Σφακιανάκης Αλέξανδρος
ΩτοΡινοΛαρυγγολόγος
Αναπαύσεως 5 Άγιος Νικόλαος
Κρήτη 72100
00302841026182
00306932607174
alsfakia@gmail.com

Αρχειοθήκη ιστολογίου

! # Ola via Alexandros G.Sfakianakis on Inoreader

Η λίστα ιστολογίων μου

Δευτέρα 21 Νοεμβρίου 2016

Childcare and School Management Issues in Food Allergy

Abstract

Purpose of Review

The goal of this review is to characterize food allergy management and anaphylaxis in schools and assess current policies and level of preparedness of schools to recognize and treat anaphylaxis.

Recent Findings

An increasing number of school-aged children have food allergies, and studies show that a significant number of school children with no known history of allergies will experience their first anaphylactic reaction at school. Stock-unassigned epinephrine auto-injectors are recommended in schools but not mandatory in most states, and therefore, epinephrine is not always available. Non-nursing staff members are sometimes administering epinephrine due to limitations in nurse staffing.

Summary

Wide variations in staff training and lack of stock epinephrine leave many schools inadequately prepared to manage anaphylactic reactions. Emphasis should be placed not only on ensuring all schools have stock epinephrine but also on training a wider range of school staff members to minimize treatment delay with epinephrine and improve clinical outcomes.



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Tropical littoral ambient noise probability density function model based on sea surface temperature

Ambient noise variability in tropical shallow water presents a critical challenge for sonar designers and operators due to site-specific sea surface fluctuations. Sea surface temperature (SST) is a direct measure of energy balance defining the local climate of the region and hence ambient noise characteristics. In this work, an ambient noise probability density function (pdf) model for a spectral band of 3–10 kHz has been designed based on the statistical distribution of SST and validated using real field data. This will enable early ambient noise prediction compared to existing wind speed based models to facilitate structured mitigation strategies for improving sonar performance.



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Addressing the challenges of oral health in the 21 st century through research

Hezekiah A Mosadomi

Saudi Journal of Oral Sciences 2014 1(2):55-56



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The nature of collagen in oral submucous fibrosis: A systematic review of the literature

VV Kamath

Saudi Journal of Oral Sciences 2014 1(2):57-64

Background: The nature of collagen in the oral potentially malignant disorder of oral submucous fibrosis (OSF) has always been a subject of interest to researchers studying the condition. The type, constitution, and morphology of the collagen has been studied to understand the pathogenesis of the disorder. Aim: The present review attempts to collate literature to assess the changes reported on the nature of collagen in OSF. Materials and Methods: A systematic analysis of literature based on the PRISMA protocol was carried out on studies that analyzed the nature of collagen in OSF. A total of 30 articles were found acceptable for analysis. The reported literature was categorized into histological, immuno-histochemistry (IHC), electron microscopic (EM)/scanning electron microscopic (SEM), biochemical, culture, and genetic studies. Results: Light microscopic studies revealed increased collagen with compaction and condensation as the disease advanced. Polarising microscopy revealed presence of greenish yellow (type I) thick fibers replacing the yellowish orange (type III) thin fibers in the submucosa. Fibrosis originated in the submucosa and subsequently involved the lamina propria as the disease advanced. EM/SEM investigations revealed frayed ends, degeneration of collagen cores. Increased expression of collagen antibodies and total collagen content were observed in IHC and biochemical studies, respectively. Cultured fibroblasts from OSF tend to produce more collagen in comparison to normal fibroblasts indicating a phenotypic change. Increased expression and upregulation of collagen genes were consistent in OSF cases. Conclusion: The collagen deposition in OSF is biochemically normal but quantitatively more. Fibroblast phenotypic change, upregulation of collagen genes, and selective remodeling due to decreased degradation seem to contribute to the increased fibrosis in the disorder.

http://ift.tt/238VSNk

The "True Triangle" in cleft lip repair: A novel technique

Abdullah I Al Atel

Saudi Journal of Oral Sciences 2014 1(2):114-116

The aim of this short communication is to describe the True Triangle (TT) technique for the repair of unilateral cleft lip (CL). A total of 309 patients were operated using the TT technique. The final cleft lip repair results showed positive outcomes. Conclusion: This technique may overcome some of the drawbacks of the previously described surgical techniques used for CL repair.

http://ift.tt/238UquC

Physical and mechanical properties of pressure-molded and injection-molded denture base acrylics in different conditions

