Dural Arteriovenous Fistula in Neuro-Behçet's Disease: Association or Chance?

Behçet's disease (BD) is a multisystemic vasculitis disorder. Neuro-Behçet's disease (NBD) is a set of neurologic symptoms imputable to an underlying Behçet vasculitis. Among the wide range of vascular abnormalities secondary to BD, a dural arteriovenous fistula (dAVF) is not classically described. Whether a dAVF is associated with BD or dAVF is a chance occurrence is still a matter of debate. Herein, we describe an NBD case of a 48-year-old male, presenting with headache and fever, where a dAVF was seen on imaging. He was treated with prednisolone and colchicine, followed by the surgical resection for dAVF. Then, we discuss the possible association between BD and dAVF based on the latest literature.

Anatomy of the Frontal Sinus Drainage Pathway Evaluated in 247 Cadavers to Prevent Cerebrospinal Fluid Leakage After Frontobasal Craniotomy.

BACKGROUND AND OBJECTIVES: The frontal sinus (FS) drainage pathway (FSDP) may be a conduit for cerebrospinal fluid leakage after frontobasal craniotomy. In this cadaveric study, we aimed to evaluate the anatomy of the FSDP. METHODS: The FSs and FSDPs of 247 cadavers were investigated. We counted the number of FSs and FSDPs in each half-head, verified the presence of a narrowing section in each FSDP, and evaluated the depth, shape, and size of each narrowing FSDP section. RESULTS: We investigated 494 sides and 472 FSDPs of 247 cadavers. FSs were unilaterally undeveloped in 13 of 247 cadavers (5.3%) and bilaterally in 8 (3.2%). FSs were unilaterally duplicated in 7 of 247 cadavers (2.8%), and no FSs were bilaterally duplicated or triplicated. No FSs had 2 or more FSDPs, and all 472 investigated FSDPs were invariably narrowed at various depths. The narrowing FSDP sections were elliptical (78.6%), circular (18.1%), triangular (1.8%), or crescent-shaped (1.4%) and of varying thickness and orientation. Although FSDPs were asymmetric in 92.2% of cadavers and narrowing FSDP sections were located deep (8.9 ± 4.4 mm from the anterior skull base), the narrowing FSDP sections were typically small (area: 5.9 ± 3.3 mm 2 ) or thin (short diameter: 2.1 ± 0.7 mm). CONCLUSION: Each FS had only one FSDP, all FSDPs were invariably narrowed at various depths, and the narrowing FSDP sections were sufficiently small or thin to allow local closure, facilitating prevention of cerebrospinal fluid leakage after frontobasal craniotomy.

Single-Cell Analysis of Unidirectional Migration of Glioblastoma Cells Using a Fiber-Based Scaffold.

Glioblastoma (GBM) is a malignant incurable brain tumor in which immature neoplastic cells infiltrate brain tissue by spreading along nerve fibers. The aim of the study was to compare the migration abilities of glioma cells with those of other cancer cells and elucidate the migratory profiles underlying the differential migration of glioma cells using a fiber-based quantitative migration assay. Here, wound healing and transwell assays were used to assess cell mobility in four cell lines: U87-MG glioblastoma cells, MDA-MB-231 breast cancer cells, HCT116 colorectal cancer cells, and MKN45 gastric cancer cells. We also assessed cell mobility using a fiber model that mimics nerve fibers. Time-lapse video microscopy was used to observe cell migration and morphology. The cytoskeleton arrangement was assessed in the fiber model and compared with that in the conventional cell culture model. The conventional evaluation of cell migration ability revealed that the migration ability of breast cancer and glioblastoma cell lines was higher than that of colon cancer and gastric cancer cell lines. The fiber model confirmed that the glioblastoma cell line had a significantly higher migration ability than other cell lines. Tubulin levels were significantly higher in the glioblastoma cells than in other cell lines. In conclusion, the developed fiber-based culture model revealed the specific migratory profile of GBM cells during invasion.

Nanofiber-Mâché Hollow Ball Mimicking the Three-Dimensional Structure of a Cyst.

