Sporadic periventricular nodular heterotopia: Classification, phenotype and correlation with Filamin A mutations.

OBJECTIVE: The purpose of this study was to better delineate the clinical spectrum of periventricular nodular heterotopia (PNH) in a large patient population after long term follow up. Specifically, this study aimed to relate PNH subtypes to clinical or epileptic outcomes, epileptic discharges and underlying Filamin A (FLNA) mutations by analyzing anatomical features. METHODS: The study included 100 patients with radiologically confirmed nodular heterotopia. Patients' FLNA gene sequences and medical records were analyzed. Two-sided Chi-square test and Fisher's exact t-test were used to assess associations between the distribution of PNHs and specific clinical features. RESULTS: Based on imaging data, patients were subdivided into three groups: (a) classical (bilateral frontal and body, n=41 patients), (b) bilateral asymmetrical or posterior (n=16) and (c) unilateral heterotopia (n=43). Most patients with classical heterotopia were females (P=0.033) and were likely to have arachnoid cysts (P=0.025) and cardiac abnormalities (P=0.041), but were mostly seizure-free. Additionally, hippocampal abnormalities (P=0.022), neurological deficits (P=0.028) and cerebellar abnormalities (P=0.005) were more common in patients with bilateral asymmetrical heterotopia. Patients with unilateral heterotopia were prone to develop refractory epilepsy (P=0.041). FLNA mutations were identified in 8 patients. CONCLUSIONS: Each group's distinctive genetic mutations, epileptic discharge patterns and overall clinical outcomes confirm that the proposed classification system is reliable. These findings could not only be an indicator of a more severe morphological and clinical phenotype, but could also have clinical implications with respect to the epilepsy management and optimization of therapeutic options.

Imaging Features of Neurocysticercosis: Analysis of 57 Consecutive Patients.

AIM: To investigate the imaging features of neurocysticercosis and to provide valuable information about the diagnosis and treatment for clinicians. MATERIAL AND METHODS: The data of 71 consecutive cases of neurocysticercosis, diagnosed by computed tomography (CT) and magnetic resonance imaging (MRI) between January 2009 and January 2014 in our institute were collected. Of all the cases, 57 patients met the recognized diagnostic criteria of neurocysticercosis. In this series, enzyme-linked immunosorbent assay (ELISA) examination and Cysticercosis IgG were positive in 55 cases and 3 cases, respectively. Among them, neurocysticercosis was confirmed surgically in 11 cases. Seven cases had CT examinations, including enhanced CT in one case. Fifty-four cases had MRI examinations, including contrast-enhanced MRI in 46 cases. Therapeutically, 32 patients received albendazole, while 25 patients received praziquantel. As a surgical treatment, 9 patients underwent lesionectomy, and 1 patient underwent lesionectomy plus shunt insertion for hydrocephalus. RESULTS: Parenchymal cysticerci in 53 cases (92.9%), subarachnoidal cysticerci in 39 cases (68.4%), ventricular cysticerci in 13 cases (22.8%) and spinal cysticerci in 1 case were demonstrated on CT and MRI scans. In total, the intracranial cysticerci involving multiple locations simultaneously were found in 39 patients. Thirty-five cases had associated leptomeningitis, 10 cases had hydrocephalus. In 1 case, the cysticerci were located outside of the central nervous system. CONCLUSION: The imaging findings of the cysticerci, including their location, numbers, cystic sizes, capsular thickness, densities and signals of the scolexes, as well as the peripheral edema, have distinct value in making possible timely diagnosis of neurocysticercosis for clinicians.

The Surgical Value of Neurocysticercosis: Analyzing 10 Patients in 5 Years.

AIM: To investigate the indications and clinical value of the surgical therapy of neurocysticercosis (NCC). MATERIAL AND METHODS: We retrospectively reviewed all 10 patients with NCC treated surgically from January 2009 to January 2014 in our institute. The NCC types included parenchymal cysticerci in 6 cases, subarachnoid cysticerci in 3 and ventricular cysticerci in 1. RESULTS: All 10 patients underwent resection of cysticerci through craniotomy. Parenchymal NCC included an isolated lesion in 4 cases and multi-lesions in 2 cases, who respectively had coexistence of metastasis and cysticerci and a positive history of tuberculosis. In all 3 patients with arachnoid NCC, two cases underwent lesion resection and the other one underwent cyst resection and lamina terminalis fenestration. In one case with intraventricular NCC, a ventriculoperitoneal (VP) shunt was placed as the initial treatment. Due to failure of the shunt, the secondary surgery of cyst excision via open craniotomy and VP shunt placement were performed simultaneously. CONCLUSION: For parenchymal cysticercosis, the surgical aim is to remove the lesion, treat the medically intractable epilepsy, and establish the diagnosis and subsequent treatment. As for extraparenchymal NCC, surgical therapy is a valuable treatment as an alternative due to its fatal complications. For subarachnoid cysticercosis, because of its higher failure rate in simply VP shunt, the open craniotomy could directly remove the cyst and efficaciously relieve hydrocephalus. For the patient with intraventricular NCC associated with hydrocephalus, the effect of a simple VP shunt seems unsatisfactory, and craniotomy for cyst resection is necessary.

