Risk factors for glioblastoma in adults in Japan: an exploratory cohort study based on the Shizuoka Kokuho Database, the Shizuoka study.

PURPOSE: To elucidate the risk factors associated with the onset of glioblastoma (GBM) utilizing a comprehensive administrative claims database from a major governmental district in Japan. METHODS: Using the Shizuoka Kokuho Database (SKDB) for the period from April 2012 to September 2021, we conducted a retrospective analysis of 1,465,353 participants, identifying GBM cases using specific Japanese disease codes in conjunction with associated treatments. Risk factors were assessed using both univariable and multivariable Cox proportional hazards models. RESULTS: Within the cohort, 182 participants (0.012%) received a GBM diagnosis during the study period, resulting in an incidence rate of 2.1 per 100,000 person-years. The multivariable analysis revealed that older age, male sex, and peripheral vascular disease (PVD) significantly influenced the risk of GBM onset. No clear link was found between allergic conditions and GBM risk, in contrast to some previous research. CONCLUSION: Employing a robust health insurance database, this study revealed significant associations between GBM and factors such as age, male sex, and PVD within the Japanese population. It provides key insights into GBM epidemiology and underscores the potential of health insurance databases for large-scale oncological research.

Risk factors, treatment and survival rates of late-onset acquired haemophilia A: A cohort study from the Shizuoka Kokuho Database.

INTRODUCTION: Acquired haemophilia A (AHA) is a rare disease. The risk factors have yet to be studied. AIM: We aimed to identify risk factors for late-onset AHA in Japan. METHODS: A population-based cohort study was conducted using data from the Shizuoka Kokuho Database. The study population was defined as individuals aged ≥60 years. Cause-specific Cox regression analysis was performed to calculate hazard ratios. RESULTS: Of 1,160,934 registrants, there were 34 patients with newly diagnosed AHA. The mean follow-up period was 5.6 years, and the incidence of AHA was 5.21 per million person-years. Myocardial infarction, diabetes mellitus, solid tumors, antimicrobial agents, phenytoin and anti-dementia drugs, which showed significant differences in the univariate analysis, were excluded from the multivariable analysis because of the small number of cases. Multivariable regression analysis showed that the presence of Alzheimer's disease (hazard ratio [HR]:4.28, 95% confidence interval [CI]:1.67-10.97) and rheumatic disease (HR:4.65, 95% CI:1.79-12.12) increased the risk of AHA development. CONCLUSION: We found that comorbid Alzheimer's disease is a risk factor of AHA incidence in the general population. Our findings provide insight into the etiology of AHA, and the proof of the coexistence of Alzheimer's disease may support the recent notion that Alzheimer disease is an autoimmune disease.

Characteristics and management of hydrocephalus associated with vestibular schwannomas: a systematic review.

Hydrocephalus (HC) can be associated with vestibular schwannoma (VS) at presentation. Although spontaneous resolution of HC after VS removal is reported, first-line treatment is varied including preoperative ventriculoperitoneal (VP) shunt, external ventricular drainage (EVD), or lumbar drainage (LD). We performed a systematic review to clarify optimal management of HC associated with VS at presentation, as well as characteristics of patients with initial and persistent HC after VS removal, and prevalence of HC associated with VS. Fourteen studies were included. Patients were grouped according to the timing of HC treatment. The overall rate of VP shunts was 19.4%. Among patients who received VS removal as first-line treatment, 6.9% underwent permanent shunts. In a subgroup of 132 patients (studies with no-aggregate data), t test analysis for mean tumor size (P = 0.02) and mean CSF protein level (P < 0.001) demonstrated statistically significant differences between patients with resolved HC (3.48 cm and 201 mg/dL) and patients with persistent HC (2.46 cm and 76.8 mg/dL) after VS resection. Transient treatment of HC using EVD or LD further resolved the HC in 87.5% and 82.9% of patients, respectively, before and after VS removal. The overall prevalence of HC associated with VS in a population of 2336 patients was 9.3%. Schwannoma removal as first-line treatment is justified by its low rate of persistent HC requiring VP shunt (roughly 7%). Patients with smaller VS and lower CSF proteins present higher risk of persistent HC after schwannoma removal. Temporary treatment of HC contributes to its resolution, both before and after VS removal.

