase of M1 infarctions could impair sensory processing in S1, and compensation for the inhibition could recover the temporal coding. Consistently, the optogenetic activation of M1 suppressed the sustained response in S1. Taken together, we revealed how focal stroke in M1 alters the cortical network activity of sensory processing, in which inhibitory input from M1 to S1 may be involved.

Objective Thalamic gliomas in children are less suitable for surgical resection because of their location. In cases of unavoidable resection, careful surgical planning in addition to histology and extent of resection affects prognosis. Methods A cohort of 10 pediatric patients with thalamic glioma underwent surgical resection at our department. The predominant location of tumor origins in the thalamus was defined in imaging studies. Histopathology was determined (retrospectively in a subset) according to the World Health Organization classification 2016, including the newly established type of “diffuse midline glioma, H3 K27M-mutant.” Results Three low-grade gliomas (grade I/II) and 7 high-grade gliomas (grade III/IV) were identified. The mean follow-up period was 49.8 months. All 3 low-grade gliomas did not recur (progression-free survival, 58.3 months). Six of 7 high-grade gliomas recurred, and the patients died of the primary disease (overall survival, 28.1 months). Poor outcomes, especially when located at the pulvinar region, were noticeable, with strong predictive power for poor prognosis (P = 0.0018). The presence of H3 K27M mutation and pulvinar location were closely associated (P = 0.0036). Four of 5 patients with pulvinar region tumors developed dissemination and died of the primary disease. Conclusions Pulvinar location is specifically associated with a high rate of malignancy in histology, the presence of H3 K27M mutation, and dissemination at an early disease stage. This association suggests that a distinct biological profile affects prognosis depending on location within the thalamus, especially the pulvinar. We report that tumor location is highly relevant to prognosis and should be taken into consideration when planning treatment.

Background The cause of subarachnoid hemorrhage is more likely to be intracranial than spinal. Bleeding, although common with spinal arteriovenous malformations and spinal cord tumors, rarely occurs with ruptured isolated spinal artery aneurysms. Here, we report a case of isolated thoracic posterior spinal artery aneurysm presenting with thrombosis after subarachnoid hemorrhage. Case Description A 67-year-old woman presented with sudden-onset nausea and low back and right thigh pain that worsened with movement. Computed tomography (CT) and magnetic resonance imaging (MRI) scans of the head suggested a small subarachnoid hemorrhage in the high-convexity sulcus, and lumbar puncture showed bloody cerebrospinal fluid. There was no apparent intracranial aneurysm on CT angiography; however, spinal MRI showed a lesion on the right side of the spinal cord at Th10. Contrast-enhanced CT showed an enhancing lesion at this site on day 7 that was not present on day 15. Selective right Th10 intercostal artery angiography on day 22 showed no evidence of aneurysm. The lesion was suspected to be a thrombotic spinal artery aneurysm. Given the unclear natural history of this entity, surgery was performed on day 36. After right Th10 hemilaminectomy and opening the dura, the arachnoid and adhesions were found to be thickened. A fusiform-shaped thrombosed aneurysm continuous with the radiculopial artery was removed. The patient was discharged without neurologic deterioration. Conclusions Isolated spinal artery aneurysm is a rare cause of subarachnoid hemorrhage. It is expected that additional cases will clarify the natural history and indications for treatment.

In neurosurgical operations, proper craniotomy using retractors is necessary. Various surgical instruments are used for this purpose, including standard retractors and multipurpose head frame retractor systems. However, the conventional multipurpose head frame system is often not optimal for use in some craniotomies and postures because of its size and complexity of setting. We have invented a new omnidirectional tin-alloyed (ODT) ring retractor for craniotomy with malleability and shape memory characteristics to resolve these issues. It is principally elliptical in shape, approximately 30 × 20 cm in diameter, and sufficiently firm. Accordingly, this ODT ring can retract the surgical field in all directions. Here, we report our experiences of 281 neurosurgical craniotomies using this ODT ring retractor system in various craniotomy sites and postures. Our novel ODT ring retractor is useful because of its low profile, multidirectional retractability, and less obstructiveness with its malleability. It could be used with pediatric patients where strong traction is not desirable.

