[A Surgical Case of Currarino Syndrome with Syringomyelia].

Currarino syndrome is characterized by a triad of anorectal malformations, sacral defects, and presacral masses. Although it is not extremely rare, this report presents a surgical case of Currarino syndrome with syringomyelia and discusses related literature. The patient is a girl, aged 2 years and 2 months, who presented with marked constipation, chronic cystitis, and lower limb weakness. After examining the patient through magnetic resonance imaging, we diagnosed her with rectal compression due to meningocele and syringomyelia. The base of the meningocele was detached, and the spinal cord was untethered. One week after surgery, her lower limb weakness and constipation improved. Following up on symptoms and performing imaging is essential to determine a treatment plan for Currarino syndrome. (Received 28 February, 2023; Accepted 22 March, 2023; Published 1 August, 2023).

Perioperative Surgical Risks in Patients With Hemangioblastomas: A Retrospective Nationwide Review in Japan.

BACKGROUND: The perioperative risk of sporadic hemangioblastomas (HBs) and von Hippel-Lindau disease (VHL)-associated hemangioblastomas (VHL-associated HBs) remains unclear due to the rare prevalence of HB. Therefore, this study aimed to clarify risk factors for better surgical management of patients with HBs. METHODS: A retrospective analysis of surgically treated HB patients registered in the Diagnosis Procedure Combination database of Japan, between 2010 and 2015, was performed. Age, sex, sporadic HBs or VHL-associated HBs, medical history, tumor location, hospital case load, postoperative complications, and Barthel index (BI) deterioration were assessed. We also evaluated the outcomes and factors of perioperative BI deterioration. RESULTS: In total, 676 patients with 609 intracranial lesions, 64 spinal lesions, and 3 with both types were eligible. Among them, 618 and 58 patients had sporadic HBs and VHL-associated HBs, respectively. The rates of perioperative BI deterioration were 12.5% and 12.2% for sporadic HBs and VHL-associated HBs, respectively. Perioperative mortality was 1.8% and 0% for sporadic HBs and VHL-associated HBs, respectively. Male sex, old age, high hospital case load, and medical history of diabetes mellitus were significantly associated with perioperative BI deterioration in all cases and sporadic HBs. Only medical history of diabetes mellitus was a significant risk factor for perioperative BI deterioration in VHL-associated HBs. CONCLUSIONS: No differences in perioperative BI deterioration rates between sporadic HBs and VHL-associated HBs were found. However, different risk factors for perioperative BI deterioration were identified. Consideration of these risk factors is recommended in all patients undergoing surgery for HB.

Factors associated with the location of perivascular space enlargement in middle-aged individuals undergoing brain screening in Japan.

OBJECTIVE: In elderly populations, the enlargement of the perivascular space is related to small vessel disease and the glymphatic system. Enlarged perivascular spaces (EPVS) in the basal ganglia (EPVS-BG) and EPVS in the centrum semiovale (EPVS-CSO) are associated with different pathophysiological processes. However, the prevalence of EPVS and the factors associated with EPVS location in healthy middle-aged individuals are still unclear. We aimed to determine the prevalence of EPVS and the factors associated with EPVS location among healthy individuals in their 40 s METHODS: This study included 5000 consecutive healthy individuals who underwent screening for brain diseases in Japan from August to December 2018. Of them, the data of individuals in their 40 s were extracted and analyzed. The associations of age, sex, body mass index, smoking and drinking history, and medical history with EPVS location were investigated. Similar analyses were performed for the other age groups. A literature review on the factors associated with EPVS location was also performed. RESULTS: A total of 1720 individuals in their 40 s were finally included. The prevalence of EPVS-BG and EPVS-CSO was 7.7% and 9.2%, respectively. Age (years), smoking history, and hypertension were associated with EPVS-BG; none of the studied factors were found to be associated with EPVS-CSO. In the elderly, the factors previously reported to be associated with EPVS-BG included atherosclerosis change, while the factors associated with EPVS-CSO were cerebral amyloid angiopathy-related formation. CONCLUSION: Both EPVS-BG and EPVS-CSO occurred among healthy individuals in their 40 s, but they did so rarely, and less prevalently than in older age groups. EPVS-BG and EPVS-CSO may represent early imaging signs of the atherosclerotic and cerebral amyloid angiopathy processes, respectively. DATA AVAILABILITY: The anonymized data for this study will be shared upon any qualified investigator's request to the corresponding author. Primary data from this study will be made available upon reasonable request in accordance with the review board of the research institute.