Yousef A Shibat Al Hamd, Veerendra B Dhuru

Saudi Journal of Oral Sciences 2014 1(2):65-70

Background and Aim: The aim of the study was to compare the physical (processing shrinkage) and mechanical properties (bending deflection, flexure strength, and flexure modulus) of pressure-molded (Lucitone 199 and ProBase Hot) and injection-molded (SR-Ivocap) denture base materials in different conditions. Materials and Methods: Two denture base materials for pressure molding, Lucitone 199 and ProBase Hot, and one for injection molding (SR-Ivocap) were tested. Polymerization shrinkage (PS) was determined by measuring the linear distances between the reference points on the wax patterns (65 mm × 55 mm × 6 mm) and the corresponding cured acrylic plates and calculating the difference. Rectangular specimens (50 mm × 10 mm × 2.5 mm) were machined from these acrylic plates and used to measure bending deflection (BD) at various load levels, flexure strength (FS), and modulus of elasticity using a three-point bend test on an Instron Universal Testing Machine. A minimum of seven specimens were tested for each variable category. For each material, the percentage of specimens that failed within the load range of 9-10 kg was noted. Statistical analysis involved calculation of mean and standard deviations followed by group comparison of properties of different materials by using analysis of variance (ANOVA) and Tukey's multiple range tests. Level of significance was set at P < 0.05. Result: There was no significant difference between the PS values. ProBase Hot exhibited significantly less BD and greater FS values than the other two materials (P < 0.05). Within the load range of 9-10 kg., 5% of the lucitone specimens, 25% of the SR-Ivocap specimens, and all of the ProBase Hot specimens fractured. Conclusion: The three tested denture base acrylics did not show any significant differences in processing shrinkage. However, ProBase Hot showed significantly lower bending deflection values than Lucitone 199 and SR-Ivocap. Compared to Lucitone 199 and SR-Ivocap, ProBase Hot is a tougher and a stiffer material. Hence, it is more likely to fracture readily if subjected to extreme loading conditions due to the accidental fall of the denture on a hard surface or if the denture wearer inadvertently bites on a particularly hard particle.

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The effect of different types of oral mouth rinses on the hardness of Silorane-based and Nano-hybrid composites

Rohit Ashok Antony Fernandez, Marwan El Araby, Mohamed Siblini, Ayed Al-Shehri

Saudi Journal of Oral Sciences 2014 1(2):105-109

Background: The restorative materials used in dentistry are required to have long-term durability in the oral cavity. Effect of various fluids in the oral environment can affect the hardness of these restorations. This study was carried out to evaluate the effect of different mouth rinses on the hardness of Silorane and Nano-hybrid resin composites. Materials and Methods: Thirty specimens were prepared from each type of composite and stored in artificial saliva for 24 h at 37°C. They were then divided into six experimental groups based on the test solution and were immersed and stored for 24 h at 37°C. Thereafter, the specimens were washed, dried and the hardness was measured for each specimen by Vickers microhardness tester. Results: Nano-hybrid resin composite showed higher Vickers hardness number (VHN) than Silorane composite in any test solution. There was only a mildly significant difference in the mean VHN of Silorane composite immersed in alcohol-containing mouthwash compared with artificial saliva. However, there was a significant difference in the mean VHN of Nano-hybrid composite when immersed in different mouthwashes. Conclusion: The microhardness values of Silorane-based and Nano-hybrid resin composites were decreased after immersion in the mouthwashes. The hardness of Nano-hybrid composite immersed in artificial saliva showed the highest VHN and was significantly affected when immersed in other test solutions. Silorane-based composite is more resistant to alcohol-containing mouthwash than Nano-hybrid composite.

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Comparative study of marginal adaptation and mechanical properties of CAD/CAM versus dual polymerized interim fixed dental prosthesis