Introduction: Surgical approaches to intrinsic pontine lesions are technically difficult and prone to complications. The surgical approach to the brainstem through midline pontine splitting is regarded as safe since there are no crossing vital fibers in the midline between the abducens nuclei at the facial colliculi in the pons and the oculomotor nucleus in the midbrain, although its actual utilization has not been reported previously. Case Presentation: A 6-year-old boy presented with a large intrinsic cystic lesion in the pons. We successfully achieved gross total removal via the median sulcus of the fourth ventricle. The fixation in adduction and limitation of abduction were newly observed in the left eye after surgery. Discussion: The advantage of the surgical approach through the median sulcus is the longer line of dissection in an axial direction and the gain of a wider operative view. On the other hand, the disadvantage of this approach is the limited orientation and view toward lateral side and a possible impairment of the medial longitudinal fasciculi and paramedian pontine reticular formation, which are located lateral to the midline sulcus bilaterally and are easily affected via the median sulcus of the fourth ventricular floor. Ongoing developments in intraoperative neuro-monitoring and navigation systems are expected to enhance this promising approach, resulting in a safer and less complicated procedure in the future. Conclusion: The surgical approach through midline pontine splitting is suitable for midline and deep locations of relatively large pontine lesions that necessitate a wider surgical window.

Background Vagal nerve stimulation (VNS) is an alternative palliative therapy for pharmacoresistant epilepsy. It has been reported to be effective for both focal and generalized epilepsy; however, most of the relevant studies have involved children or young patients. Some patients develop intractable epilepsy after stroke, despite taking antiepileptic drugs (AEDs). In this study, we investigated the efficacy of VNS for pharmacoresistant poststroke epilepsy (PPSE). Methods We retrospectively studied 10 patients who underwent vagal nerve stimulator implantation for poststroke epilepsy after the seizures had proved refractory to appropriate AEDs. The seizure outcome was evaluated using the McHugh classification 2 years after the implantation. Results In total, 4 patients (40%) remained seizure-free throughout the 2-year duration. In addition, seizures were reduced by >50% after 2 years in 6 patients (60%). McHugh classification was class I for 5 patients and class II for 5 patients. Neither intraoperative complications nor postoperative adverse effects were reported. The average intensity of VNS was 1.75 mA. Conclusions In this small study, VNS proved to be a safe and effective therapy for PPSE. Patients with poststroke epilepsy experience physical or mental decline; therefore, it is important to control seizures in them to avoid deterioration in their quality of daily life.

Social behavior includes a variety of behaviors that are expressed between two or more individuals. In humans, impairment of social function (i.e., social behavior and social cognition) is seen in neurodevelopmental and neurological disorders including autism spectrum disorders (ASDs) and stroke, respectively. In basic neuroscience research, fluorescence monitoring of neural activity, such as immediate early gene (IEG)-mediated whole-brain mapping, fiber photometry, and calcium imaging using a miniaturized head-mounted microscope or a two-photon microscope, and non-fluorescence imaging such as functional magnetic resonance imaging (fMRI) are increasingly used to measure the activity of many neurons and multiple brain areas in animals during social behavior. In this review, we overview recent rodent studies that have investigated the dynamics of brain activity during social behavior at the whole-brain and local circuit levels and studies that explored the neural basis of social function in healthy, in brain-injured, and in autistic human subjects. A synthesis of such findings will advance our understanding of brain mechanisms underlying social behavior and facilitate the development of pharmaceutical and functional neurosurgical interventions for brain disorders affecting social function.