A nationwide registry study: The 5-factor modified frailty index of meningioma surgery in non-elderly and elderly patients.

OBJECTIVES: The simplified 5-factor modified frailty index (mFI-5) is a useful indicator of outcome for patients undergoing surgeries as frailty is considered an important risk factor in elderly patients. However, its usefulness has not been validated based on age groups. Therefore, we aimed to investigate risk factors, including the mFI-5, across age groups for complications and worse outcomes in meningioma surgery using data obtained from the nationwide database in Japan. METHODS: We extracted data from the nationwide registry database in Japan between 2010 and 2015. Age (< 65, 65-74, and ≥ 75 years), sex, Barthel Index (BI), mFI-5 scores, and complications were evaluated. Multivariate logistic regression analyses identified risk factors for worsening BI scores and complications after surgery across all age groups. RESULTS: Among 8138 included patients, an mFI-5 score ≥ 2 items was a significant risk factor for worsening BI scores in patients aged < 65 years (odds ratio: 2.3; 95 % confidence interval: 1.5-3.4), but not in patients aged 65-74 years and those aged ≥ 75 years, contrary to chronological age. Similar results were noted for any complications in patients aged < 65 years (2.5; 1.8-3.6) and aged 65-74 years (1.5; 1.1-2.1), but not in patients aged ≥ 75 years. CONCLUSION: Although the mFI-5 scores could predict the risk of in-hospital worsening outcomes, mortality, and complications, it was more useful in non-elderly patients aged < 65 years rather than in elderly patients aged ≥ 75 years, contrary to chronological age. Further prospective studies should be performed in the future to clarify the utility of the mFI-5.

Risk Factor for Poor Outcome in Elderly Patients with Aneurysmal Subarachnoid Hemorrhage Based on Post Hoc Analysis of the Modified WFNS Scale Study.

OBJECTIVE: There is currently no precise guide for the treatment and management of elderly patients with aneurysmal subarachnoid hemorrhage (aSAH). Thus, the aim of this study was to clarify the factors of poor outcome and mortality in elderly patients with aSAH. METHODS: In the modified World Federation of Neurosurgical Societies (mWFNS) scale study, 1124 patients were divided into 2 groups, elderly (age ≥65 years) and non-elderly (age <65 years), with aSAH investigated between October 2010 and March 2013 in Japan. The odds ratio (OR) and 95% confidence interval (CI) of each risk factor was calculated through multivariate logistic regression analysis for poor outcomes, as indicated by the modified Rankin Scale (mRS) score ≥3 and mortality at 3 months after onset in each group. RESULTS: Both groups demonstrated that the mWFNS scale was significant as a grade order risk factor for poor outcomes and mortality associated with disease. In the elderly group, risk factors for poor outcomes at 3 months after onset were older age (OR 1.10, 95% CI 1.06-1.14), male sex (OR 2.03, 95% CI 1.10-3.73), and severe cerebral vasospasm category (OR 10.13, 95% CI 4.30-23.87). Risk factors for mortality at 3 months after onset were older age (OR 1.06, 95% CI 1.01-1.11) and severe vasospasm category (OR 2.17, 95% CI 1.00-4.72). CONCLUSIONS: The mWFNS scale is a useful prognostic predictor for both non-elderly and elderly patients with aSAH. Elderly male patients with aSAH presenting with severe vasospasm should be managed more carefully.

Characteristics of Cerebral Aneurysms in Japan.