Restoration of periventricular vasculature after direct bypass for moyamoya disease: intra-individual comparison.

BACKGROUND: While periventricular anastomosis, a unique abnormal vasculature in moyamoya disease, has been studied in relation to intracranial hemorrhage, no study has addressed its change after bypass surgery. The authors sought to test whether direct bypass surgery could restore normal periventricular vasculature. METHODS: Patients who had undergone direct bypass surgery for moyamoya disease at a single institution were eligible for the study. Baseline, postoperative, and follow-up magnetic resonance angiography (MRA) scans were scheduled before surgery, after the first surgery, and 3 to 6 months after contralateral second surgery, respectively. Sliding-thin-slab maximum-intensity-projection coronal MRA images of periventricular anastomoses were scored according to the three subtypes (lenticulostriate, thalamic, and choroidal anastomosis). Baseline and postoperative MRA images were compared to obtain a matched comparison of score changes in the surgical and nonsurgical hemispheres within individuals (intra-individual comparison). RESULTS: Of 110 patients, 42 were identified for intra-individual comparisons. The periventricular anastomosis score decreased significantly in the surgical hemispheres (median, 2 versus 1; p < 0.001), whereas the score remained unchanged in the nonsurgical hemispheres (median, 2 versus 2; p = 0.57); the score change varied significantly between the surgical and nonsurgical hemispheres (p < 0.001). Of the 104 periventricular-anastomosis-positive hemispheres undergoing surgery, 47 (45.2%) were assessed as negative in the follow-up MRA. Among the subtypes, choroidal anastomosis was most likely to be assessed as negative (79.7% of positive hemispheres). CONCLUSIONS: Periventricular vasculature can be restored after direct bypass. The likelihood of correction of choroidal anastomosis is a subject requiring further studies.

[Risk Factors for Early Progression of Carotid Stenosis after Cervical Radiation Therapy].

OBJECTIVE: Carotid stenosis may occur as a late complication following cervical radiation therapy(RT);however, it may also progress in the early post-RT period. This study aimed to characterize the clinical features associated with the early progression of post-RT carotid stenosis. METHODS: We retrospectively reviewed clinical records of 30 patients who had undergone unilateral or bilateral cervical RT between January 2010 and November 2014. We analyzed the pre- and post-RT stenosis of their carotid arteries using contrast-enhanced computed tomography images. The arteries were classified as progressive or non-progressive according to the presence or absence of stenosis progression within five years after RT. Using univariate and multivariate analyses, we evaluated the following potential clinical risk factors:age;gender;history of hypertension, hyperlipidemia, diabetes mellitus, coronary artery disease, or smoking habit;antiplatelet or statin use;radiation dose;and prior presence of carotid stenosis before RT. RESULTS: In total, we reviewed 57 irradiated carotid arteries and identified 9 with early post-RT progression. Carotid stenosis before RT was observed in 88.9% of arteries in the progressive group but only 2% of arteries in the non-progressive group and it predicted progression(univariate and multiple logistic regression analyses, p<0.0001). No other clinical characteristics had a significant association with the progression of carotid stenosis. CONCLUSION: Prior presence of carotid stenosis may be a risk factor for its early progression after RT. Pre-RT screening of cervical arteries may be useful, and strict management of carotid stenosis is critical in patients with cervical radiation therapy.

[Therapeutic Effects of Low-Dose Bevacizumab for the Treatment of Recurrent Brain Metastases].

OBJECTIVE: Molecularly targeted therapy has been adopted to treat a number of cancers. Bevacizumab, a recombinant humanized monoclonal antibody against vascular endothelial growth factor, is a representative agent used in molecularly targeted therapeutic regimens. However, the therapeutic effect of bevacizumab for the treatment of brain metastases remains unknown. We report the clinical effects of low dose bevacizumab(≤2.5mg/kg/week)to treat recurrent brain metastases. METHODS: We retrospectively analyzed patients with brain metastases who had been treated with bevacizumab between 2012 and 2016 at our institution. We identified clinical characteristics, including age, gender, primary tumor site, dose of bevacizumab, therapeutic and adverse effects, and magnetic resonance imaging results. The lesions were assessed with the RECIST criteria based on gadolinium-enhanced T1-weighted, T2-weighted, and FLAIR images. Statistical analysis was performed using t-test and Fisher's exact test. RESULTS: The cohort comprised 26 patients(8 men, 18 women)with a median age of 61 years(range 39-82 years). There were no significant clinical differences between the low dose and non-low dose groups. Patients in the low dose group did not report any adverse effects from bevacizumab. Three patients with brain metastases from colon cancer are illustrated to report the clinical course of low dose bevacizumab. CONCLUSION: Low dose bevacizumab may be a safe and effective therapeutic option to treat recurrent brain metastases from bevacizumab-sensitive cancers.

