The neurobiological substrates of behavioral manifestations during temporal lobe seizures: A neuroethological and ictal SPECT correlation study

Ictal behavior coupled with SPECT findings during 28 seizures in patients with temporal lobe epilepsy (TLE) with unilateral hippocampal sclerosis (13 left; 15 right) was displayed as flowcharts from right-sided (RTLE) plus left-sided (LTLE) seizures. Ictal SPECT was classified blind to neuroethology. Behaviors were categorized as ipsilateral to the epileptogenic zone (IL), contralateral to the epileptogenic zone (CL), or bilateral. SPECT intensity and region were categorized as IL or CL to the epileptogenic zone. All patients developed automatisms and had hyperperfusion in their temporal lobes. Patients` verbal responses to questions had statistical interactions in RTLE but not in LTLE sum. Most CL dystonic posturing was correlated to IL basal ganglia hyperperfusion. Basal ganglia activation occurred in seizures without dystonic posturing and CL manual automatisms, and lack of IL dystonic posturing and the presence of CL cerebellar hemispheric hyperperfusion were also observed. Coupling of neuroethology and SPECT findings reliably evaluates ictal behavior and functionality of associated brain areas. (C) 2010 Elsevier Inc. All rights reserved.

Characterizing the phenotypic manifestations of MFN2 R104W mutation in Charcot-Marie-Tooth type 2

Mutations of the mitofusin 2 (MFN2) gene have been reported to be the most common cause of the axonal form of Charcot Marie Tooth disease (CMT). The aim of this study was to describe a de novo MFN2 p.R104W mutation and characterize the associated phenotype. We screened the entire coding region of MFN2 gene and characterized its clinical phenotype, nerve conduction studies and sural nerve biopsy. Neuropsychological tests and brain MRI were also performed. A de nova mutation was found in exon 4 (c.310C > T; p.R104W). In addition to a severe and early onset axonal neuropathy, the patient presented learning problems, obesity, glucose intolerance, leukoencephalopathy, brain atrophy and evidence of myelin involvement and mitochondrial structural changes on sural nerve biopsy. These results suggest that MFN2 p.R104W mutation is as a hot-spot for MFN2 gene associated to a large and complex range of phenotypes. (C) 2011 Elsevier B.V. All rights reserved.

Systemic lupus erythematosus: Association with KIR and SLC11A1 polymorphisms, ethnic predisposition and influence in clinical manifestations at onset revealed by ancestry genetic markers in an urban Brazilian population

Systemic lupus erythematosus (SLE) is an autoimmune disorder of the connective tissue with a wide and heterogeneous spectrum of manifestations, with renal and neurological involvement usually related to worse prognosis. SLE more frequently affects females of reproductive age, and a high prevalence and renal manifestation seem to be associated with non-European ethnicity. The present study aims to investigate candidate loci to SLE predisposition and evaluate the influence of ethnic ancestry in the disease risk and clinical phenotypic heterogeneity of lupus at onset. Samples represented by 111 patients and 345 controls, originated from the city of Belem, located in the Northern Region of Brazil, were investigated for polymorphisms in HLA-G, HLA-C, SLC11A1, MTHFR, CASP8 and 15 KIR genes, in addition to 89 Amerindian samples genotyped for SLC11A1. We also investigated 48 insertion/deletion ancestry markers to characterize individual African, European and Amerindian ancestry proportions in the samples. Predisposition to SLE was associated with GTGT deletion at the SLC11A1 3`UTR, presence of KIR2DS2 +/KIR2DS5 +/KIR3DS1 + profile, increased number of stimulatory KIR genes, and European and Amerindian ancestries. The ancestry analysis ruled out ethnic differences between controls and patients as the source of the observed associations. Moreover, the African ancestry was associated with renal manifestations. Lupus (2011) 20, 265-273.