Marwa Eltayeb I Elagra, Yousria Shalaby, Moustafa Fakhry Khalil, Nagiub ElFawal

Saudi Journal of Oral Sciences 2014 1(2):71-78

Background: With the advent of new interim crown materials, it has become imperative to evaluate its strength in order to select the appropriate interim crown and partial fixed dental prosthesis material. Aim: The purpose of this in vitro study was to compare marginal adaptation, wear resistance, and fracture resistance of a computer-aided design/computer-aided manufacturing (CAD/CAM) versus a dual polymerized interim fixed dental prosthesis (FDP). Materials and Methods: Twenty models were randomly allocated into two equal groups (I and II). Group (I) received 10 CAD/CAM fabricated interim FDP (CAD-Temp) and Group (II) received 10 directly fabricated interim FDP using a dual polymerized material (TempSpan). All specimens were stored before testing. Marginal adaptation was tested using stereomicroscope and image analyzing software to measure the amount of marginal gap. For wear resistance test, all specimens were subjected to mechanical tooth brushing. Stereomicroscope and image analyzing software were used to measure the difference in height before and after tooth brushing. Specimens were subjected to cyclic loading; specimens intact from defects were then loaded to fracture. Data were analyzed using Student's T- test, Mann Whitney test and Fisher exact test (α = 0.05). Results: CAD-Temp (15.026 ± 4.340 μm) showed significantly higher marginal adaptation than TempSpan (145.418 ± 25.365μm)(P < 0.001). The wear resistance of CAD-Temp (7.3322 ± 0.99853 μm) was significantly higher than TempSpan (22.3165 ± 2.35459 μm) (P < 0.001). The fracture resistance of CAD-Temp (605.1 ± 31.718N) was also significantly higher than that of TempSpan (225.76 ± 32.469N) (P < 0.001). Conclusions: CAD/CAM fabricated interim restorations have better marginal adaptation, wear resistance and fracture resistance than dual polymerized interim restorations hence, might withstand longer duration in the oral cavity.

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Rare presentation of radicular cyst with sebaceous differentiation

Malay Kumar, Tapan G Modi, Manas Bajpai, Ronak Nanavati

Saudi Journal of Oral Sciences 2014 1(2):120-122

Sebaceous glands in the oral mucosa are said to be a normal variation, but the presence of the sebaceous gland in the jaw is extremely rare. Sebaceous gland differentiation in dentigerous cysts and in keratocystic odontogenic tumor (earlier odontogenic keratocysts) has been reported, but it has never been reported in any radicular cyst. We presented a case of a radicular cyst in an 18-year-old male with sebaceous gland differentiation.

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A retrospective study of panoramic radiographs for zygomatic air cell defect in children

Srikanth H Srivathsa, Suchetha N Malleshi, Karthikeya Patil, Mahima V Guledgud

Saudi Journal of Oral Sciences 2014 1(2):79-82

Background and Aim: The aim was to determine the prevalence of zygomatic air cell defect (ZACD) among children of Indian origin and also to determine the dominant form of ZACD. Materials and Methods: One thousand six hundred and eighty-eight routine panoramic radiographs of subjects between 7 and 18 years, processed between January 2006 and January 2009 were screened, recorded and analyzed for ZACD. Results: Of the 1688 panoramic radiographs examined, 768 were of males and 920 of females. The radiographs were divided into two groups, Group I comprising of subjects in the age 7-12 years and other Group II in the age range 13-18 years. ZACD was found in 50 subjects, accounting for prevalence of 2.96%. Other occurrences found were unilateral 38 cases, bilateral 12, unilocular 20 and multilocular 42 respectively. Conclusion: This study described the prevalence and characteristics of ZACD among selected Indian children. It is essential to know and determine this variant of normal anatomy and differentiate it from pathologies.

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Knowledge and orientations of medical interns toward periodontal disease in Saudi Arabia

Farah Asa'ad, Nassr Al-Maflehi, Bushra Alelyan, Lama Asaad, Walaa Alrumaih, Fatimah Alassad, Sara Alshakhs, Basma Alshakhs

Saudi Journal of Oral Sciences 2014 1(2):98-104

Background and Aim: This study was conducted to assess the periodontal knowledge, orientations of Saudi medical interns and the medical curriculum. Study Population and Methodology: Questionnaires were distributed to 221 medical interns chosen randomly from two major educational institutions in Saudi Arabia. Questions were developed from literature reviews. The questionnaire had five true/false knowledge items, and eight Likert-scale questions. Data were analyzed by computerized SPSS (version 20). Level of significance was set at P < 0.05. Results: Of the 221 respondents, 66% were male and 34% were female. Most of the participants were 24 years old (48%). About 40% of the interns answered all five true/false general knowledge questions correctly, nearly half of the respondents (49.8%) reported they never asked patients if they were diagnosed with periodontal disease, 93.2% reported not receiving any training about periodontal disease in medical school, 23.5% reported they would feel absolutely uncomfortable performing a simple periodontal examination, about one-third of interns (29.4%) agreed that patients expect physicians to discuss/screen for periodontal disease, 43.9% felt that discussing/evaluating the periodontal status of their patients was peripheral to their role as physicians, nearly half of interns (48.4%) reported never screening patients for periodontal disease, and 33% stated they never referred patients to dentists. Conclusion: Medical interns were not prepared nor trained to screen patients for periodontal disease. They had limited knowledge about the association between periodontal health and general health. Oral health training as part of the medical curriculum is strongly recommended.

