Background: Recent genome-wide and locus-specific association studies identified RNF213 as an important Moyamoya disease (MMD) susceptibility gene. But the results of these studies are limited by the few subjects, different methodologies and ethnicities. Aims: To investigate the association between p.R4810K (rs 112735431, ss179362673; G > A) and p.R4859K (c.14576 G > A) polymorphisms of the RNF213 gene and MMD susceptibility. Settings and Design: We conducted a meta-analysis to evaluate the association. Materials and Methods: Two investigators independently searched the PubMed, Medline, and Embase databases for studies published before October 2012. For included studies, we performed meta-analyses using Cochrane RevMan software. Statistical Analysis: Summary odds ratios (ORs) and 95% confidence intervals (CIs) for RNF213 p.R4810K and p.R4859K polymorphisms; MMD were calculated in a fixed-effects model and a random effects model whenever appropriate. Results: Five eligible studies were reviewed and analyzed, which included two studies for p.R4810K polymorphisms (421 cases and 1214 controls) and three studies for p.R4859K polymorphisms (398 cases and 765 controls). Overall, the pooled results indicated that both p.R4810K polymorphisms and p.R4859K polymorphisms were associated with MMD risk (OR 92.03, 95% CI 54.06-156.65, P < 0.00001 and OR 157.53, 95% CI 85.37-290.7, P < 0.00001, respectively). Stratified analyses by ethnicity revealed the population attributable risks in the Japanese and Korean populations were larger than that in the Chinese population (P =0.0006). Conclusions: This meta-analysis demonstrated that there are strong associations between p.R4859K and p.R4810K polymorphisms of the RNF213 gene and MMD. The discoveries of its association with MMD may help in early diagnosis and prevention of this disease. Further study is still necessary to clarify the biochemical function and pathological role of RNF213 in MMD.

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Background: Intracranial arachnoid cyst (IAC) is a benign cystic lesion filled with cerebrospinal fluid (CSF). Different surgical treatments were evaluated to determine the most effective technique among several. Materials and Methods: A consecutive series of 68 adult patients (43 males, mean age 30.3 years, range 18-42 years) with IAC were surgically treated between January 2004 and January 2011. The cysts were supratentorial in location in 53 and infratentorial in 15 patients. Symptoms at presentation, location of the IAC, surgical treatment modalities, and postoperative complications were evaluated. Results: Of the 51 patients with headache, 44 (86.27%) patients had complete relief of the headache, five (9.80%) patients had significant improvement, and two (3.92%) had no worthwhile change. Three of the four patients with hydrocephalus and gait disturbances had relief of the symptoms and one patient had significant improvement. Of the five patients with cognitive decline and weakness, three (60.00%) patients showed improvement, and two (40.00%) patients had no significant change. Five (62.50%) of the eight patients with epilepsy had seizure remission, two (25.00%) patients had non-disabling seizures, and one had no change. Follow-up computed tomography (CT) scans showed variable change in the mass effect of IAC in 68 patients; cystic size was significantly reduced in 51 patients, no significant change in two patients of supratentorial arachnoid cysts. Cystic size was reduced in seven patients, but no significant change was observed in eight patients of infratentorial cysts. Three patients with enlarged head circumference had no further increase in the head circumference. Conclusion: Adult patients with IAC symptoms should be treated efficiently. Surgical treatment is associated with significant improvement in the symptoms and signs.

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Background: Central nervous system (CNS) involvement in the form of meningitis or meningoencephalitis is common in scrub typhus. As specific laboratory methods remain inadequate or inaccessible in developing countries, prompt diagnosis is often difficult. Aim: To identify the clinical and laboratory parameters that may help in differentiating scrub typhus meningitis from bacterial meningitis. Setting and Design: This is a cross-sectional analysis of adult patients admitted with scrub typhus and bacterial meningitis to a tertiary care teaching institute in South India. Materials and Methods: A comparison of clinical and laboratory features of 25 patients admitted with meningitis to a university teaching hospital during a 15-month period was made. These patients had meningitis diagnosed based on abnormal cerebrospinal fluid (CSF) analysis with either positive IgM scrub typhus ELISA serology (n =16) or with CSF culture isolating bacteria known to cause bacterial meningitis (n =9). The clinical and laboratory features of the patients with scrub typhus meningitis and bacterial meningitis were compared. Results: The mean age was similar in the scrub typhus and bacterial meningitis groups (44.0 ± 18.5 years vs. 46.3 ± 23.0 years). Features at admission predictive of a diagnosis of scrub typhus meningitis were duration of fever at presentation >5 days (8.4 ± 3.5 days vs. 3.3 ± 4.2 days, P < 0.001), CSF white cell count of a lesser magnitude (83.2 ± 83.0 cells/cumm vs. 690.2 + 753.8 cells/cumm, P < 0.001), CSF lymphocyte proportion >50% (83.9 ± 12.5% vs. 24.8 ± 17.5% P < 0.001), and alanine aminotransferase (ALT) elevation more than 60 IU (112.5 ± 80.6 IU vs. 35 ± 21.4 IU, P =0.02). Conclusion: This study suggests that clinical features, including the duration of fever and laboratory parameters such as CSF pleocytosis, CSF lymphocyte proportion >50%, and ALT values are helpful in differentiating scrub typhus from bacterial meningitis.