Follow-up after undersized dilatation of targeted lesions in carotid artery stenting.

BACKGROUND AND PURPOSE: We assessed whether intentional undersized dilatation of targeted lesions during carotid artery stenting (CAS) carried a higher risk of in-stent restenosis (ISR) and correlation to subsequent ischemic stroke in qualifying arteries in the follow-up period. METHODS: Consecutive patients undergoing CAS between April 2003 and May 2010 were retrospectively reviewed. The use of a filter device as a distal embolic protection device (EPD) was first approved by Japanese governmental health insurance in April 2008; previously, transient balloon occlusion was used off-label. Until March 2008 (Group A), the target diameter of balloon dilatation was 80-100% of the normal vessel diameter just distal to the stenotic lesion. Moderately undersized dilatation (70-80% of the normal vessel diameter) using the distal EPD was adopted in April 2008 (Group B) in an attempt to reduce the amount of released plaque debris. RESULTS: We analyzed 132 CAS procedures (125 patients) in Group A and 53 CAS procedures (52 patients) in Group B. The mean follow-up period was 35.4 months (35.3 months in Group A and 36.0 months in Group B). Eight lesions (4.3%; 7 in Group A and 1 in Group B) developed ISR. None of the patients had symptomatic ISR, and ISR did not increase in Group B (odds ratio, 0.34; 95% confidence interval, 0.04-2.86; p = 0.32). CONCLUSIONS: Undersized dilatation of targeted lesions did not increase the risk of developing ISR, and we suggest it as a viable treatment option to prevent ischemic events during CAS.

Traumatic Falcine Artery Aneurysm Resulting in Intracranial Hemorrhage.

A 50-year-old man presented with mild unconsciousness after a fall-induced head injury. Initial imaging revealed a left-sided acute subdural hematoma. After transportation to our hospital, his condition deteriorated, leading to the discovery of a new hemorrhage and an anterior falcine artery aneurysm upon further examination. The patient underwent successful decompressive craniectomy and endovascular occlusion. This case, the first reported of a traumatic anterior falcine artery aneurysm, suggests the initial injury caused both the hematoma and aneurysm. The aneurysm's specific location near the crista galli likely contributed to the formation of the traumatic aneurysm, and the compression of the left frontal lobe by the acute subdural hematoma caused the subsequent hemorrhage. This case highlights the importance of considering traumatic aneurysms in atypical postinjury hemorrhages and adds to the understanding of traumatic intracranial aneurysms' mechanisms.

Delayed formation and rupture of intracranial aneurysm following Abiotrophia defectiva endocarditis.

Abiotrophia defectiva is a type of Streptococci and is a rare cause of infectious endocarditis. The progression and outcomes of infectious intracranial aneurysms (IIAs) associated with this species are unknown due to a limited number of reported cases. A woman in her 20s with a sudden headache had a subarachnoid haemorrhage on a head CT scan. Cerebral angiography showed an aneurysm on the right middle cerebral artery. She was diagnosed with infectious endocarditis caused by A. defectiva and underwent parent artery occlusion. Despite initiating targeted antibiotic therapy, a new IIA developed and ruptured 14 days postadmission. A second parent artery occlusion was performed on the new IIA. Following 6 weeks of continued antibiotic therapy, she underwent mitral valve repair and was discharged with no neurological symptoms. Endocarditis caused by A. defectiva can lead to the delayed formation of an IIA. Endovascular treatment was effective for repeated ruptured IIAs.

Diffusion tensor imaging combined with the dual-echo steady-state (DESS) protocol for the evaluation of the median nerve in the carpal tunnel: A preliminary study.