OBJECTIVEIn this study on the effectiveness and safety of photodynamic therapy (PDT) using talaporfin sodium and a semiconductor laser, the long-term follow-up results of 11 patients with glioblastoma enrolled in the authors’ previous phase II clinical trial (March 2009–2012) and the clinical results of 19 consecutive patients with newly diagnosed glioblastoma prospectively enrolled in a postmarket surveillance (March 2014–December 2016) were analyzed and compared with those of 164 patients treated without PDT during the same period.METHODSThe main outcome measures were the median overall survival (OS) and progression-free survival (PFS) times. Moreover, the adverse events and radiological changes after PDT, as well as the patterns of recurrence, were analyzed and compared between the groups. Kaplan-Meier curves were created to assess the differences in OS and PFS between the groups. Univariate and multivariate analyses were performed to identify the prognostic factors, including PDT, among patients with newly diagnosed glioblastoma.RESULTSThe median PFS times of the PDT and control groups were 19.6 and 9.0 months, with 6-month PFS rates of 86.3% and 64.9%, respectively (p = 0.016). The median OS times were 27.4 and 22.1 months, with 1-year OS rates of 95.7% and 72.5%, respectively (p = 0.0327). Multivariate analyses found PDT, preoperative Karnofsky Performance Scale score, and IDH mutation to be significant independent prognostic factors for both OS and PFS. Eighteen of 30 patients in the PDT group experienced tumor recurrence, including local recurrence, distant recurrence, and dissemination in 10, 3, and 4 patients, respectively. Conversely, 141 of 164 patients in the control group experienced tumor recurrence, including 101 cases of local recurrence. The rate of local recurrence tended to be lower in the PDT group (p = 0.06).CONCLUSIONSThe results of the present study suggest that PDT with talaporfin sodium and a semiconductor laser provides excellent local control, with few adverse effects even in cases of multiple laser irradiations, as well as potential survival benefits for patients with newly diagnosed glioblastoma.

Camptocormia is a rare, involuntary movement disorder, presenting as truncal flexion while standing or walking, and is mainly observed as a feature of Parkinson’s disease (PD) and primary dystonia. Deep brain stimulation (DBS) of the globus pallidus internus is effective for refractory camptocormia observed with PD or dystonia. However, the effectiveness of pallidotomy for camptocormia has not been investigated. The authors report the case of a 38-year-old man with anterior truncal bending that developed when he was 36 years old. Prior to the onset of the symptom, he had been taking antipsychotic drugs for schizophrenia. There were no features of PD; the symptom severely interfered with his walking and daily life. He was given anticholinergics, clonazepam, and botulinum toxin injections, which did not result in much success. Because of the patient’s unwillingness to undergo implantation of a hardware device, he underwent staged bilateral pallidotomy with complete resolution for a diagnosis of tardive dystonic camptocormia. The Burke-Fahn-Marsden dystonia rating scale subscore for the trunk before and after bilateral pallidotomy was 3 and 0, respectively. No perioperative adverse events were observed. Effects have persisted for 18 months. Bilateral pallidotomy can be a treatment option for medically refractory dystonic camptocormia without the need for device implantation.

Background Ganglioneuroma is a well-differentiated benign tumor that develops from the ganglion cells of the posterior mediastinum, retroperitoneum, cervical spine, and adrenal glands. The paravertebral body, in which the sympathetic trunk exists, is a common tumor site, and tumor sometimes invades the spinal canal through the intervertebral foramen. There have been no reports regarding tumors with intradural and extradural continuity. We report a paravertebral ganglioneuroma extending between the intradural and extradural spaces and its surgical treatment. Case Description A 33-year-old man was admitted to the hospital with progressive left lower limb numbness. A dumbbell-type tumor progressing to the spinal canal via the left intervertebral foramen from the paravertebral body at L1-2 was detected, and intradural calcified lesions were found. Pathologic examination of a computed tomography–guided biopsy sample revealed a ganglioglioma. The extradural tumor was removed; however, the left lower limb pain gradually worsened. As complete block was observed on myelography, the intradural tumor was removed 8 months later. Intraoperative findings revealed that the intradural and extradural tumors were continuous through the L1 nerve root. Conclusions This is the first known reported case of paravertebral ganglioneuroma presenting in continuity between the intradural and extradural spaces.