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Background: Meningiomas account for 35.5% of central nervous system (CNS) tumors, of which 21-37.8% are atypical or anaplastic/malignant. High-grade meningiomas have higher rates of recurrence and worse outcome than grade I/II meningiomas. Thus, it is of importance to assess the tumor biology before treatment initiation. Materials and Methods: This study reviewed 1737 patients with histologically confirmed meningioma at a single institution. Meningiomas were classified according to World Health Organization (WHO) 2007 grading and the location of the tumor was confirmed from the operation records and preoperative imaging. Univariate and multivariate logistic regression were used to analyze the potential risk factors for high-grade pathology. Results: Young men and pediatric patients were less likely to have meningioma, but they had high-grade meningioma. Tumors originated from non-skull base and lateral intracranial are more likely to be grade II/III meningioma. Conclusions: Lateral and non-skull base location, male sex, and the younger patients increase the risk for grade II and III pathology. These factors should be considered while deciding treatment choice, surgical resection, and prognosis as well.

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Background: Catechol-O-methyltransferase (COMT) gene has been reported to be associated with the risk of Parkinson's disease (PD). Aims: To evaluate the associations of PD risk with COMT polymorphisms. Materials and Methods: A retrieval of studies that investigated associations between COMT polymorphisms and PD was carried out. Studies were included if they met the eligibility criteria. Statistical Analysis: Data were analyzed using Stata version 12.0. Results: A total of 18 studies including 2926 PD cases and 3151 controls were included. The results showed no significant association with all genotypes and alleles in Caucasians. However in Asians, the homozygote A/A (Odds ratio [OR] =1.51, 95% confidence interval [CI] =1.16-1.98, P =0.002) tends to increase risk of PD, however, the homozygote G/A (OR =0.85, 95% CI =0.74-0.98, P =0.03) may be a slightly protective effect against PD. Conclusions: This study showed that the COMT polymorphisms may be associated with PD in Asians rather than Caucasians. But further studies are needed to confirm our results.

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Background: Corticobasal syndrome (CBS) is characterized by progressive asymmetric rigidity and localized cortical disorders namely apraxia, cortical, sensory loss, focal dystonia, and refractory to levodopa treatment. Aim: To study the cerebral glucose-metabolism, cognitive, and magnetic resonance imaging (MRI) features in patients with CBS. Materials and Methods: Seventeen consecutive patients with CBS who fulfilled the criteria proposed by Lang et al., were studied. A detailed neurological including higher cortical functions and Unified Parkinson's Disease Rating Scale (UPDRS) on and off motor scores and neuropsychological assessment were done. All patients except one had undergone MRI brain and 18-Flouro 5-Deoxy Glucose Positron Emission Tomography (FDG PET CT) brain scan for assessing cortical atrophy and cerebral glucose metabolism, respectively. Results: Most of the 17 patients had Parkinsonian features with focal cortical signs of dystonia, apraxia, and cortical sensory loss and the hypokinesia was predominantly on left side. Neuropsychological assessment had shown impairment of frontal executive function, visuospatial function, and language. MRI brain showed asymmetrical cortical atrophy in left temparoparietal areas and basal ganglia. The MRI findings correlated with FDG PET brain scan findings, asymmetric focal hypometabolism in basal ganglia and inferior parietal and temporal, frontal lobes. Conclusion: 18 FDG PET brain is more sensitive than MRI brain in the early diagnosis of CBS and also correlates well with neuropsychological assessment.