Background: Carpal tunnel syndrome (CTS) is diagnosed based on neurological, electrophysiology, and radiological findings. Due to the technical development of magnetic resonance imaging (MRI), the median nerve is evaluated with several MRI protocols. However, diffusion tensor imaging (DTI) combined with a dual-echo steady-state (DESS) protocol is not frequently used to evaluate the median nerve of CTS. This study aimed to evaluate the median nerve in the carpal tunnel using DTI combined with a DESS protocol. Methods: Five healthy volunteers and seven patients with CTS were enrolled. The patients underwent MRI for CTS pre- and post-operatively. The median nerve was evaluated using a 3-T MRI scanner. The parameters of the DESS protocol were as follows: Repetition time (TR)/echo time (TE) = 10.83/3.32 ms, slice thickness = 0.45 mm, field of view (FoV) = 350 × 253 × 350 mm, and 3D voxel size = 0.5 × 0.5 ×0.4 mm. The parameters of the DTI sequence were as follows: TR/TE = 4000/86 ms, slice thickness = 3 mm, FoV = 160 × 993 × 90 mm, 3D voxel size = 1.2 × 1.2 ×3.0 mm, and b value = 0.1000 s/mm2. The apparent diffusion coefficient (ADC) and fractional anisotropy (FA) values of the median nerve were statistically analyzed. Statistical significance was set at P< 0.05. Results: The FA value of healthy volunteers was 0.576 ± 0.058, while those of the patients were 0.357 ± 0.094 and 0.395 ± 0.062 pre-and post-operatively, respectively. Statistically significant differences were identified between the FA values of healthy volunteers and pre-operative/post-operative patients. The ADC values of healthy volunteers and pre-operative patients were 0.931 ± 0.096 and 1.26 ± 0.282 (10-3 mm2/s), respectively (P< 0.05). Conclusion: This MRI protocol may be useful for evaluating the median nerve in the carpal tunnel.

Hydrocephalus associated with vestibular schwannomas: perioperative changes in cerebrospinal fluid.

BACKGROUND: Cerebrospinal fluid (CSF) protein levels are known to increase in patients with vestibular schwannomas (VS) with concomitant hydrocephalus, however the only information available on perioperative changes in CSF in these patients comes from case reports. Here, we investigated the relation between CSF protein and hydrocephalus in a large series of patients undergoing resection of VS. METHOD: We classified 376 patients undergoing resection for VS at our institute into two groups, namely VS and no hydrocephalus (control, n = 319) and VS with concomitant hydrocephalus (n = 57), and compared clinical parameters. Among the 57 patients diagnosed with hydrocephalus, hydrocephalus status was examined by lumbar puncture in 20 patients with communicative hydrocephalus, and pre- and postoperative scores in CSF properties were compared. RESULTS: Patients in the hydrocephalus group were significantly older than those in the control group (mean, 55.8 vs. 43.8 years), and had a longer disease duration (median, 76 vs. 12 months), larger tumors (median, 15.6 vs. 5.5 ml), and a higher protein concentration in CSF (median, 147.3 vs. 65.1 mg/dl). Perioperative CSF samples of hydrocephalus patients showed a significantly decrease in cerebrospinal pressure after tumor removal (median, -75mmH2O), followed by a decrease in CSF protein (median, -74.5 mg/dl). No patients required the placement of a shunt. CONCLUSIONS: Extended disease duration and elevated CSF protein secondary to the presence of a tumor contribute to the occurrence of hydrocephalus. Primary maximal tumor removal for VS with coexisting hydrocephalus avoids an unnecessary shunt.

Craniocervical Junction Dural Arteriovenous Fistula and Pial Arteriovenous Fistula Presenting Concomitantly in Separate Locations with Subarachnoid Hemorrhage.

Craniocervical junction dural arteriovenous fistula and pial arteriovenous fistula are rare cerebrovascular lesions. While their pathophysiology is different, both conditions can cause intracranial hemorrhage attributable to venous congestion. We present, to our knowledge, the first case report of craniocervical junction dural arteriovenous fistula and pial arteriovenous fistula presenting concomitantly in separate locations with subarachnoid hemorrhage. This case appears to have been due to increased venous hypertension caused by a merging of the venous drainage of the 2 lesions, resulting in hemorrhage.

A Progressive Spontaneous Cervical Compression Fracture Over Years Following Long-Term Corticosteroid Use.