The occasional malignant transformation of intracranial epidermoid cysts into squamous cell carcinomas remains poorly understood; the development of an in vitro cyst model is urgently needed. For this purpose, we designed a hollow nanofiber sphere, the "nanofiber-mâché ball." This hollow structure was fabricated by electrospinning nanofiber onto alginate hydrogel beads followed by dissolving the beads. A ball with approximately 230 mm3 inner volume provided a fibrous geometry mimicking the topography of the extracellular matrix. Two ducts located on opposite sides provided a route to exchange nutrients and waste. This resulted in a concentration gradient that induced oriented migration, in which seeded cells adhered randomly to the inner surface, formed a highly oriented structure, and then secreted a dense web of collagen fibrils. Circumferentially aligned fibers on the internal interface between the duct and hollow ball inhibited cells from migrating out of the interior, similar to a fish bottle trap. This structure helped to form an adepithelial layer on the inner surface. The novel nanofiber-mâché technique, using a millimeter-sized hollow fibrous scaffold, is excellently suited to investigating cyst physiology.

Deflation of a Rathke cleft cyst triggered rupture of a superior hypophyseal artery aneurysm: a case report.

A 57-year-old woman was diagnosed as a Rathke cleft cyst (RCC). Endoscopic transsphenoidal surgery (TSS) was performed uneventfully. She developed subarachnoid haemorrhage on postoperative day 3. The vessels adhered the cyst had been pulled into the pituitary fossa, causing an aneurysm.

Pathological examination of cerebral amyloid angiopathy in patients who underwent removal of lobar hemorrhages.

Cerebral amyloid angiopathy (CAA) is a degenerative disorder characterized by amyloid-ß (Aß) deposition in the brain microvessels. CAA is also known to contribute not only to cortical microbleeds but also lobar hemorrhages. This retrospective study examined CAA pathologically in patients who underwent direct surgeries for lobar hemorrhage. Thirty-three patients with lobar hemorrhage underwent open surgery with biopsy from 2007 to 2016 in our hospital. Cortical tissues over hematomas obtained surgically were pathologically examined using hematoxylin, eosin stain, and anti-Aß antibody to diagnose CAA. We also investigated the advanced degree of CAA and clinical features of each patient with lobar hemorrhage. In the 33 patients, 4 yielded specimens that were insufficient to evaluate CAA pathologically. Twenty-four of the remaining 29 patients (82.8%) were pathologically diagnosed with CAA. The majority of CAA-positive patients had moderate or severe CAA based on a grading scale to estimate the advanced degree of CAA. About half of the CAA-positive patients had hypertension, and four took anticoagulant or antiplatelet agents. In five patients who were not pathologically diagnosed with CAA, one had severe liver function disorder, three had uncontrollable hypertension, and one had no obvious risk factor. Our pathological findings suggest that severe CAA with vasculopathic change markedly contributes to lobar hemorrhage. The coexistence of severe CAA and risk factors such as hypertension, anticoagulants or antiplatelets may readily induce lobar hemorrhage.

Evaluation of the Use of Calcium Phosphate Cement for Aesthetic Neurosurgical Cranial Reconstruction.

OBJECTIVE: The objective of this article is to evaluate whether newly developed calcium phosphate cement (CPC), mounted around the titanium plates, is useful for aesthetic cranial reconstruction by using 2 methods. METHODS: The morphologic changes of CPC were observed in videos of 6 patients who had undergone cranial reconstruction with CPC during the first surgery and required second surgery. The facial aesthetic outcomes of 74 consecutive patients, more than 12 months after frontotemporal or bifrontal craniotomy and reconstruction with or without CPC, were evaluated. RESULTS: Observations of CPC changes were available 1 day, 2 weeks, 2 months, 5 months, 10 months, and 26 months after the first surgeries. CPC, applied superficially on the cranial surface, had not set sufficiently. CPCs, mounted thickly around the titanium plates and forming hydroxyapatite, were residual during the latter period. Comparison between the aesthetic reconstruction group (with CPC) and the simple reconstruction group (without CPC) showed that the objective bump detected by the investigator, and the subjective bump noticed by the patients themselves, were significantly more frequent in the simple reconstruction group. Comparison between the patients without an objective bump and the patients with an objective bump during the follow-up period showed that the proportion of patients after aesthetic cranial reconstruction with CPC was significantly higher in patients without an objective bump. Patients' characteristics, craniotomy procedure, use of a vascularized pericranial flap, and craniotomy-associated complications did not influence the objective bump significantly. CONCLUSIONS: Use of CPC was expected to bring better aesthetic outcomes after neurosurgical cranial reconstructions.