Malformations of cortical development and epilepsy: A cohort of 150 patients in western China.

PURPOSE: Malformations of cortical development (MCDs) are abnormalities of the cerebral cortex that arise from abnormal formation of the cortical plate, and have become increasingly identified as an important etiology for refractory epilepsy. Little is known about the spectrum, distribution and clinical features of MCDs, especially in resource-limited regions. This study investigates the distribution of MCDs and compares the clinical features and long-term prognosis between the two forms of MCDs: Simple and Multiple. METHOD: One hundred and fifty epilepsy patients (138 adults, 12 pediatric patients) with radiologically diagnosed MCDs were identified at a tertiary epilepsy center in western China. Patients were divided into three subtypes according to the Barkovich classification. They were further divided into either Simple or Multiple MCD forms based on whether they had a single type of MCDs or other co-existing developmental brain abnormalities. RESULTS: The most common type of MCD is focal cortical dysplasia. We found perinatal insults more common in group III patients. Multiple MCD was identified in 36 of 150 patients, and was associated with higher rates of delayed milestones (p=0.005), cognitive impairment (p=0.023) and neurological deficits (p=0.002) compared to Simple MCD. Extra-temporal epilepsy was more commonly seen among individuals with Multiple MCD (p=0.017). Participants with Multiple MCD were younger at time of seizure onset (p=0.003) and at assessment (p=0.002), had a lower seizure-free rate (p=0.033) and had worse outcomes overall. Patients with heterotopias were more commonly associated with other abnormalities. CONCLUSION: MCDs are a critical cause of epilepsy and pose a big challenge for resource-limited countries. Imaging techniques are crucial in diagnosing and classifying cortical deformities. Multiple malformations lead to more severe clinical features and worse prognosis. Identifying and classifying MCDs can help physicians to better estimate patient prognosis and seek the best, individualized therapeutic options.

Morphological study of the pterygoid canal with high-resolution CT.

The purpose of our study was to accurately visualize and measure the normal anatomy and size of the pterygoid canal with thin-section (0.5 mm), high-resolution computed tomography (HRCT) as well as multiplanar reconstruction (MPR) and curved planar reconstruction (CPR) technologies to obtain credible and comprehensive information for clinical management. Both axial HRCT and MPR images of the pterygopalatine fossa were obtained in 167 normal adult subjects, who exhibited variable positions of pterygoid canal relative to the sphenoid sinus floor and cavity. The morphology and size of the pterygoid canal was observed and measured, respectively. All pterygoid canals (100%, 334/334) were well delineated on HRCT images. Statistical analyses showed no significant difference between the mean length of the left (12.6 ± 2.3 mm) and right pterygoid canals (12.5 ± 2.9 mm) (P = 0.405). The mean diameter of anterior, median and posterior opening of the left and right pterygoid canals exhibited no significant differences (all P > 0.05); the bilateral median opening possessed the smallest diameter (P < 0.001). Submillimeter, thin-section HRCT scan and appropriate postprocessing reconstruction technologies could clearly visualize the morphologic features of the pterygoid canal and adjacent structures, which may be helpful for making diagnostic and therapeutic decisions.

Detecting atheromatous plaques in the aortic arch or supra-aortic arteries for more accurate stroke subtype classification.