Spontaneous vertebral compression fractures in the cervical region can have a significant impact on a patient's condition even after surgical management. Due to the rarity of spontaneous cervical vertebral compression fractures and the lack of a comprehensive description of this condition, the establishment of a clear understanding of its natural course remains incomplete. In this case study, a 73-year-old woman on long-term corticosteroid therapy underwent combined anterior and posterior fixation for a spontaneous vertebral compression fracture at the C3-C4 level. The vertebral compression fracture gradually worsened over a span of four years. Following the surgery, the patient experienced a temporary improvement in her neurological symptoms. However, seven months after the second operation, an instrumentation failure resulted in the patient becoming bedridden. This highlights the importance of considering the potential long-term implications and monitoring patients closely even after surgical intervention.

Identification of subgroups within a Japanese older adult population for whom statin therapy is effective in reducing mortality.

Use of statins for primary prevention can reduce all-cause mortality in Asian elderly populations, but their effect and the specific effective subgroups in the elderly Japanese population remain unclear. This study examined the relationship between statin therapy for primary prevention and mortality reduction in older Japanese adults, and investigated the effective subgroups. The cohort study was conducted using the Shizuoka Kokuho Database (SKDB). Data were compared between the statin-treated group and a non-statin-treated (control) group using the inverse probability of treatment weighting (IPTW) method. In the SKDB cohort aged ≥65 years, new statin use was associated with a decreased risk of all-cause mortality (hazard ratio, 0.40; 95% confidence interval [CI], 0.33-0.48) after IPTW adjustment. The risk difference for mortality at 5 years in the statin-treated group compared with that in the control group was 0.05 (95% CI, 0.04-0.06), and the number needed to treat was 21.20 (95% CI, 18.10-24.70). In conclusion, statin use for primary prevention in older adults may reduce the risk of all-cause mortality in the population without atherosclerotic disease. Furthermore, statin use for primary prevention is feasible in patients aged 75 to <85 years and in patients with comorbidities such as diabetes, or dementia.

Administration of Tranexamic Acid After Burr Hole Craniotomy Reduced Postoperative Recurrence of Chronic Subdural Hematoma in a Japanese Regional Population.

BACKGROUND: Although tranexamic acid (TXA) has occasionally been used to prevent postoperative recurrence of chronic subdural hematoma (CSDH) after burr hole craniotomy (BC), robust evidence of its efficacy has been lacking. OBJECTIVE: To assess the efficacy and safety of postoperative oral administration of TXA after BC for CSDH among the elderly. METHODS: This retrospective, propensity score-matched cohort study was carried out with a large Japanese local population-based longitudinal cohort in the Shizuoka Kokuho Database between April 2012 and September 2020. Patients included were age 60 years or older and had undergone BC for CSDH but were not undergoing dialysis. Covariates were collected from records of the preceding 12 months from the month of first BC, and patients were followed up for 6 months after surgery. The primary outcome was repeat surgery, and the secondary outcome was death or the onset of thrombosis. Data on postoperative TXA administration were collected and compared with controls using propensity score matching. RESULTS: Of the 8544 patients who underwent BC for CSDH, 6647 were included, with 473 placed in the TXA group and 6174 placed in the control group. After 1:1 matching, repeated BC was found to have been performed in 30 of 465 patients (6.5%) in the TXA group and in 78 of 465 patients (16.8%) in the control group (relative risk, 0.38; 95% CI, 0.26-0.56). No significant difference was observed for death or the onset of thrombosis. CONCLUSION: Oral administration of TXA reduced the occurrence of repeat surgery after BC for CSDH.

Esophageal cancer presenting with brain abscess.

A 76-year-old man experienced fatigue and progressive dysphagia. He underwent endoscopy at another hospital and was diagnosed with thoracic esophageal cancer. Three days after the endoscopy, the patient was rushed to our hospital with sudden seizures of the right upper and lower extremities. Contrast-enhanced computed tomography scan revealed a ring-shaped contrast-enhanced mass formation in the left parietal lobe with edema in the surrounding brain parenchyma. Contrast-enhanced magnetic resonance imaging showed a ring-shaped lesion with a high intensity on diffusion-weighted images. He was diagnosed with a brain abscess in the left parietal lobe. For abscess drainage, a quasi-emergent small craniotomy was performed. Culture of the drainage fluid revealed Streptococcus species and Haemophilus parainfluenzae. After 6 weeks of antibiotic therapy, the patient underwent a thoracoscopic esophagectomy. After the esophagectomy, there was no recurrence of the brain abscess for more than 2 years and only symptomatic epilepsy remained. Conclusively, although brain abscesses caused by esophageal cancer are rare, the possibility of brain abscess and metastasis should be considered when patients present with convulsions or higher brain disorders.