Predictive Factors for the Occurrence of Visual and Ischemic Complications After Open Surgery for Paraclinoid Aneurysms of the Internal Carotid Artery.

OBJECTIVE: To investigate the predictive factors for visual and ischemic complications after open surgery for paraclinoid aneurysms of the internal carotid artery (ICA). MATERIALS AND METHODS: Thirty-eight consecutive patients with unruptured paraclinoid aneurysms of ICA operated on between 2009 and 2013 were included in this study. The male:female ratio was 6:32 and the ages ranged from 33 to 81 (mean: 60 ± 2). Twenty cases were asymptomatic and 18 had ophthalmological symptoms. The sizes of the aneurysms ranged from 2 to 35 mm (mean: 10.6 ± 9 mm). Twenty-three patients were treated by clipping and 15 by trapping with bypass (high-flow bypass in 11, and low-flow in 4). Twenty-four patients underwent removal of the anterior clinoid process. Among them, 8 underwent en bloc anterior clinoidectomy with a high-speed drill, and 16 had piecemeal excision with a microrongeur or ultrasonic bone curette. Intraoperative monitoring was performed using motor-evoked potentials (MEP) and visual-evoked potentials (VEP) in 27 and 15 cases, respectively. RESULTS: Complete obliteration of the aneurysm was achieved in 37 cases (97.4 %). The patency rate of bypass was 100 %. Postoperative worsening of visual acuity, including one case of blindness, was observed in six cases (11 %). Worsening of visual field defects occurred in 14 cases (38 %), but 10 of them were transient. Transient oculomotor nerve palsy occurred in six cases (15 %). Postoperative stroke was detected by diffusion-weighted imaging (DWI) in five cases (13 %), four of which were symptomatic. Statistical analysis showed that piecemeal anterior clinoidectomy was significantly safer than en bloc removal in preserving visual function. Trapping with high-flow bypass had a significantly greater risk of postoperative stroke than direct clipping. CONCLUSIONS: Intraoperative VEP monitoring might be useful for preventing postoperative worsening of visual function. Two-stage treatment with bypass and endovascular trapping might be safer than single-stage trapping alone.

Clinicopathologic features of small cell glioblastomas.

Small cell glioblastoma (SCGBM) is a variant of glioblastomas characterized by a predominant population of small and monomorphic glial cells. The aim of the present study was to investigate clinical, neuroimaging, pathologic, and genetic features of SCGBM. The clinicopathologic and genetic features were evaluated in 14 patients with SCGBM. All cases were divided into multifocal and solitary type by MRI, and extent of microvascular proliferation, intratumoral necrosis, and perivascular lymphocytic accumulation were investigated. IDH1 mutations by immunohistochemistry (IDH1 R132H) and 1p 19q codeletion by fluorescence in situ hybridization were detected. Patients ranged from 23 to 92 years of age (median: 71 years), with three females and eleven males. The overall survival time of the patients ranged from 7 to 23 months (mean: 11 months). Nine patients (64%) were the multifocal type. Pathologic study revealed that the microvascular proliferation, necrosis, and lymphocytic infiltration were limited in SCGBM. Immunohistochemically, tumor cells were negative for IDH1 R132H in all patients. FISH analysis demonstrated that no SCGBM had 1p/19q codeletion in informative patients. Our investigation suggested that an elderly onset and multifocal lesions were characteristics of SCGBM associated with degradation of the immune response, infiltrative feature of tumor cells, and an unfavorable prognosis.

[Determining the tumor-cell density required for macroscopic observation of 5-ALA-induced fluorescence of protoporphyrin IX in cultured glioma cells and clinical cases].