INTRODUCTION: To investigate the correlations of atheromatous plaques in the aortic arch or supra-aortic arteries with intracranial arterial stenosis and carotid plaques in stroke patients, and to determine whether taking these plaques into account will reduce the proportion of patients in the undetermined etiology group. METHODS: We prospectively enrolled 308 ischemic stroke patients, whose clinical characteristics and A-S-C-O classifications were compared with analyses of intracranial arteries, carotid arteries, aortic arch, and supra-aortic arteries. RESULTS: 125(40.6%) patients had plaques in the aortic arch or supra-aortic arteries, of which 106 (84.8%) had complex plaques. No correlations were observed between these plaques and carotid plaques ( p = 0.283) or intracranial arterial stenosis ( p = 0.097). After detecting the mobile thrombi in the aortic arch and supra-aortic arteries, the proportion of patients in the atherothrombosis group was increased from 33.8% to 55.5% ( p = 0.00), whereas the proportion of patients in stroke of undetermined etiology group was decreased from 19.2% to 11.0% ( p = 0.00). DISCUSSION: Examining only the carotid and intracranial arteries may not provide adequate information about large arteries in stroke patients. Therefore, it would be better to include a search for relevant plaques in the aortic arch or supra-aortic arteries in modern stroke workup, for it may lead to more accurate stroke subtype classification and guide secondary prevention.

[The clinical application of hydrogen magnetic resonance spectroscopy combining with diffusion weight imaging in brain gliomas grading].

The present study was aimed to investigate the function of diffusion weight imaging (DWI) combining with magnetic resonance spectroscopy (MRS) in the grading of brain gliomas. 12 cases low grade and 17 cases high grade of brain gliomas patients were examined with DWI and MRS, with all tumors confirmed by pathology in advance. The apparent diffusion coefficient (ADC) values, their corresponding metabolite ratios of Cho/Cr, Cho/NAA and tumor cellularities of tumor solid enhanced parts were measured. The ratios of Cho/Cr and Cho/NAA and their corresponding ADC values had significant differences between their high and low grade gliomas values, respectively. The ADC values demonstrated a negative correlation with Cho/Cr, Cho/NAA, and a significant negative correlated with Cho/Cr. And the ADC values demonstrated strong negative correlations with tumor cellularities. DWI combining with MRS could provide more valuable information in evaluating gliomas grading.

Features of cranio-maxillofacial trauma in the massive Sichuan earthquake: analysis of 221 cases with multi-detector row CT.

OBJECTIVE: In a massive earthquake, cranio-maxillofacial trauma was common. The present study was to determine the features of cranio-maxillofacial trauma sustained in the massive Sichuan earthquake by multi-detector row computed tomography (MDCT). METHODS: The study included 221 consecutive patients (123 males and 98 females; age range, 1-83 years; median age, 35 years) with cranio-maxillofacial trauma in the Sichuan earthquake, who underwent cranio-maxillofacial MDCT scans. The image data were retrospectively reviewed focusing on the injuries of the cranio-maxillofacial soft tissue, facial bones and cranium. RESULTS: All patients had soft tissue injuries frequently with foreign bodies. Ninety-seven (43.9%) patients had fractures (1.5 involved sites per patient, range from 1 to 8) including single cranial fractures in 36 (37.1%) cases, single maxillofacial fractures were seen in 48 (49.5%) and cranio-maxillofacial fractures in 13 (13.4%). Single bone fracture was more common than multiple bone fractures (p<0.05). Nasal, ethmoid bones and the orbits were the most commonly involved sites of the craniofacial region. Thirty-eight (17.2%) patients had intracranial injuries, the commonest being subarachnoid haemorrhage and the commonest sites were the temporal and frontal regions. Coexisting intracranial injuries were more common in patients with cranial fractures than in patients with maxillofacial fractures (p<0.05). CONCLUSION: Our results indicate that the cranio-maxillofacial trauma arising from the massive Sichuan earthquake had some characteristic features, and a significant number of individuals had the potential for combined cranial and maxillofacial injuries, successful management of which required a multidisciplinary approach.

Carotid and cerebrovascular disease in symptomatic patients with type 2 diabetes: assessment of prevalence and plaque morphology by dual-source computed tomography angiography.

BACKGROUND: Plaque morphology directly correlates with risk of embolism and the recently developed dual-source computed tomography angiography (DSCTA) may help to detect plaques more precisely. The aim of our study was to evaluate the prevalence and morphology of carotid and cerebrovascular atherosclerotic plaques in patients with symptomatic type 2 diabetes mellitus (DM) by DSCTA. METHODS: From July 2009 to August 2010, DSCTA was prospectively performed in 125 consecutive patients with symptomatic type 2 DM. We retrospectively analyzed plaque type, distribution, and extensive and obstructive natures were determined for each segment for all patients. RESULTS: Atherosclerotic plaques were detected in 114 (91.2%) patients. Relatively more noncalcified (45%) and calcified (39%) plaques and less mixed (16%) plaques were observed (p < 0.001). Noncalcified plaques were found mainly in the intracranial arteries (81.8%), mixed plaques in the intracranial arteries (25.2%) and intracranial internal carotid artery (ICA) (56.1%). Calcified plaques were found mainly in the intracranial ICA (65.9%) and extracranial arteries (28.2%) (for all, p < 0.001). Extension of plaques from the 1(st) to 5(th) segments was observed in 67 (58.8%) patients and from the 6(th) to 10(th) segments in 35 (30.7%) patients. The most common site of all detected plaques was the cavernous segment. Regarding stenosis, there were significantly more nonobstructive than obstructive stenosis (91% vs. 9%, p < 0.001). CONCLUSION: DSCTA detected a high prevalence of plaques in patients with symptomatic type 2 DM. A relatively high proportion of plaques were noncalcified, as well as with nonobstructive stenosis. The distribution of plaques was extensive, with the cavernous portion of ICA being the most common site.