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Aim: To quantify iron deposition in Alzheimer's disease (AD), amnestic mild cognitive impairment (aMCI), and control individuals using susceptibility weighted imaging (SWI). Materials and Methods: Sixty participants (22 aMCI, 20 AD, 18 normal controls) underwent conventional magnetic resonance imaging (MRI) and SWI using axial/oblique coronal sequences. Phase images were used to calculate bilateral iron deposition in 18 regions of interest (ROI). The radian angle value was calculated and compared between the three participant groups. Results: The difference in radian angle value was significant between the aMCI and control groups in the left (L)-hippocampus, L-head of the caudate nucleus, R-lenticular nucleus, L-lenticular nucleus (P =0.02239, <0. 001, 0.03571, 0.00943, respectively). The difference in radian angle value was significant between the AD and aMCI groups in the R-cerebellar hemisphere, L-cerebellar hemisphere, R-hippocampus, L-hippocampus, R-red nucleus, R-thalamus, L-thalamus, and splenium of corpus callosum (P =0.02754, 0.01839, 0.00934, 0.04316, 0.02472, 0.00152, <0.001, 0.01448, respectively). Pearson correlation coefficients of the Mini-Mental State Examination score were all significant for the bilateral cerebellar hemisphere, hippocampus, red nucleus, lenticular nucleus, thalamus, R-head of the caudate nucleus, and splenium of corpus callosum. Conclusion: Iron deposition in the hippocampus, head of the caudate nucleuslenticular nucleus, and thalamus are significantly different between individuals with aMCI, AD, and controls. The thalamus is a particularly sensitive area. Using SWI to quantify the iron deposition is a useful tool in detecting aMCI and AD.

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Background: Previous studies have shown that the phosphodiesterase 4D (PDE4D) gene is a susceptibility gene for ischemic stroke (IS) primarily in elder populations. However, few studies have reported the role of the PDE4D gene polymorphisms in a young cohort. Aims: To investigate the association between the PDE4D gene polymorphisms and young-onset IS in Chinese population. Materials and Methods: A total of 186 young patients (18-45 years) with IS and 232 matched control subjects were recruited. Two SNPs (rs918592 and rs2910829) in PDE4D gene were genotyped by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. Odds ratio and 95% confidence intervals (95% CI) were calculated to test the association between the genetic factors and IS. Results: The rs918592A/A genotype frequency and A allele frequency, rs2910829 CT/TT genotype frequency and T allele frequency of young IS group were significantly higher than those of the control group ( P < 0.05). Besides, the frequency of Hap (A-T) was remarkably higher in the young patients than that in the controls (OR =4.047, 95% CI: 3.521-4.652). Hap (A-C) and Hap (G-C) were associated with decreased risk of IS (OR =0.640, 95% CI: 0.452-0.906; OR =0.675, 95% CI: 0.466-0.978, respectively). Conclusions: Our findings suggest that the rs918592 and rs2910829 polymorphisms and haplotypes of PDE4D gene are significantly associated with IS in Chinese young population.

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Background: A cerebral aneurysm with incomplete treatment may re-grow and cause new bleeding. This meta-analysis calculates the sensitivity and specificity of computed tomographic angiography (CTA) in the detection of the residual or recurrent cerebral aneurysms after clipping, in comparison with digital subtraction angiography (DSA). Materials and Methods: Between October 1997 and October 2012, relevant data were systematically and prospectively collected without language restrictions from the PUBMED and EMBASE databases for analysis. A total of 10 eligible studies compared CTA with DSA in the detection of the residual or recurrent cerebral aneurysms after clipping by two independent observers. The sensitivity, specificity, positive likelihood ratio (+LR), and negative likelihood ratio (-LR) were calculated on a per-aneurysm basis, and the area under the sROC curve (AUC), heterogeneity, and publication bias also were evaluated. Results: A total of 487 aneurysms were included for meta-analysis. The pooled sensitivity, specificity, +LR, -LR, DOR, and AUC of CTA for detecting the residual or recurrent aneurysms after clipping were 71%, 94%, 9.39, 0.32, 28.32, and 0.8558, respectively. The between-study heterogeneity of DOR and the presence of publication bias were not statistically significant. Conclusions: As a noninvasive and convenient screening method, CTA has a high diagnostic value for the detection of the residual or recurrent aneurysms after clipping. In the future, it may be used as a routine diagnostic tool for evaluation of aneurysms after clipping.