Oral manifestations of human T-cell lymphotropic virus infection in adult patients from Brazil

Objective: Human T-cell lymphotropic virus type 1 (HTLV-1) was the first human retrovirus discovered and its pathogenesis is related to T cells infection. This study aimed to verify the presence of oral manifestations in a Brazilian population of patients who was seropositive for HTLV, and identify risk factors for oral manifestations. Subjects and methods: An assessment was made of 139 patients at the Emilio Ribas Institute of Infectious Diseases. Results: A total of 112 (80.5%) patients were HTLV-1, 26 (18.7%) were HTLV-2+. About 35.2% of patients had myelopathy/tropical spastic paraparesis (HAM/TSP), with 48 of them being HTLV-1+ and one patient was seropositive for HTLV-1 and -2. The most common oral manifestations were: xerostomia (26.8%), candidiasis (20.8%), fissured tongue (17.9%), and loss of tongue papillae (10.0%). A multivariate logistic regression analysis showed that HAM/TSP is an independent risk factor for xerostomia (P = 0.02). The patients who were HAM/TSP+ were three times more likely to develop xerostomia when compared with patients without HAM/TSP (odds ratio = 2.69, 95% confidence interval = 1.17-6.17). Conclusion: Despite the fact that the findings of this study suggest a relationship between xerostomia and HAM/TSP, more studies should be developed to show what the association would be between xerostomia presented by HTLV patients and pathogenesis of the virus.

Gingival Manifestations of Orofacial Granulomatosis

Background: Orofacial granulomatosis is a clinical entity presenting with swelling of the facial and/or oral tissues in association with histologic evidence of noncaseating granulomatous inflammation. Labial swelling is the most common finding. Compromise of the gingival and periodontal tissues may occur but has rarely been described in the literature. Our objective was to characterize granulomatous gingivitis in patients with orofacial granulomatosis. Observations: The study included 29 cases of orofacial granulomatosis seen in our clinic between January 1, 1989, and December 31, 2006. Of these 29, 5 had clinical evidence of gingival tumefaction and underwent gingival biopsy. Histologic examination of all the gingival biopsy specimens showed noncaseating granulomas, edema of the superficial lamina propria, and a chronic inflammatory infiltrate consisting predominantly of lymphocytes and multinucleated giant cells. Treatment options included anti-inflammatory therapy associated with periodontal care. Conclusion: Gingival tumefaction with histologic evidence of granulomatous inflammation may occur in orofacial granulomatosis and might be more common than reported in the literature.

Oral manifestations after immune reconstitution in HIV patients on HAART

In order to verify possible association between immune reconstitution inflammatory syndrome (IRIS) and oral manifestations (OMs), we selected AIDS patients who had low CD4 count before the initiation of highly active antiretroviral therapy (HAART) and who returned three months later for therapy evaluation. The oral lesions observed three months after the initiation of HAART were evaluated and associated with the type of antiretroviral therapy (ART), CD4 count and HIV-RNA load levels (before and three months after HAART initiation). A total of 105 patients matched the selected criteria. Immune reconstitution (IR) was identified in 35.2%. Among these patients, the mean CD4 cell count rose from 105.97 to 330.29 and the mean viral load dropped from 168.005 (log 5.22) to 21.852 (log 4.33). There was no significant difference in age (P=0.78), sex (P=0.41) or previous history of ART (P=0.55) between IR and non-IR patients. In the IR group, the most common OM was. parotid enlargement (57.14%) (P=0.619), whereas in the non-IR group candidiasis (46.15%) was the most common OM. The results of our study suggest that the parotid gland enlargement found in the studied population might be an IRIS event, as it was found in patients with IR three months after the initiation of HAART.

Evaluation of proximal caries in images resulting from different modes of radiographic digitalization

Objective: The aim of this study was to evaluate the performances of observers in diagnosing proximal caries in digital images obtained from digital bitewing radiographs using two scanners and four digital cameras in Joint Photographic Experts Group (JPEG) and tagged image file format (TIFF) files, and comparing them with the original conventional radiographs. Method: In total, 56 extracted teeth were radiographed with Kodak Insight film (Eastman Kodak, Rochester, NY) in a Kaycor Yoshida X-ray device (Kaycor X-707;Yoshida Dental Manufacturing Co., Tokyo, Japan) operating at 70 kV and 7 mA with an exposure time of 0.40 s. The radiographs were obtained and scanned by CanonScan D646U (Canon USA Inc., Newport News, VA) and Genius ColorPage HR7X (KYE Systems Corp. America, Doral, FL) scanners, and by Canon Powershot G2 (Canon USA Inc.), Canon RebelXT (Canon USA Inc.), Nikon Coolpix 8700 (Nikon Inc., Melville, NY), and Nikon D70s (Nikon Inc.) digital cameras in JPEG and TIFF formats. Three observers evaluated the images. The teeth were then observed under the microscope in polarized light for the verification of the presence and depth of the carious lesions. Results: The probability of no diagnosis ranged from 1.34% (Insight film) to 52.83% (CanonScan/JPEG). The sensitivity ranged from 0.24 (Canon RebelXT/JPEG) to 0.53 (Insight film), the specificity ranged from 0.93 (Nikon Coolpix/JPEG, Canon Powershot/TIFF, Canon RebelXT/JPEG and TIFF) to 0.97 (CanonScan/TIFF and JPEG) and the accuracy ranged from 0.82 (Canon RebelXT/JPEG) to 0.91 (CanonScan/JPEG). Conclusion: The carious lesion diagnosis did not change in either of the file formats (JPEG and TIFF) in which the images were saved for any of the equipment used. Only the CanonScan scanner did not have adequate performance in radiography digitalization for caries diagnosis and it is not recommended for this purpose. Dentomaxillofacial Radiology (2011) 40, 338-343. doi: 10.1259/dmfr/67185962