A new method to classify pathologic grades of astrocytomas based on magnetic resonance imaging appearances.

BACKGROUND: Astrocytoma is the most common neuroepithelial neoplasm, and its grading greatly affects treatment and prognosis. OBJECTIVE: According to relevant factors of astrocytoma, this study developed a support vector machine (SVM) model to predict the astrocytoma grades and compared the SVM prediction with the clinician's diagnostic performance. PATIENTS AND METHODS: Patients were recruited from a cohort of astrocytoma patients in our hospital between January 2008 and April 2009. Among all astrocytoma patients, nine had grade I, 25 had grade II, 12 had grade III, and 60 had grade IV astrocytoma. An SVM model was constructed using radial basis kernel. The SVM model was trained with nine magnetic resonance (MR) features and one clinical parameter by fivefold cross-validation and differentiated astrocytomas of grades I-IV at two levels, respectively. The clinician also predicted the grade of astrocytoma. According to the two prediction methods above, the areas under receiving operating characteristics (ROC) curves to discriminate low- and high-grade groups, accuracies of high-grade grouping, overall accuracy, and overall kappa values were compared. RESULTS: For SVM, the overall accuracy was 0.821 and the overall kappa value was 0.679; for clinicians, the overall accuracy was 0.651 and the overall kappa value was 0.466. The diagnostic performance of SVM is significantly better than clinician performance, with the exception of the low-grade group. CONCLUSIONS: The SVM model can provide useful information to help clinicians improve diagnostic performance when predicting astrocytoma grade based on MR images.

[Application of radial basis function neural network for grading of gliomas].

This retrospective investigation was directed to the applicability of Radial Basis Function Neural Network (RBF-NN) and Discriminant Analysis in the grading of gliomas. The data on 116 patients with primary glioma in our hospital from February 2008 to April 2009 were collected. Kruskal-Wallis H test was used to draw in the variable age ranks and then to take them out from the range of different grades of gliomas. The results of RBF-NN model, discriminant analysis, and the combined model of RBF-NN and discriminant analysis were evaluated and compared respectively with and without age. In this study, different classifications of gliomas showed statistically significant differences in age: and the accuracy of the models with age was better than the ones without age. The predictive accuracy and Kappa value of RBF-NN model and the combined model were also better than those exhibited by Bayes discriminant analysis. Consequently, as a prediction model, or to help other models, RBF-NN is of significance to predicting the grade of gliomas.

Masticator space: CT and MRI of secondary tumor spread.

OBJECTIVE: In this article, we review the CT and MRI features of secondary involvement of the masticator space in a variety of tumors. We focus on showing various patterns of tumor spread to the masticator space. CONCLUSION: Secondary masticator space involvement is not rare. Familiarity with the anatomy of the masticator space and its anatomic relationship with adjacent structures is important for imaging interpretation.

[CT and MRI features of diseases involving masticator spaces].

OBJECTIVE: This is a study on the CT and MRI features of diseases involving the masticator spaces; the purpose is to improve the diagnostic accuracy. METHODS: Fifty-seven cases involving the masticator spaces were collected, among which, 11 cases were originated from the masticator space, another 46 cases were secondary invasion from the adjacent structures, including 18 cases from the buccal mucosa, 10 from the nasopharynx, 5 from the gingiva, 3 from the parotid gland, 6 from the maxillary sinus, 1 from the intracranial location, 2 from the palate and 1 from the orbit. The CT and MRI features of the masticator space involvement were analysed. RESULTS: Masticator space involvement is visualized on CT and MRI as effacement of the fat plane, swelling of the masticatory muscles, and erosion of the mandibular ramus. Disease from surrounding structures can invade the masticator space via the pterygopalatine fossa, the buccal space immediately anterior to the ramus, the foramen ovale, or by way of direct invasion. CONCLUSION: Masticator space involvement is not uncommon in the maxillofacial diseases, which must be noticed in the imaging diagnosis.