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Detection of bacteria in endodontic samples and its association with defined clinical signs and symptoms of endodontic infection

Shaikha Al-Samahi, Mohammad A Al-Omari

Saudi Journal of Oral Sciences 2014 1(2):83-89

Introduction : The role of bacteria in pulpal and periradicular lesions is well established. However, the association of bacterial species with signs and symptoms of endodontic infection is not well elucidated. The purpose of this study was to investigate the types of bacterial species in infected root canals (using MicroSeq 500 16S ribosomal ribonucleic acid, rRNA) and the association of bacterial species with clinical signs and symptoms of different pulpal and periapical infections. Materials and Methods: Microbial samples were obtained from 92 adult patients (one tooth with single root canal for each patient). Clinical data including signs and symptoms were collected. Polymerase chain reaction (PCR) (MicroSeq 500 16S rRNA) was used to detect bacteria in the infected root canals. Results: A total of 179 bacterial species were identified. Actinomyces viscosus and Streptococcus sanguis were associated with the presence of pain. While Staphylococcus haemolyticus and Veillonella spp. were associated with pain of palpation, the pain to percussion was associated with Actinomyces spp., Actinomyces naeslundii, and Actinomyces viscosus. Also, Streptococcus mitis, Bacteroides spp., and Veillonella spp. were associated with periapical swelling. The presence of sinus tract was found to be significantly associated with Neisseria spp. and Staphylococcus haemolyticus. The wet canal was significantly associated with the presence of Propionibacterium acnes and Bacteroides spp. Conclusions: There are significant associations between the presence of some bacterial species with clinical signs and symptoms of infected root canals.

http://ift.tt/238UCtO

Intra-alveolar auto-transplantation to correct a single tooth rotation: A case report with four years of follow-up

Sunil Babu Kotha

Saudi Journal of Oral Sciences 2014 1(2):110-113

It is uncommon for children or young adults to have rotated central incisors. The restorative options are to carry out an orthodontic treatment or root canal treatment followed by crown placement for their correction. Often overlooked and misunderstood, another treatment option in the intra-alveolar auto-transplantation where the tooth is rotated within the same socket to an appropriate position. This article highlights the overview of intra-alveolar auto-transplantation along with a case of rotated central incisor. A 12-year-old complaining of rotated tooth and wanted it to be corrected. On clinical examination, there was rotation of maxillary left central incisor with no history of any gingival and periodontal involvement. After obtaining the consent from the patient and his parent, intra-alveolar auto-transplantation procedure was carried out. After a four-year follow-up, the concerned tooth, had retained its vitality and showed no signs of relapse.

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Erratum to: Quality of life improves early after gender reassignment surgery in transgender women



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Dentin hypersensitivity among undergraduates in a university community

Omoigberai Bashiru Braimoh, Nzube Anthony Ilochonwu

Saudi Journal of Oral Sciences 2014 1(2):90-93

Background and Aim: There is limited data on dentin hypersensitivity (DH) among young adults in Africa. The objective of this study was to determine the prevalence and trigger factors associated with DH among young adults in a university community in Nigeria. Materials and Methods: The study population consisted of 360 young adults, 188 males and 172 females, aged 18-33 years. All individuals answered questions regarding demography, self-reported dentin sensitivity, trigger factors, action taken, and impact of DH on quality of life. Statistical analysis used descriptive statistics and the Chi-square test. Results: The prevalence of DH was 228 (63.3%) among the surveyed population and was significantly higher in females as compared to males (P = 0.03). All the participants who reported DH were right-handed. Among the participants with DH, majority 139 (61.0%) have not taken any action. The major precipitant for the DH was a cold drink 78 (34.2%). Of the 228 who experienced DH, 92 (40.3%) indicated eating as the precipitant, 76 (33.3%) indicated tooth brushing and 22 (9.6%) indicated talking as the precipitant. Approximately, 30% of the participants expressed unhappiness due to tooth sensitivity. Individuals with functional and psychological disturbance were significantly more likely to visit a dentist. Conclusion: The prevalence of DH found in this study was higher than previously reported, suggesting an increase in the levels of sensitivity within the general population. All the participants were right-handed and left side of the mouth was the most commonly affected.