The characteristics of cerebral aneurysms in Japan differ from other countries in the higher incidence of unruptured cerebral aneurysm detected by brain check-up screening, higher rupture rate of unruptured cerebral aneurysm, higher incidence of subarachnoid hemorrhage, and superior outcome after subarachnoid hemorrhage based on meta-analysis. Head shape, genetic features, environmental factors, demographics, and medical system in Japan are also different from other countries. Unruptured cerebral aneurysms are 2.8 times more likely to rupture in Japanese than western aneurysms, resulting in the highest incidence of subarachnoid hemorrhage in the world. The exact and specific mechanisms of de novo, growth, and rupture of cerebral aneurysms have not been elucidated. Investigations will contribute to the understanding of cerebral aneurysms and subarachnoid hemorrhage worldwide. Some features of cerebral aneurysm in Japan are discussed for possible research guidance in the elucidation of the predominance of subarachnoid hemorrhage in Japan.

Questionnaire Survey Regarding Prevention of Surgical Site Infection after Neurosurgery in Japan: Focus on Perioperative Management and Administration of Surgical Antibiotic Prophylaxis.

Various guidelines regarding surgical site infection (SSI) have recently been established. However, perioperative management of the wound and use of antibiotics have never been standardized completely in departments of neurosurgery in Japan. This survey investigated current perioperative management and administration of surgical antibiotic prophylaxis (SAP) and compared with guidelines intended to reduce SSI associated with neurosurgery in Japan. Questionnaires were distributed to members of the conference on Neurosurgical Techniques and Tools and the Japan Society of Aesthetic Neurosurgery via internet. The questionnaires asked about methods of perioperative management. A total of 255 members returned answers to the questionnaires. The questionnaires revealed that partial or no removal of the hair and hair shampooing at the day before surgery were performed in 96.1% and 88.1% of each institute following the World Health Organization (WHO) guidelines. Use of SAP at just before, during, and after surgery were 65.0%, 86.2%, and 63.0%, respectively. The postoperative period of use of intravenous SAP prolonged beyond 24 h in 80.0% against the recommendation of WHO. Perioperative management of wounds and use of SAP varies in institutes in Japan and some procedures were far different from the WHO guidelines. Japanese neurosurgeons should notice the prolonged SAP and comply with the WHO guidelines.

Ruptured dissecting aneurysms of the A1 segment of the anterior cerebral artery: three case reports and a review of the literature.

Dissecting aneurysms in the anterior cerebral artery (ACA), although rare, can cause ischemic and/or hemorrhagic stroke. Hemorrhagic dissecting aneurysms in the A1 portion of the ACA are often associated with a poor prognosis. We retrospectively investigated three rare cases of hemorrhagic dissecting aneurysms in the A1 portion. Dissecting aneurysms were diagnosed by carotid angiography or computed tomography angiography to visualize morphological changes in the vessel. All patients presented with diffuse subarachnoid hemorrhage. In one case, computed tomography angiography performed at the onset of the subarachnoid hemorrhage revealed fusiform dilatation at the right ACA (A1), which did not appear on a magnetic resonance angiogram obtained 1 year prior to the onset of the subarachnoid hemorrhage. In the other two cases, A1 dissecting aneurysms were diagnosed from a growing aneurysmal bulge revealed at a non-bifurcated site via repeated carotid angiography. Two patients underwent surgical intervention (trapping or clipping), and their outcome was favorable, whereas the third patient died of delayed rebleeding before receiving surgical treatment. Hemorrhagic dissecting aneurysms in the A1 portion cause severe subarachnoid hemorrhage. Surgical treatments that include revascularization are necessary to prevent rebleeding, and direct surgery is recommended, particularly at the A1 portion.

[A Case of Cervical Spinal Dural Arteriovenous Fistula with Extradural Drainage Presenting with Subarachnoid Hemorrhage due to a Ruptured Anterior Spinal Artery Aneurysm].