Percutaneous transluminal angioplasty for atherosclerotic stenosis of the subclavian or innominate artery: angiographic and clinical outcomes in 36 patients.

The purpose of the study was to evaluate stenting and percutaneous transluminal angioplasty (PTA) for the treatment of stenotic lesions of the subclavian or innominate artery based on surgical results and long-term follow-up with 36 patients. In particular, we evaluated the efficacy of self-expanding stents compared to balloon-expandable stents. Between February 2000 and March 2008 at the Kyoto Medical Center, 36 patients underwent both stenting and PTA of the subclavian or innominate artery. Twenty-four patients had severe subclavian stenotic disease, ten patients had total occlusion of the subclavian artery, and two patients had stenoses of the innominate artery. Successful dilatation (less than 30% residual stenosis) was obtained in 34 of the 36 cases. In two cases (20%) of total subclavian occlusion, the guidewires were not able to penetrate the lesions, although the success rate was 100% for stenoses. All patients had no signs of neurological side effects with the exception of two pseudoaneurysms of the femoral arteries that required surgical intervention. In the first 30 days after treatment, there were no strokes or deaths. Outpatient follow-up was done with 30 patients (83.3%) after a mean of 30.9 months (range 3-114). Among these 30 patients, four patients (13.3%) developed restenoses of over 50%. Restenoses occurred in 4 of 20 individuals (20%) who received balloon-expandable stents but were not observed in those who received self-expanding stents. Endovascular therapy for the subclavian and innominate arteries is less invasive and safer than open surgery, making it the preferable option. In this clinical period, the rate of restenosis using self-expanding stents was lower than the rate using balloon-expandable stents.

A Case of an Early Pregnant Woman with Congenital Protein S Deficiency Who Underwent Mechanical Thrombectomy.

Objective: Large vessel occlusion (LVO) stroke during pregnancy is rare but a life-threatening issue for the mother and fetus. We report a rare case of a pregnant woman with congenital protein S deficiency who underwent mechanical thrombectomy. Case Presentation: A 35-year-old woman presented with right hemiplegia and aphasia. The National Institutes of Health Stroke Scale was 23 and MRI revealed acute infarction on the left hemisphere. MRA showed disruption of the left middle cerebral artery. Mechanical thrombectomy was performed following intravenous thrombolysis, and then complete recanalization was achieved. The reduction in protein S activity due to pregnancy was suspected to have affected LVO. Subsequently, the patient was diagnosed with congenital protein S deficiency and recovered to modified Rankin scale 2 at 3 months after the onset. Conclusion: Aggravation of congenital protein S deficiency due to pregnancy led to the onset of LVO. The patient showed a good outcome after mechanical thrombectomy.

Ruptured Blister-like Aneurysm Arising From the Proximal Posterior Cerebral Artery.

BACKGROUND: Blister-like aneurysms (BLAs) arise mostly at the supraclinoid internal carotid artery. We report a rare case of ruptured BLA arising at the P1 segment of the posterior cerebral artery (PCA). CASE DESCRIPTION: A 34-year-old woman presented with disturbance of consciousness. Computed tomography (CT) of the head showed diffuse subarachnoid hemorrhage (SAH). A tiny bulge on the right PCA P1 segment was observed on initial CT angiography. The lesion enlarged little-by-little, with re-rupture occurring 10 days after initial hemorrhage. We diagnosed BLA arising at the P1 segment, and performed emergent endovascular parent artery occlusion (PAO) of the P1 segment. No infarction was observed in the territory of the PCA postoperatively. CONCLUSIONS: Proximal PCA is a rare but possible location for BLA. When the cause of bleeding SAH cannot be identified, repeated radiologic assessments including posterior circulation should be performed. If perforators of the unaffected site supply the thalamus and midbrain bilaterally and an ipsilateral posterior communicating artery exists, PAO of P1 seems feasible as a treatment. Elective intervention is not recommended because of the characteristics of ruptured BLAs.