Tumor recurrence patterns after resection of intracranial low-grade gliomas (LGG) generally remain obscured. The objective of the present retrospective study was their multifaceted analysis, evaluation of associated factors, and assessment of impact on prognosis. Study group comprised 81 consecutive adult patients (46 men and 35 women; median age, 37 years) with recurrent diffuse astrocytomas (DA; 51 cases) and oligodendrogliomas (OD; 30 cases). The median length of follow-up after primary surgery was 6.7 years. Early (within 2 years after primary surgery) and non-early (> 2 years after primary surgery) recurrence was noted in 23 (28%) and 58 (72%) cases, respectively. Fast (≤ 6 months) and slow ( > 6 months) radiological progression of relapse was noted in 31 (38%) and 48 (59%) cases, respectively. Tumor recurrence was local and non-local in 71 (88%) and 10 (12%) cases, respectively. Recurrence patterns have differed in OD, IDH1-mutant DA, and IDH wild-type DA. Early onset, fast radiological progression, and non-local site of relapse had statistically significant negative impact on overall survival of patients and were often associated with malignant transformation of the tumor (38 cases). However, in subgroup with extent of resection ≥ 90% (56 cases) no differences in recurrence characteristics were found between 3 molecularly defined groups of LGG. Recurrence patterns after resection of LGG show significant variability, differ in distinct molecularly defined types of tumors, and demonstrate definitive impact on prognosis. Aggressive resection at the time of primary surgery may result in more favorable characteristics of recurrence at the time of its development.

Subarachnoid hemorrhage due to rupture of intracranial aneurysms is a life-threatening disease. Although some previous reports have demonstrated an association between lipid accumulation and degenerative changes in aneurysmal walls in humans, epidemiological studies have failed to identify dyslipidemia as a risk factor for intracranial aneurysms. Thus, we examined whether an increase in serum cholesterol levels facilitates the progression of intracranial aneurysms in a rat model. Rats were given a high-fat diet (HFD) and subjected to an intracranial aneurysm model. The HFD elevated their serum cholesterol levels. The intracranial aneurysms induced at the anterior cerebral artery-olfactory artery bifurcation were significantly larger in the high-fat group than in the normal-chow group. Histological analysis demonstrated that the loss of medial smooth muscle layers was exacerbated in the high-fat group and indicated the presence of macrophage-derived foam cells in the lesions. In in vitro experiments, the expression levels of the pro-inflammatory genes induced by LPS in RAW264.7-derived foam cells were significantly higher than those in RAW264.7 cells. The combination of these results suggests that increased serum cholesterol levels facilitate degenerative changes in the media and the progression of intracranial aneurysms presumably through foam cell transformation.

Background Brain arteriovenous malformations (BAVMs) are vascular malformations composed of tangles of abnormally developed vasculature without capillaries. Abnormal shunting of arteries and veins is formed, resulting in high-pressure vascular channels, which potentially lead to rupture. BAVMs are generally considered a congenital disorder. But clinical evidence regarding involution, regrowth, and de novo formation argue against the static condition of this disease. Recently, the presence of the somatic activating KRAS mutations in more than half of BAVM cases was reported, suggesting the role of KRAS function in the pathogenesis. Methods KRAS mutation in codon35 (G→A, G12D; G→T, G12V) was examined by a digital polymerase chain reaction analysis using genome purified from paraffin-embedded slides of human BAVMs. We also examined protein expression of KRAS G12D in lesions to corroborate results from digital polymerase chain reaction analysis. Results We detected codon35 G→A mutation in 15 (39.5%) among 38 samples and codon35 G→T mutation in 10 (27.0%) among 37 samples we could assess mutations. There were no samples positive for both codon35 G→A and G→T mutation. The ratio of codon35 G→A mutation ranged from 0.60% to 12.28% and that of G→T was from 1.20% to 8.99%. We next examined protein expression of KRAS G12D in BAVM lesions in immunohistochemistry. A KRAS G12D mutant was detected mainly in endothelial cells of dilated vessels in lesions. Conclusions KRAS mutations in codon35 were detected in about two thirds of specimens examined. KRAS function may actively contribute to the pathobiology of BAVM and can become a therapeutic target.