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Neumann's tumor in new born: A case requiring surgical intervention

Murali Nirupama, Ganesh Pai, Anand Pai, Reshma Pai

Saudi Journal of Oral Sciences 2014 1(2):117-119

Congenital epulis (CE) also known as granular cell tumor of new born was first described by Neumann in the year 1871. Most frequent location includes maxillary alveolar ridge with a female preponderance up to 8 times more than males. These tumors are usually small and can be solitary or multiple, pedunculated or protuberant mucosa covered nodules presenting at birth. Multiple theories have been proposed for their origin. Though, it may cause panic at birth they are indolent and regress spontaneously. Rare cases require surgical intervention in the neonatal period when symptomatic, causing difficulties in respiration or deglutition. We describe a case of CE in a 2-day-old female neonate, who presented with pink protuberant mass in the anterior alveolar ridge of maxilla. The mass was symptomatic and surgery was indicated. The mass was excised and the benign nature was confirmed by histopathology.

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Prevalence of torus palatinus and torus mandibularis in an Indian population

Santosh Patil, Sneha Maheshwari, Suneet Khandelwal Khandelwal

Saudi Journal of Oral Sciences 2014 1(2):94-97

Background and Aim: Oral tori are bony growth present in the oral cavity and are not considered as pathological lesions. Smaller tori do not cause any problems, but larger sized tori can result in significant problems. The aim of this study was to determine the prevalence of torus palatinus (TP) and torus mandibularis (TM) in the Indian population. Study Design: A total of 3087 patients were examined between August, 2010 and October, 2012 for the presence of TP and TM by inspection and palpation. The data were collected and analyzed using SPSS 12.0 (SPSS Inc., Chicago, USA). Results: A total of 36 patients (1.3%) presented with TP, while 214 patients (6.9%) presented with TM. Tori were more frequent in males and this difference was statistically significant (P < 0.05). TP was more common in 31-40 years age group (13 patients, 36.1%), whereas TM was more in the 41-50 years age group (81 patients, 37.9%). The most commonly observed type of TP was flat shaped, whereas bilateral solitary types were the most common TM. Conclusion: The results of this study showed a significantly higher prevalence of tori in males. No difference in the prevalence of TP and TM was noted regarding the age groups. Although, not pathologically significant, these bony overgrowths very often need surgical removal, especially when prosthesis are indicated.

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Hairy intraoral flaps: An uncommon complication of surgical therapy in oral cancer

Gaurav Sharma, Archna Nagpal

Saudi Journal of Oral Sciences 2014 1(2):123-124



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Oxidative Priority, Meal Frequency, and the Energy Economy of Food and Activity: Implications for Longevity, Obesity, and Cardiometabolic Disease

Metabolic Syndrome and Related Disorders , Vol. 0, No. 0.


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Associations Between Lifestyle Characteristics and the Presence of Nonalcoholic Fatty Liver Disease: A Case–Control Study

Metabolic Syndrome and Related Disorders , Vol. 0, No. 0.


http://ift.tt/2eZ2TRY

Autoinflammatory Skin Disease: A Review of Concepts and Applications to General Dermatology

We provide an up-to-date summary of important concepts of autoinflammation as well as describe important but rare monogenic autoinflammatory disorders that may present with cutaneous findings. Finally, of particular interest to a practicing general dermatology audience, we review concepts of autoinflammation as they apply to understanding the disease pathogenesis of common skin disorders.
Dermatology

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Prognostic impact of the putative cancer stem cell markers ABCG2, CD133, ALDH1A1 and CD44V7/8 in metastatic melanoma

Despite recent therapeutic advances, a diagnosis of metastatic melanoma still foreshadows a grim prognosis for the majority of patients. One of the factors believed to be associated with the failure of current conventional cancer therapies are the so-called Cancer Stem Cells (CSCs). CSCs share common features with corresponding tissue stem cells, such as self-renewal capacity and the ability to give rise to progeny with the potential to proliferate and differentiate. Therapeutically targeting CSCs may thus eliminate the root cause of the cancer, however this would require the accurate identification of markers distinguishing CSCs from normal cells. The expression of candidate CSC markers is associated with a poor prognosis in a number of cancer types, but their clinical significance remains unclear and to the best of knowledge there have been no clinical studies in melanoma. Here, we aimed to determine the clinical significance of four molecules identifying putative CSCs, namely CD133, ABCG2, ALDH1A1 and CD44v7/8.

