Expression of cellular retinoic acid-binding protein-I (CRABP-I) in the cerebrospinal fluid of adult onset moyamoya disease and its association with clinical presentation and postoperative haemodynamic change.

Objective: The elevation of cellular retinoic acid-binding protein-I (CRABP-I) has been suggested as a candidate in the pathogenesis of paediatric moyamoya disease (MMD). However, few studies have addressed CRABP-I in adult onset MMD. The aim of this study was to examine the expression of CRABP-I in the cerebrospinal fluid (CSF) of adult onset MMD, and to evaluate its association with clinical presentation and postoperative haemodynamic change.

Analysis of a bleeding mechanism in patients with the sylvian arachnoid cyst using a finite element model.

Objective: The sylvian arachnoid cyst (AC) is a common benign disease; however, it sometimes leads to subdural or intracystic hemorrhage without major trauma. The reason of easy bleeding of the AC is not fully understood. The purpose of this study was to investigate the bleeding mechanism of the sylvian AC in biomechanical aspect and suggest treatment guidelines.

TERT promoter mutations are highly recurrent in SHH subgroup medulloblastoma.

Telomerase reverse transcriptase (TERT) promoter mutations were recently shown to drive telomerase activity in various cancer types, including medulloblastoma. However, the clinical and biological implications of TERT mutations in medulloblastoma have not been described. Hence, we sought to describe these mutations and their impact in a subgroup-specific manner.

Smooth-muscle progenitor cells isolated from patients with moyamoya disease: novel experimental cell model.

Object: Moyamoya disease (MMD) is a cerebrovascular occlusive disease affecting bilateral internal carotid termini. Smooth-muscle cells are one of the major cell types involved in this disease process. The characteristics of circulating smooth-muscle progenitor cells (SPCs) in MMD are poorly understood.

Identification of brain tumour initiating cells using the stem cell marker aldehyde dehydrogenase.

Aldehyde dehydrogenase (ALDH) has been identified in stem cells from both normal and cancerous tissues. This study aimed to evaluate the potential of ALDH as a universal brain tumour initiating cell (BTIC) marker applicable to primary brain tumours and their biological role in maintaining stem cell status. Cells from various primary brain tumours (24paediatric and 6 adult brain tumours) were stained with Aldefluor and sorted by flow cytometry.

Erratum to: Retrospective analysis of treatment outcome of pediatric ependymomas in Korea: analysis of Korean multi-institutional data.

The name of the 14th author was incorrect in the original publication. It is correct in this erratum (Young-Shin Ra).

Experimental models of spinal open neural tube defect and Chiari type II malformation.

Introduction: Experimental animal models are essential for investigation of the pathoembryogenesis, pathophysiology, and management strategy of spinal open neural tube defect (ONTD) and its associated anomalies including Chiari type II malformation. Genetic, chemical/nutrient, and surgical models have been widely used for a variety of purposes. The aim of this article is to review the representative animal models of spinal ONTD and associated Chiari type II malformation with respect to their advantages and disadvantages.

Electroencephalography in pediatric moyamoya disease: reappraisal of clinical value.

Purpose: The clinical value of electroencephalography (EEG) in pediatric moyamoya disease has been underestimated, though the characteristic patterns are well known. We undertook this study to evaluate the clinical value of EEG as a diagnostic and postoperative follow-up modality in pediatric moyamoya disease.

Methods: We retrospectively reviewed the pre and postoperative EEG with effective hyperventilation in 127 pediatric moyamoya patients and compared their patterns with hemodynamic images.

Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute.

Background: Choroid plexus tumor is a rare brain tumor with variable clinical features according to the histological grade. We reviewed the treatment outcome of 23 children, focusing on the biological behavior of the atypical choroid plexus papilloma (ACPP) and the current therapeutic strategy in choroid plexus carcinoma (CPC).

Methods: The demographics, clinical features, surgical treatments, adjuvant therapies, and survival were reviewed.

Subgroup-specific prognostic implications of TP53 mutation in medulloblastoma.

Purpose: Reports detailing the prognostic impact of TP53 mutations in medulloblastoma offer conflicting conclusions. We resolve this issue through the inclusion of molecular subgroup profiles.

Patients And Methods: We determined subgroup affiliation, TP53 mutation status, and clinical outcome in a discovery cohort of 397 medulloblastomas.

Latency of intracranial germ cell tumors and diagnosis delay.

Background: Intracranial germ cell tumors (GCTs) frequently take an insidious clinical course before diagnosis. To date, clinical latency has been discussed in the context of germinoma in the suprasellar area and basal ganglia.

Objective: In this study, we classified the clinical latency of intracranial GCTs into three categories and described their characteristics in order to understand the full spectrum of the phenomenon.

Primary alveolar soft part sarcoma arising from the cerebellopontine angle.

Introduction: Alveolar soft part sarcoma (ASPS), a rare soft tissue malignant neoplasm, frequently metastasizes to the brain. However, primary intracranial ASPS is extremely rare. We present a case of primary intracranial ASPS arising from the cerebellopontine angle (CPA) without demonstrable systemic lesions.

Pathoembryogenesis of terminal myelocystocele: terminal balloon in secondary neurulation of the chick embryo.