We report a rare case of a cervical spinal dural arteriovenous fistula(AVF)at the C2 level presenting with subarachnoid hemorrhage(SAH)due to a ruptured anterior spinal artery aneurysm. A 61-year-old man presented with sudden onset headache. Initial computed tomography revealed SAH around the brainstem. Digital subtraction angiography(DSA)demonstrated a cervical dural AVF that was fed by the left C1 radicular, left C2 radicular, and anterior spinal arteries, and drained into the epidural plexus. An aneurysm in the branch of the cervical anterior spinal artery was considered the bleeding point. A left lateral suboccipital craniotomy and C1 hemilaminectomy were performed on day 43. The feeding arteries were clipped, followed by coagulation of the draining veins. However, the aneurysm was not clipped because we deemed that obliteration of the aneurysm would be difficult without disrupting the blood flow of the parent artery. The patient showed no neurological deterioration after the operation. Postoperative DSA revealed residual dural AVF. Therefore, a second surgery was performed. After the second open surgery, DSA showed that the dural AVF and aneurysm disappeared. The patient also showed no neurological deterioration after the second surgery.

A case of transient hypothermia after trans-lamina terminalis and third ventricle clipping of an extremely high-position basilar tip aneurysm.

Reports on the trans-lamina terminalis and trans-third ventricular approach are rare. The risk associated with this approach is unknown. After an unsuccessful endovascular surgery, we performed direct surgical clipping via the third ventricle on a 78-year-old woman presenting with an extremely high-positioned, ruptured basilar tip aneurysm. She experienced transient hypothermia for 5 days, and it was considered that this was due to hypothalamic dysfunction. It is necessary to recognize that there is the potential for hypothermia after surgery via the lamina terminalis and third ventricle, even though the mechanisms of hypothalamic thermoregulation are still unclear.

[A case of cavernous angioma at the convexity in the dura mater: characteristics of images in the literature].

We describe a rare case of cavernous angioma in the dura mater and discuss published MRI findings on similar cases. The patient was a 78-year-old woman who was referred to Shimane Prefectural Central Hospital with complaints of headaches. We were subsequently able to identify a tumor at the convexity in the dura mater. The tumor showed a high intensity on T2-weighted images and was heterogeneously enhanced on contrast-enhanced T1-weighted images. The maximum size of the tumor was 35 mm. Moreover, preoperative angiography showed a slight vascularity in the tumor. We performed surgery with the expectation of finding a meningioma, metastatic brain tumor, or another mesenchymal tumor. The tumor was dark and red, attached to the dura mater, and adhered to the arachnoid. However, we were able to peel the tumor away from the meninges and achieved a total removal of the mass, successfully cutting a fine feeding cortical artery. The patient was discharged without neurological defects 9 days after surgery;the pathological diagnosis was cavernous angioma. In conclusion, it is difficult to discern between meningioma and cavernous angioma in the dura mater. However, the specificity of high intensity on T2-weighted images is relatively high, and preoperative diagnosis can be determined by MRI and angiography findings.

Extracranial internal carotid artery pseudoaneurysm associated with neurofibromatosis type 1 treated with endovascular stenting and coil embolization.

An internal carotid artery (ICA) pseudoaneurysm associated with neurofibromatosis type 1 (NF-1) is rare. We report the first case of unruptured extracranial pseudoaneurysm of the ICA in a patient with NF-1 successfully treated with endovascular stenting and coil embolization.A 66-year-old woman diagnosed with NF-1 had sudden left neck pain and massive swelling 3 years earlier. Radiological examination showed a ruptured pseudoaneurysm of the left internal thoracic artery (ITA). The posttreatment computed tomography (CT) scan revealed complete obliteration of the aneurysm of the left ITA and an unruptured pseudoaneurysm of the right ICA. After 3 years of follow-up, a CT scan revealed the enlargement of the pseudoaneurysm of the right extracranial ICA. Endovascular stenting and coil embolization were performed to prevent rupture, and the lesion was completely obliterated. Follow-up angiography at 6 months revealed good flow of the ICA through the stent without any filling of the aneurysm.