We investigated the association between the cell density and intensity of 5-aminolevulinic acid-induced fluorescence of protoporphyrin IX in 3-dimensionally cultured C6 glioma cells. The ratio between 636-nm red fluorescence excited by a 405-nm laser and 513-nm green autofluorescence of the tissue was measured as the fluorescence intensity. A ratio exceeding 0.68 was macroscopically judged as fluorescence-positive by observers, and the cell density at this fluorescence intensity was 1×107< cells/mL. In clinical surgical fields, the fluorescence ratio was about 0.65 in cases judged as fluorescence-positive, similarly to that in cultured cells. Neurosurgeons and pathologists should recognize that tumor cells are present in fluorescence-negative regions. It is necessary to develop a device which measures fluorescence more simply than macroscopic observation for cutting-edge brain surgery.

The adaptation of a neuroendoscopic sheath to a Leksell stereotactic frame to make it suitable for approaching small ventricles: a technical note.

Efficient endoscopic surgery in cases involving small ventricles is difficult but possible. The authors describe a neuroendoscopic sheath that has been improved to make it suitable for use with a Leksell stereotactic frame, which enabled us to accurately insert a stereotactic needle into the small ventricle and provided an adequate surgical corridor. A biopsy was performed on a 6-year-old girl who had a primary central nervous system lymphoma in the pineal body without hydrocephalus.

Coincident choroid plexus carcinoma and adrenocortical tumor in an infant.

We report a case of a 20-month-old girl with a large choroid plexus carcinoma arising in the left lateral ventricle and an adrenocortical tumor. Following brain tumor resection, the patient was treated with radiation and chemotherapy. The adrenocortical tumor was found with the manifestation of precocious puberty. TP53 gene mutation (exons 4-10) was not detected in either specimen. The patient had leptomeningeal dissemination and died 26 months later.

Lymphomatosis cerebri: clinical characteristics, neuroimaging, and pathological findings.

Lymphomatosis cerebri is a rare variant of primary central nervous system lymphoma. We present a case involving a 56-year-old immunocompetent woman who complained of rapid deterioration of her higher brain function over a 4-month period. Magnetic resonance imaging showed extensive white-matter lesions. During brain biopsy, a diffusely infiltrating lymphoma with distinctive immunohistochemical features was detected. Awareness of this unique presentation and early tissue diagnosis provide the best hope for instituting appropriate treatments.

Usefulness of intraoperative computed tomography in surgery for low-grade gliomas: a comparative study between two series without and with intraoperative computed tomography.

We have routinely used an intraoperative CT (i-CT) system in over 800 neurosurgical procedures since 1997. To investigate the utility of i-CT in low-grade glioma (LGG) surgery, we investigated whether i-CT improved the extent of tumor resection and prognosis in 46 patients with histologically confirmed LGG consisting of 27 patients with World Health Organization grade II astrocytoma, 12 with oligodendroglioma, and 7 with oligoastrocytoma. The patients were divided into two groups, 23 who underwent tumor resection without i-CT (non i-CT group) and 23 who underwent surgery using i-CT (i-CT group). We investigated the extent of tumor resection, pre- and postoperative Karnofsky performance status scores, and overall survival in each group. The extent of tumor resection was biopsy 26.1%, partial resection 60.9%, subtotal resection 13.0%, and gross total resection 0% in the non i-CT group, and 4.4%, 21.7%, 34.8%, and 39.1%, respectively, in the i-CT group. The i-CT group showed significantly longer overall survival than the non i-CT group among patients with astrocytoma (p < 0.05) and oligodendroglioma or oligoastrocytoma (p < 0.005). Prolonged survival was related to the extent of resection. There were no significant differences between pre- and postoperative Karnofsky performance status scores between the groups. Surgical resection using i-CT may improve the outcomes of patients with LGG. Additional resection or emergency treatment can be quickly performed as the surgical results are confirmed intraoperatively or immediately after the operation using i-CT.

Nestin expression in astrocytic tumors delineates tumor infiltration.