Purpose: Terminal myelocystocele (TMC) is thought to be caused by a misstep during secondary neurulation. However, due to the paucity of data on secondary neurulation and the rarity of TMC, proofs of this pathogenetic mechanism are unavailable. Based on a previous observation that TMC resembles a step of secondary neurulation in chick, a closer look was taken at secondary neurulation of chick embryos focusing on the cerebrospinal fluid-filled distal neural tube (terminal balloon).

ID3 contributes to cerebrospinal fluid seeding and poor prognosis in medulloblastoma.

Background: The inhibitor of differentiation (ID) genes have been implicated as promoters of tumor progression and metastasis in many human cancers. The current study investigated the expression and functional roles of ID genes in seeding and prognosis of medulloblastoma.

Methods: ID gene expression was screened in human medulloblastoma tissues.

Secondary neurulation of human embryos: morphological changes and the expression of neuronal antigens.

Purpose: The morphological changes and expression patterns of neuronal antigens of human embryos, obtained from the therapeutic termination of pregnancy or from surgical procedures, were analyzed in order to characterize the secondary neurulation.

Methods: A total of 21 human embryos from Carnegie stages 12 to 23 and two fetuses in early stages were studied. The markers used for immunohistochemical study were neural cell adhesion molecule (N-CAM), neuronal nuclear antigen (NeuN), neurofilament-associated protein (3A10), synaptophysin, and glial fibrillary acidic protein (GFAP).

Postoperative epidural hematoma covering the galeal flap in pediatric patients with moyamoya disease: clinical manifestation, risk factors, and outcomes.

Object: Postoperative epidural hematoma (EDH), a blood collection between the inserted galeal flap and the overlying skull flap (epigaleal flap hematoma), is a frustrating complication of the surgical treatment of moyamoya disease (MMD) in pediatric patients. The symptoms of postoperative EDH are often similar to those of postoperative cerebral ischemia, and may cause confusion during clinical decision making. The authors designed this study to evaluate the incidence, clinical presentation, risk factors, and treatment outcomes of postoperative EDH in pediatric patients with MMD.

Genetic grouping of medulloblastomas by representative markers in pathologic diagnosis.

A recent analysis of the genetic features of medulloblastoma (MB) suggested classification into distinct subgroups according to gene expression profiles, including the Wingless signaling pathway-activated group (WNT group), the Sonic Hedgehog signaling pathway-activated group (SHH group), group 3, and group 4. To classify MB according to genetic features in practice, we analyzed 74 MBs using representative markers of each group. Based on immunohistochemistries (IHC), cytogenetic alterations, and a CTNNB1 mutation study, the patients were divided into the following three groups: cases showing nuclear β-catenin and/or CTNNB1 mutation and/or monosomy 6 were included in the WNT group (14/74, 18.

Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery.

Purpose: Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease, and progressive involvement of the posterior cerebral artery (PCA) has been reported. However, majority of MMD articles are presenting classic anterior circulation related issues. This study investigates the preoperative factors related to the long-term outcome of posterior circulation in MMD.

Imaging of integrin α(V)β(3) expression using (68)Ga-RGD positron emission tomography in pediatric cerebral infarct.

Enhanced expression of integrin αvβ3 is commonly used as a biomarker for angiogenesis, which is one of the key pathophysiologic processes in cerebral infarct. Integrin αvβ3 can be imaged with arginine-glycine-aspartic acid (RGD) peptide agents. In this study, characteristics of positron emission tomography (PET) using a 68Ga-labeled RGD were investigated in pediatric cerebral infarct.

Retrospective analysis of treatment outcome of pediatric ependymomas in Korea: analysis of Korean multi-institutional data.

We analyzed the treatment outcomes of intracranial ependymomas in Korean children aged <18 years. Data for 96 patients were collected from five hospitals. Survival rates were calculated using the Kaplan-Meier method.

Tumors in the cerebellopontine angle in children: warning of a high probability of malignancy.

Cerebellopontine angle (CPA) tumors are uncommon in children, and the pathological spectrum is different from that of adults. In this study, we reviewed the pathological diagnosis of pediatric patients with a CPA tumor to determine the pattern in this age group. In a cohort of 267 patients with posterior fossa tumor, tumor locations were determined with preoperative magnetic resonance imaging (MRI).

Combination therapy of cilengitide with belotecan against experimental glioblastoma.

The prognosis of patients diagnosed with glioblastoma remains dismal in spite of the current concomitant chemoradiotherapy with temozolomide. In particular, the resistance to temozolomide appears to be the greatest obstacle to the treatment of glioblastoma. In the present study, we evaluated in vitro and in vivo the antitumor effects of combination therapy of cilengitide with belotecan, a camptothecin derivate, to treat experimental glioblastoma.

Analysis of the BRAF(V600E) Mutation in Central Nervous System Tumors.

BRAF(V600E) mutations are involved in the development of melanoma, colon cancer, and papillary thyroid carcinoma. These mutations are also found in primary brain tumors at low to moderate frequencies. In this study, we investigated a series of brain tumors to determine the prevalence and associated clinicopathologic features of BRAF(V600E) mutations.