[A case of tumor progression after gamma knife radiosurgery for brain metastasis of thyroid papillary carcinoma in deep subcortical white matter].

We report a case of a brain metastasis of thyroid papillary carcinoma. A 67-year-old man had undergone a radical operation for thyroid papillary carcinoma 6 years before. He had no neurological deficit, but CT showed an enhanced lesion in the left frontal lobe. He underwent gamma knife radiosurgery. Four months later, the lesion with massive peritumoral edema was identified on magnetic resonance imaging (MRI). The edema was treated medically, however, recovery was seen neither on MRI nor clinically. Then left craniotomy was performed, with total resection of the tumor. During the operation Komai's stereotactic instruments were used for CT guided stereotactic surgery. Histopathological analysis of the surgical specimen confirmed mixed necrosis and papillary carcinoma of the thyroid gland. The patient was discharged without neurological deficit and is now kept under observation as an outpatient. Brain metastases from thyroid cancer are rare and we report a case of resection of brain metastasis of a thyroid papillary carcinoma after gamma knife radiosurgery.

[Accessory nerve schwannoma of the intracisternal type: a case report].

Accessory nerve schwannomas are extremely rare and they are classified into three types according to their locations; intrajugular, intracisternal and spinal canal type. We report a case of intracisternal schwannoma that arose from the spinal accessory nerve roots and we describe it's clinical characteristics. A-48-year-old female was admitted to our hospital with a complaint of left occipital pain. Magnetic resonance imaging (MRI) showed a well-defined mass 2.5×1.5 cm in the left cerbellomedullary cistern. It was enhanced heterogeneously with gadolinium. Cerebral angiography showed a mildly hypervascular lesion. Total removal of the tumor was performed by the left lateral suboccipital approach and the histological diagnosis was schwannoma.

The combination of low cytoplasmic and high nuclear expression of p27 predicts a better prognosis in high-grade astrocytoma.

BACKGROUND: The function of the cyclin-dependent kinase (cdk) inhibitor p27 is regulated by translocation between the nucleus (activate) and the cytosol (inactivate). No previous reports have examined the subcellular localization of p27 in glioma which was evaluated here regarding the prognosis in high-grade astrocytomas. PATIENTS AND METHODS: The pattern of subcellular localization of p27 expression was examined immunohistochemically in 49 patients with high-grade astrocytoma who were over 20 years of age. The relationship between p27 localization and the prognosis was statistically examined. RESULTS: Kaplan-Meier survival analysis showed that cytoplasmic p27 expression was statistically associated with a worse prognosis (p = 0.0203), while nuclear p27 expression showed some tendency towards a better prognosis (p = 0.1180). Cox multiple regression analysis showed the combination of high nuclear and low cytoplasmic p27 expression associated with a significantly better prognosis in high-grade astrocytoma. CONCLUSION: A combination of low cytoplasmic and high nuclear expression of p27 predicts a better prognosis in high-grade astrocytomas and thus the subcellular localization of p27 expression is useful for predicting the prognosis for these patients.

Penetrating injury of the upper cervical spine by a chopstick--case report.

A 35-year-old man presented with penetrating spinal injury after attempting suicide by stabbing a wooden chopstick into his mouth. The object penetrated the pharynx, and the tip entered the spinal canal of the atlantoaxial vertebrae. Emergent surgery disclosed that the tip of the chopstick had penetrated between the dural sac and the vertebral artery. There was no dural tear or vertebral artery injury. The foreign body was removed successfully from the oral side. He recovered without neurological sequelae.

Retinoblastoma protein prevents staurosporine-induced cell death in a retinoblastoma-defective human glioma cell line.