Nestin is an intermediate filament protein expressed in undifferentiated cells during central nervous system development, and glioma is known to be a highly infiltrative tumor. We determined whether nestin was expressed in astrocytic tumors and could identify infiltrating tumor cells. We screened 65 archival, paraffin-embedded adult astrocytic tumors using immunohistochemical staining and computerized overlaid photographs. Normal biopsied brains and metastatic brain tumors were also examined. The intensity of nestin expression corresponded to the tumor grade. All 33 glioblastoma cases showed positive and extensive staining, which was less positive in diffuse astrocytoma. Overlaid images showed that nestin immunostaining delineated tumor invasion into adjacent gray and white matter. Nestin is a useful marker for examining the infiltration of malignant cells into surrounding tissue.

Proliferation of vascular smooth muscle cells in glioblastoma multiforme.

OBJECT: Glioblastomas multiforme (GBM) contain a higher number of alpha-smooth muscle actin (SMA)-positive vascular smooth muscle cells (VSMCs) than those in the respective normal neuronal tissue. The role of VSMCs during angiogenesis is unclear, and it is also uncertain whether and to what extent angiogenic factors might be involved in GBM VSMCs. In GBMs, the contribution of VSMCs in angiogenesis accompanying endothelial proliferation and the correlation of VSMC proliferation with vascular endothelial growth factor (VEGF) expression were examined using an immunohistochemical method. METHODS: The examined material, including surrounding brain tissue, came from 12 cases (6 men and 6 women) with classic GBM. Microvessel densities (MVDs) of CD31-immunoreactive vessels (CD31-MVD) and SMA-immunoreactive vessels (SMA-MVD) were obtained in areas selected from white matter, boundary, tumor (concentrated area of tumor cells), and perinecrosis. Subsequently, the SMA-MVD/CD31-MVD (SMA/CD31) rate, representing the percentage of vessels with VSMCs in the region, was calculated in each area. The VEGF immunoreactivity of tumor cells was examined, and cases were divided into 2 groups: < 30% VEGF expression of tumor cells (low VEGF group) and > 30% VEGF expression of tumor cells (high VEGF group). RESULTS: The SMA/CD31 rate of the boundary was significantly lower than that of the tumor (p < 0.005) and perinecrosis (p < 0.001). The SMA/CD31 rate of the high VEGF group was significantly higher than that of the low VEGF group (p < 0.05) in the tumor. CONCLUSIONS: In GBMs, the transformation and proliferation of VSMCs may accompany neovascularization and may also be induced by angiogenic factors.

[Hypoxic ischemic encephalopathy with atypical findings on CT and MR imaging: two case reports].

We report 2 cases of hypoxic ischemic encephalopathy with atypical findings on computed tomographic (CT) and magnetic resonance (MR) imaging for the acute to subacute stage. Case 1: A 78-year-old man with larynx cancer suffered cardiac arrest after suffocation. Cardiopulmonary resuscitation, was performed; the patient then went into a deep coma and also developed severemyoclonus. CT scans on day 0 and day 3 after the arrest revealed no abnormalities MR imaging was performed on day 13 to evaluate cerebral anoxia; however, no abnormal findings were obtained. Since no abnormalities were detected both on CT and MR imaging, we expected that the prognosis would be good; however, the patients did not recover from coma and remains in a persistent vegetative state. Case 2: A 54-year-old man developed cardiac arrest after anaphylactic shock caused by insect bite. After cardiopulmonary resuscitation, the patient lapsed into a deep coma. CT scans performed on day 1 and 3 after the cardiac arrest revealed slight subarachnoid hemorrhage at the surface of the right cerebral cortex along the falx cerebri. MR images obtained on day 10 demonstrated slight hemorrhage at the surface of right cerebral cortex, but no abnormalities in basal ganglia, thalamus, cortex, and white matter. The transient damage of the blood brain barrier caused by hypoxia and ischemia was thought to induce the slight subarachnoid hemorrhage after cardiopulmonary resuscitation. The patient demonstrated early recovery and was almost completely recovered with slight agnosia. MR imaging to rule out hypoxic-ischemic encephalopathy may not have been timed appropriately in both the case, our radiological findings are usual as compare to the findings presented in other similar reports. The variations in the findings of CT and MR imaging in the case of hypoxic-ischemic encephalopathy should be clarified, and the prognosis and management of this condition should be planned on the basis of not only the neuroradiological images but also the neurological signs and symptoms.