Hydraulic Performances of Minimum Energy Loss Culverts in Australia

Culverts are among the most common hydraulic structures. Modern designs do not differ from ancient structures and are often characterised by significant afflux at design flows. A significant advance was the development of the Minimum Energy Loss (MEL) culverts in the late 1950s. The design technique allows a drastic reduction in upstream flooding associated with lower costs. The development and operational performances of this type of structure is presented. The successful operation of MEL culverts for more than 40 years is documented with first-hand records during and after floods. The experiences demonstrate the design soundness while highlighting the importance of the hydraulic expertise of the design engineers.

The prediction of sharkskin instability observed during film blowing

The characteristics of sharkskin surface instability for linear low density polyethylene are studied as a function of film blowing processing conditions. By means of scanning electron microscopy and surface profilometry, is it found that for the standard industrial die geometry studied, sharkskin only occurs on the inside of the film bubble. Previous work suggests that this instability may be due to critical extensional stress levels at the exit of the die. Isothermal integral viscoelastic simulations of the annular extrusion process are reported, and confirm that the extensional stress at the die exit is large enough to cause local melt rupture. However the extensional stress level at the outer die wall predicts melt rupture of the outside bubble surface also, which contradicts the experimental findings. A significant temperature gradient is expected to exist across the die gap at the exit of the die, due to the external heating of the die and the low conductivity, of the polymer melt. It is shown that a gradient of 20 degreesC is required to cause sharkskin to only appear on the inner bubble surface.

Communism and Carnival: Ralph de Boissieres Crown Jewel and its Australian Context

The Australasian Book Society (ABS) was established in Melbourne in 1952 by a small group ofleft-leaning writers, unionists and literary supporters. It was inspired in part by the success of Frank Hardy's scandalous novel of Labar betrayal, Power Without Glory, which in 1950 had been published independently - and printed secretly - outside the commercial publishing networks. Many of those involved were communists but although the ABS came to operate in accord ,..rith Communist Party cultural politics it was never Simply a Party front nor did it publish only communist writers or communist texts. For the most part it operated under a broader notion of 'progressive' , nationalist or democratic literature.

Ross River virus disease in tropical Queensland: evolution of rheumatic manifestations in an inception cohort followed for six months

Objective: To describe the natural history of rheumatic manifestations of Ross River virus (RRV) disease. Design: Prospective longitudinal clinical review. Setting: North Queensland local government areas of Cairns, Douglas, Mareeba and Atherton during January to May 1998. Participants: General practice patients diagnosed with RRV disease on the basis of symptoms and a positive RRV IgM result. Main outcome measures: Rheumatic symptoms and signs assessed as soon as possible after disease onset and on two subsequent occasions (up to 6.5 months after onset). Results: 57 patients were recruited, 47 of whom were reviewed three times (at means of 1.1, 2.4 and 3.6 months after disease onset). Results are reported for these 47: 46 (98%) complained of joint pain at first review, with the ankles, wrists, fingers, knees and metacarpophalangeal joints (II-IV) most commonly involved. Prevalence of joint pain decreased progressively on second and third reviews, both overall (92% and 68% of patients, respectively), and in the five joints most commonly affected. The prevalence of other common rheumatic symptoms and signs, and use of non-steroidal anti-inflammatory drugs, also progressively declined over the three reviews. Conclusions: Earlier studies may have overestimated the prevalence and duration of symptoms in RRV disease. Progressive resolution over 3-6 months appears usual.