Fragile abnormal collaterals in moyamoya disease, known as "moyamoya vessels," have rarely been defined. While flow-sensitive black-blood magnetic resonance angiography (FSBB-MRA) is a promising technique for visualizing perforating arteries, as of this writing no other reports exist regarding its application to moyamoya disease. Six adults with moyamoya disease underwent FSBB-MRA. It depicted abnormal collaterals as extended lenticulostriate, thalamic perforating, or choroidal arteries, which were all connected to the medullary or insular artery in the periventricular area and supplied the cortex. This preliminary case series illustrates the potential for FSBB-MRA to reveal abnormal moyamoya vessels, which could be reasonably defined as periventricular collaterals.
Objective High-normal, the intermediate category between normal fasting glucose (NFG) and impaired fasting glucose (IFG), was introduced in the criteria of the disordered glucose metabolism in 2008. The aim of this study was to investigate the risk for future incidence of type 2 diabetes of the subjects with high-normal and to examine how other metabolic variables could be useful for their risk stratification. Methods A historical cohort study was conducted from 2001 to 2008, inclusive, in 4,165 non-diabetic employees at public schools (2,229 men and 1,936 women; age 45.8±5.9 years, range 25-55 years). They were classified at baseline as NFG with fasting plasma glucose (FPG)<100 mg/dL, high-normal with FPG 100-109 mg/dL, and IFG with FPG 110-125 mg/dL. The incidence of type 2 diabetes (defined either by FPG≥126 mg/dL or by receiving treatments) was measured. Results The cumulative incidence during a mean follow-up of 5.1 years were 16/3,364 (0.5%), 40/613 (6.5%), and 53/188 (28.2%) in subjects with NFG, high-normal, and IFG, respectively. Multivariate-adjusted odds ratios for the incidence were still significant both in high-normal and IFG compared to NFG. Body mass index (BMI) and alanine aminotransaminase (ALT) were associated with the incidence of type 2 diabetes independently of FPG categories (p<0.05). Conclusion The future incidence of type 2 diabetes in subjects with high-normal was significantly higher than in those with NFG in this population. BMI and ALT can improve risk stratification in high-normal subjects.
BACKGROUND Recurrent cervical internal carotid artery vasospasm syndrome (RCICVS) causes cerebral infarction, ocular symptoms, and occasionally chest pain accompanied by coronary artery vasospasm. The etiology and optimal treatment remain unclear. OBSERVATIONS The authors report a patient with drug-resistant RCICVS who underwent carotid artery stenting (CAS). Magnetic resonance angiography revealed recurrent vasospasm in the cervical segment of the internal carotid artery (ICA). Vessel wall imaging during an ischemic attack revealed vascular wall thickening of the ICA, similar to that in reversible cerebral vasoconstriction syndrome. The superior cervical ganglion was identified at the anteromedial side of the stenosis site. Coronary artery stenosis was also detected. After CAS, the symptoms of cerebral ischemia were prevented for 2 years, but bilateral ocular and chest symptoms did occur. LESSONS Vessel wall imaging findings suggest that RCICVS is a sympathetic nervous system-related disease. CAS could be an effective treatment for drug-resistant RCICVS to prevent cerebral ischemic events.
Abstract Molecular diagnosis in brain tumors has been widely spread after the publication of WHO 2016 classification. But it become a major problem that there are some tumors not to be classified on its criteria, especially in pediatric neuroepithelial tumors. To clarify the characteristics of gliomas in pediatric and adolescent and young adult age (AYA), we picked up 131 neuroepithelial tumors under 30-year-old at Kyoto University and analyze their molecular profiles. Hot spot mutations in IDH1/2, H3F3A, HIST1H3B, TERT promoter, and BRAF were analyzed by Sanger sequencing, and 1p/19q codeletion was examined by FISH or MLPA. With the pathohistological diagnosis and genetic information, all tumors were classified based on WHO 2016 classification. The terms “not otherwise specified” (NOS) and “not elsewhere classified” (NEC) were used based on cIMPACT-NOW. There were 25 glioblastomas and 34 pilocytic astrocytomas, which accounted for a larger percentage than in adult tumors. IDH-wild type gliomas accounted for 55% in diffuse astrocytomas and 69% in anaplastic astrocytomas. The percentages of gliomas with NEC were 50% of oligodendrogliomas and 20% in anaplastic oligodendrogliomas, respectively. Most pilocytic astrocytomas were under 20-year-old (27 patients) and located in infratentorial area (21 patients). Based on WHO 2016 classification, not a few neuroepithelial tumors in pediatric and AYA ages could be classified clearly. These tumors had more different genetic abnormalities than those in adult. Therefore, it may be important to evaluate these tumors with comprehensive genetic analysis.
Introduction: Intimal flap-like protrusion of the carotid artery is referred to as a carotid web (also known as carotid diaphragm and atypical fibromuscular dysplasia) that can cause recurrent ischemic stroke. We retrospectively studied the prevalence, demographics, clinical presentation, and imaging features of all patients in a series with defined carotid webs, and describe the treatment and histopathology of two that were symptomatic. Methods: This retrospective study analyzed data from a series of 505 patients who underwent head and neck computed tomography angiography (CTA) at Kyoto University Hospital between April 2011 and Oct 2016. Reports have indicated that CTA is reliable for detecting carotid webs, which are defined on images of oblique sagittal sections as a thin intraluminal filling defect along the posterior wall of the carotid bulb. Ultrasonic echo and MRI findings were also matched when available. Carotid webs were finally defined by interpretation of CTA images by a radiologist and an experienced neurosurgeon. Results: The prevalence of carotid webs in this series was 1.8% (9 of 505 patients), the mean age was 56 (range, 42 - 67) years, and six patients were women. Two (22.2%) of the nine patients with carotid webs had recurrent stroke and both were treated radically by carotid endarterectomy. The histopathological findings suggested fibromuscular dysplasia accompanied by early atherosclerotic changes. Conclusions: The prevalence of carotid webs was low and they tended to occur more frequently among women in the present series. An optimal management strategy has not yet been devised. However, recurrent ischemic stroke might be caused by turbulent blood flow at the point of recess that leads to the formation of thrombus and emboli. We recommend that such patients undergo radical treatment.
Medulloblastoma is the most common of the embryonal tumor arising in the cerebellum and the molecular mechanism of its oncogenesis has been revealed by recent studies. The constitutive expression of high mobility group AT-hook 2 (HMGA2) is associated with a highly malignant phenotype and reduced survival in several malignancies. However, little is known about the clinical and biological significance of HMGA2 in medulloblastoma. In this study, we explore the expression, prognostic and therapeutic role of HMGA2 in medulloblastoma. HMGA1 and HMGA2 expressions were examined using immunohistochemistry in 20 patients with medulloblastoma. To examine the function of HMGA2 expression, knock-down HMGA2 expression was applied in Daoy, D283 and D341 cells. Overexpressions of HMGA1 and HMGA2 were frequently identified in medulloblastoma tissues. In addition, high expression of HMGA2 correlated with high proliferation marker Ki-67 labeling index. High expression of HMGA2 was significantly associated with progression free survival and overall survival of patients with medulloblastoma (p=0.021 and p=0.016, respectively). Functional experiments confirmed that knock-down of HMGA2 resulted in inhibited cell proliferation, migration/invasion and enhanced apoptosis in vitro. In conclusion, HMGA2 overexpression is frequent in medulloblastoma, and its expression is related to proliferation marker and poor prognosis. These findings suggest that HMGA2 could serve as a potential target for future therapeutic strategies in medulloblastoma.