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Background: Intracranial lipomas are uncommon tumors, which produce susceptibility artifacts on susceptibility weighted images. The cause for the susceptibility artifact on SWI images remains speculative. Our purpose was identifying the possible causes of susceptibility artifacts in lipoma. Materials and Methods: We retrospectively reviewed 15 cases harboring 16 lipomas of head region. All the lipomas are evaluated on SWI images for the presence of blooming and types of blooming artifacts. Computed tomography (CT) images were evaluated for presence of calcification. Results: All three pericallosal tubulonodular lipomas showed peripheral rim-like susceptibility artifacts. All the curvilinear lipomas (four cases) showed complete blooming. Five out of eight nodular lipomas showed peripheral susceptibility artifacts, whereas, one showed complete blooming. Two nodular lipomas showed peripheral and central susceptibility artifacts. Scalp and craniovertebral lipomas (four in number) showed peripheral susceptibility artifacts. Specks of calcification were identified in two out of seven cases on CT scan. Conclusions: Contribution of the macroscopic calcification to susceptibility blooming appears to be insignificant. Microscopic mineralization and chemical shift artifact appears to be a major cause of susceptibility blooming.

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This report presents a 72-year-old woman with posterior cranial fossa neurovascular compression syndrome that included paroxysmal supraventricular tachycardia. The patient underwent surgical exploration of the posterior cranial fossa, and a gross left vertebral artery was identified as the offending vessel. The neurovascular conflicts were associated with the cranial nerves V, VII, VIII, IX, and X. The patient experienced significant postoperative relief. Probably this is the first report of a single microvascular decompression, having cured such a high number of syndromes, including paroxysmal supraventricular tachycardia.

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A 65-year-old woman, a case of cranio-vertebral junction anomaly, presented with a sudden onset of quadriparesis, dysphagia, and breathing difficulty and required emergency surgery. She underwent image-guided endoscopic transnasal odontoidectomy followed by occipito-cervical decompression and stabilization. She recovered well, with immediate improvement of respiratory function. Burning paresthesias disappeared and the upper limbs function normalized.

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Background: The covered stent is one of the most promising tools for the treatment of intracranial fusiform aneurysms. We developed an in vivo model of fusiform aneurysms and evaluated the effectiveness of double telescoping Willis covered stents for their treatment. Materials and Methods: An external jugular vein graft was anastomosed with the common carotid artery (CCA) to construct the fusiform aneurysm model. After at least 4 weeks, two Willis covered stents were implanted in a telescopic fashion. Angiography follow-up was performed at 2 weeks and 1 and 3 months to examine the grafts. The animals were sacrificed at 1 or 3 months of the follow-up period, and the stents were examined histologically. Results: A total of eight fusiform aneurysms in four canines were created and 16 covered stents were implanted successfully. No technical or device-related difficulties occurred. The angiographic follow-up results showed that six fusiform aneurysms were completely occluded, and a minimal endoleak occurred in two fusiform aneurysms. Histological examination revealed endothelial progress and all aneurysm sacs were filled with thrombi. Conclusion: Vein graft anastomosis with CCA to construct a model of fusiform aneurysm may reproduce the clinical conditions. This study demonstrated that the implantation of two Willis covered stents in a telescoping fashion is an effective way to treat an experimental model of fusiform aneurysm.

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Subgaleo-peritoneal (SP) shunting for pseudomeningoceles (PMCs) is an effective and safer alternative as compared to the lumboperitoneal (LP) shunt. SP shunting was done in six patients (14-60 years) with persistent or recurrent PMCs using the cranial (ventricular part) and the distal parts of a Chhabra shunt connected by a rigid connector without any intervening chamber or valve. Two patients had undergone a prior LP shunt that had failed. One patient was unsuitable for a LP shunt placement. The PMC subsided completely in all the patients following the SP shunt. In one patient, the shunt got displaced and required repositioning. None of the patients developed symptoms of over-drainage or any other complication. All patients were asymptomatic at a mean follow-up of 15 months. These results suggest that SP shunting is a safe, simple, and effective alternative to the traditional LP shunt in the management of persistent or recurrent cranial PMCs.

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Nonconvulsive status epilepticus (NCSE) represents a diagnostic dilemma, and so far several different definitions and diagnostic criteria have been proposed in the literature order to address and try to solve the diagnostic problems related to this condition. NCSE is practically diagnosed as an enduring epileptic condition with reduced or altered consciousness, but without major convulsive movements together with epileptiform discharges on EEG. The diagnosis of NCSE is therefore electro-clinical, and diagnostic criteria for this epileptic condition should take into account both clinical and EEG features. Based on a comprehensive search of the literature, this brief review critically appraises the electro-clinical diagnostic criteria for NCSE present in the Literature, with particular emphasis on EEG features encountered in NCSE.

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