[CT diagnosis of palatal malignant tumors].

To investigate CT features of palatal malignant tumors and to evaluate, CT diagnostic value. CT manifestations of 32 cases of palatal malignant tumors were analyzed retrospectively. All cases were confirmed by pathology. There were 27 males and 5 females between 23 and 80 years of age. Axial contrast enhancement CT scan was performed in all cases, among them, nonenhancement CT scan was also performed in 8 cases and coronal CT scan was performed in 2 cases. The main signs of palatal malignant tumors were as follows: (1) Masses in palate in 26 cases, thickened palatal soft tissue in 6 cases and destruction of palate bone in 8 cases. The lesions were located in soft palate in 22 cases, in hard palate in 4 cases, and in the junction region between soft and hard palate in 3 cases; (2) Other adjacent structures and organs were involved in 27 cases; (3) Lymph nodes of neck metastasis were demonstrated in 17 cases. CT is an excellent technique, which can show the gross pathologic features and the invasion pathway of palatine malignant tumors, and it can provide some important information for the relevant clinical treatment and prognosis.

[Hippocampal formations, amygdala and anterior temporal lobes: normative volumetric measurements from MR imaging in normal adults of China].

OBJECTIVE: To define the normal range of volumetric values of hippocampal formations (HPF), amygdala (AM) and anterior temporal lobes (ATL). METHODS: One hundred and two Chinese normal adults underwent MRI scan on oblique coronal section. Volumes of the anterior temporal lobes, hippocampal formations and amygdala were obtained on T1WI. The mean values and the range of 95% normal values were obtained by statistical data processing and analysis. RESULTS: The mean values of standardized volumes of anterior temporal lobes in the subjects aged 20 yr.-, 40 yr.- and > or = 60 yr. were: 59.07 +/- 2.02 cm3, 55.55 +/- 2.23 cm3, 52.88 +/- 2.07 cm3 (Right); 57.25 +/- 2.15 cm3, 53.23 +/- 1.72 cm3, 51.63 +/- 2.18 cm3 (Left); and of amygdala in those aged 20 yr.-, 40 yr.- and > or = 60 yr. were: 1.54 +/- 0.09 cm3, 1.38 +/- 0.08 cm3, 1.29 +/- 0.09 cm3 (R); 1.37 +/- 0.09 cm3, 1.29 +/- 0.08 cm3, 1.23 +/- 0.09 cm3(L). The 95% normal values of HPF in the subjects aged 20 yr.- ranged from 2.52 to 3.11 cm3(R), and from 2.40 to 2.98 cm3(L); in those aged > or = 60 yr. ranged from 2.33 to 2.65 cm3 (R) and from 1.98 to 2.64 cm3(L). There was no significant difference in the volumes of hippocampal formations between two groups under 60 yr.. And a decrease of the volumes of hippocampal formations could been seen in the > or = 60 yr. group. No significant difference in the volumes of hippocampal formations, amygdala and anterior temporal lobes was seen in the sex groups and handedness groups. CONCLUSION: The data on standardized volumes of HPF, AM, ATL in this study can serve as a foundation on which to provide normal index and useful guidelines for reference in early diagnosis and treatment of Alzheimer's disease and temporal lobe epilepsy.

[CT and MR imaging of pachygyria and agyria].

The CT and MRI manifestations of pachygyria and agyria are presented with a review of the pathological features of the disorders and an evaluation of the diagnostic value of both imaging modalities. 15 cases were analysed retrospectively; of them, 12 were examined with CT and 3 with MRI. The analyses confirm 7 cases with agyria, 5 with pachygyria of bilateral cerebral hemisphere and 3 with limited pachygyria of unilateral hemisphere. On CT and MRI of the whole lesions, the cortex is thickened and the white matter is reduced in proportion. The surface of the brain remains flat and smooth or shows only a few broad gyri and shallow sulci. The border between gray and white matter is smooth. In 12 patients with agyria and pachygyria of the whole brain, the sylvian fissures are shallow, and insulae are exposed. The middle cerebral arteries course superficially along sylvian grooves close to the inner table of the skull. The cerebral contour is "hourglass" or "figure of eight". Associated brain anomalies include the heterotopia of gray matter in 5 cases, the schizencephaly (type I) in 2 cases and the agenesis of the corpus callosum in one case. In conclusion, the pathological changes of pachygyria and agyria are characteristics. CT and MRI are excellent modalities to evaluate these pathological features.