Chromosome 1p and 19q deletions in malignant glioneuronal tumors with oligodendroglioma-like component.

Malignant glioneuronal tumors (MGNT) are suggested to be a new entity of glioma defined morphologically as any malignant glioma showing immunohistoichemical evidence of neuronal differentiation. We encountered seven cases of MGNT with oligodendroglioma-like component and investigated alternations of chromosome 1p and 19q in these tumors. Seven patients ranged from 33 to 62 years of age, four females and three males. Immunohistochemical study of these tumors was performed using neuronal markers (synaptophysin, neurofilament, beta-tubulin, chromogranin A and NeuN), astrocytic marker (GFAP) and Ki-67. We undertook a molecular cytogenetic study of tumor specimens obtained from seven patients using fluorescence in situ hybridization (FISH) with DNA probes mapping to chromosome 1p36, 1q25, 19p13 and 19q13. Histologically, these tumors resembled anaplastic oligodendroglioma. Immunohistochemically, tumor cells were immunoreactive for synaptophysin (7/7), neurofilament (6/7), beta-tubulin (5/7), chromogranin A (4/7), NeuN (2/7) and GFAP (7/7). The Ki-67 labeling index ranged from 4.5% to 20.7%. FISH analysis demonstrated either 1p or 19q deletion in all seven cases (100%) and both 1p and 19q deletions in five cases (71%). The 1p deletion was detected in six of seven cases (86%) and 19q deletion was also detected in six (86%). 1p and 19q deletions were present in MGNT, especially those with oligodendroglial components. We suggest that the oligodendroglial-like feature was associated with not only 1p or 19q loss but also differentiation along neuronal cell lines as a factor of favorable prognosis in glial tumors. It is inappropriate to make a diagnosis of oligodendroglioma based only on morphological resemblance to oligodendroglia.

A report of supratentorial leptomeningeal hemangioblastoma and a literature review.

Hemangioblastomas of the CNS are solid or cystic vascular-rich tumors, most common in the cerebellum, less frequent in the brainstem or spinal cord, and rare in supratentorial locations with meningeal involvement. We document a case in a 58-year-old man who presented with about 2 months history of motor weakness and speech dysfunction. The tumor was a heterogeneously enhanced dural-based tumor with high vascularity and perifocal edema in the left frontal lobe. The tumor was completely removed followed by embolization and preoperative radiotherapy. Histologic examination revealed a hemangioblastoma with features resembling angiomatous meningioma. Immunohistochemistry for epithelial membrane antigen (EMA) and S100 may be helpful to make differential diagnosis. Electron microscopic investigation is essential to differentiate between meningiomas and other leptomeningeal tumors.

CD98 immunoreactivity in multinucleated giant cells of glioblastomas: an immunohistochemical double labeling study.

CD98, which is identical to fusion regulatory protein-1 (FRP-1), has been reported to induce and regulate cell fusion and multinucleated giant cell formation. To investigate the association between CD98 and multinucleated giant cells (MNGCs) in glioblastomas, we investigate the CD98 immunoreactivity of MNGCs and the proliferative potential in CD98 immunoreactive MNGCs in paraffin-embedded sections obtained from patients with glioblastomas. Double immunohistochemical staining for CD98 and Ki67 as a mitotic marker were performed in formalin-fixed and paraffin-embedded specimens obtained from 16 patients with primary glioblastomas including MNGCs. Most CD98 immunoreactive (CD98+) tumor cells were negative for Ki67. CD98+ MNGCs were identified in 15 cases. CD98+ Ki67- MNGCs were identified in 14 cases and ranged in number from one to 48 (6.7 +/- 11.5). CD98- Ki67+ MNGCs were identified in 15 cases and ranged in number from one to 32 (11.1 +/- 9.6). Mitotic index (MI) of CD98+ MNGCs (4.8 +/- 2.7%) was significantly lower than that of CD98- MNGCs (91.1 +/- 24.6%) (P < 0001). These results suggest that multinucleated giant cell formation may be developed by fusion among CD98- producing cells in glioblastomas.