OBJECTIVE: To investigate the mechanism of staurosporine-induced glioma cell death and cell cycle arrest using adenovirus-mediated gene transfection, as well as the function of retinoblastoma (Rb) and genetic instability induced by staurosporine. METHODS: Cell cycle regulation, cell death and nuclear abnormalities induced by staurosporine were examined using an adenovirus vector expressing Rb, p16 or p21 genes in human glioma cell lines. RESULTS: The Rb-defective SF-539 cell line was resistant to staurosporine compared with cell lines expressing intact Rb. SF-539 glioma cells exposed to staurosporine became multinucleated and then died. Multinucleation was prevented in SF-539 cells transfected with the Rb gene, thus decreasing the death rate of these cells. CONCLUSIONS: These results imply that enforced Rb expression protects cells from genomic instability induced by staurosporine regardless of its upstream molecular effects.

Geminin: a good prognostic factor in high-grade astrocytic brain tumors.

BACKGROUND: Geminin is a nuclear protein that belongs to the DNA replication inhibitor group. It inhibits DNA replication by preventing Cdt1 from loading minichromosome maintenance protein onto chromatin, as is required for DNA replication. For this study, the authors investigated geminin expression in high-grade astrocytic tumors, including anaplastic astrocytoma (AA) and glioblastoma multiforme (GBM), with a view to predicting clinical outcomes on this basis in patients with these malignant brain tumors. METHODS: Immunohistochemistry was used to detect geminin expression in 51 patients with high-grade astrocytic tumors (19 AA and 32 GBM). Samples were categorized by taking the median value as the cut-off point for constructing Kaplan-Meier curves. The relation of geminin expression to clinical outcome in these malignant brain tumors was analyzed by using the Kaplan-Meier method and a Cox proportional hazards regression model. RESULTS: Geminin was expressed in all high-grade astrocytomas (mean geminin labeling index [LI], 24.90%). Kaplan-Meier curves showed that the group with higher geminin LI (>or=22.50%) had a better prognosis than the group with lower LI (<22.50%; P = .0296). Similarly, the Cox regression analysis showed that geminin expression has a significant correlation with survival in patients with high-grade astrocytoma (P = .0278), especially in an early stage. CONCLUSIONS: Although it is an inhibitor of DNA proliferation and, thus, is a cell cycle inhibitor, geminin expression was found in all malignant astrocytic tumors. The geminin LI was a significant predictive factor of outcomes in patients with high-grade astrocytoma, with higher expression indicating a good prognosis.

Survivin subcellular localization in high-grade astrocytomas: simultaneous expression in both nucleus and cytoplasm is negative prognostic marker.

OBJECTIVE: Subcellularly localized (nuclear and/or cytoplasmic) survivin has various functions, and correlates with prognosis of malignant tumors. However, there have been no reports about the significance of subcellularly localized survivin in high-grade astrocytomas. The aim of the present study was to examine the relationship between prognosis and subcellular localization of survivin in high-grade astrocytoma. METHODS: We immunohistochemically examined the pattern of subcellular localization of survivin expression (nuclear, cytoplasmic, or both) in 51 patients with high-grade astrocytoma (19 anaplastic astrocytomas; 32 glioblastomas). We statistically examined the relationship between survivin localization and prognosis, using multivariate analysis including other clinicopathological factors (age, sex, WHO grade, extent of resection, MIB-1 labeling index, and expression of p53 and epidermal growth factor receptor). RESULTS: All specimens stained positive for survivin: localized in nucleus only (nuclear-positive group), 10 cases (20%); localized in cytoplasm only (cytoplasmic-positive group), 23 cases (45%); simultaneous expression in nucleus and cytoplasm (nuclear-cytoplasmic group), 19 cases (35%). There was no significant difference in prognosis between the nuclear-positive group and cytoplasmic-positive group (P=0.796). However, the nuclear-cytoplasmic group had significantly shorter overall survival than the nuclear-positive group and the cytoplasmic-positive group (P<0.0001). CONCLUSIONS: We found that simultaneous expression of survivin in both the nucleus and cytoplasm is an important prognostic factor for high-grade astrocytoma. The present findings indicate that subcellular localization of survivin expression is a reliable prognostic factor for patients with this tumor.