This article is protected by copyright. All rights reserved.



http://ift.tt/2gdtBEc

Prognostic impact of the putative cancer stem cell markers ABCG2, CD133, ALDH1A1 and CD44V7/8 in metastatic melanoma

Despite recent therapeutic advances, a diagnosis of metastatic melanoma still foreshadows a grim prognosis for the majority of patients. One of the factors believed to be associated with the failure of current conventional cancer therapies are the so-called Cancer Stem Cells (CSCs). CSCs share common features with corresponding tissue stem cells, such as self-renewal capacity and the ability to give rise to progeny with the potential to proliferate and differentiate. Therapeutically targeting CSCs may thus eliminate the root cause of the cancer, however this would require the accurate identification of markers distinguishing CSCs from normal cells. The expression of candidate CSC markers is associated with a poor prognosis in a number of cancer types, but their clinical significance remains unclear and to the best of knowledge there have been no clinical studies in melanoma. Here, we aimed to determine the clinical significance of four molecules identifying putative CSCs, namely CD133, ABCG2, ALDH1A1 and CD44v7/8.

This article is protected by copyright. All rights reserved.



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Onset of psoriatic arthritis associated with multiple wasp stings

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Publication date: Available online 20 November 2016
Source:Annals of Allergy, Asthma & Immunology
Author(s): Tara Vinyette Saco, Mark C. Glaum, Dennis K. Ledford, Richard F. Lockey




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A Case of Periodontal Necrosis following Embolization of Maxillary Artery for Epistaxis

Embolization of the maxillary artery (MA) is a common treatment modality for refractory epistaxis. Tissue necrosis after embolization of the MA is a rare complication. Here, we reported the first case of the development of necrosis of soft tissue and alveolar bone in the periodontium after embolization. A 48-year-old man with poor oral hygiene and a heavy smoking habit was referred to our clinic due to intractable epistaxis. After treatment with anterior-posterior nasal packing (AP nasal packing), the epistaxis relapsed. Therefore, he underwent embolization of the MA. Although he did not experience epistaxis after embolization, periodontal necrosis developed gradually. The wound healed with necrotomy, administration of antibiotics and prostaglandin, and hyperbaric oxygen therapy. We speculated that the periodontal necrosis was provoked by reduction of blood supply due to embolization and AP nasal packing based on this preexisting morbid state in the periodontium. Poor condition of the oral cavity and smoking may increase the risk of periodontal necrosis after embolization.

http://ift.tt/2fUuMaW

A Case of Periodontal Necrosis following Embolization of Maxillary Artery for Epistaxis

Embolization of the maxillary artery (MA) is a common treatment modality for refractory epistaxis. Tissue necrosis after embolization of the MA is a rare complication. Here, we reported the first case of the development of necrosis of soft tissue and alveolar bone in the periodontium after embolization. A 48-year-old man with poor oral hygiene and a heavy smoking habit was referred to our clinic due to intractable epistaxis. After treatment with anterior-posterior nasal packing (AP nasal packing), the epistaxis relapsed. Therefore, he underwent embolization of the MA. Although he did not experience epistaxis after embolization, periodontal necrosis developed gradually. The wound healed with necrotomy, administration of antibiotics and prostaglandin, and hyperbaric oxygen therapy. We speculated that the periodontal necrosis was provoked by reduction of blood supply due to embolization and AP nasal packing based on this preexisting morbid state in the periodontium. Poor condition of the oral cavity and smoking may increase the risk of periodontal necrosis after embolization.

http://ift.tt/2fUuMaW

Infantile neuroaxonal dystrophy and PLA2G6-associated neurodegeneration: An update for the diagnosis

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Publication date: Available online 21 November 2016
Source:Brain and Development
Author(s): Alessandro Iodice, Carlotta Spagnoli, Grazia Gabriella Salerno, Daniele Frattini, Gianna Bertani, Patrizia Bergonzini, Francesco Pisani, Carlo Fusco
Infantile neuroaxonal dystrophy is a rare neurodegenerative disorder characterized by infantile onset of rapid motor and cognitive regression and hypotonia evolving into spasticity. Recessively inherited mutations of the PLA2G6 gene are causative of infantile neuroaxonal dystrophy and other PLA2G6-associated neurodegeneration, which includes conditions known as atypical neuroaxonal dystrophy, Karak syndrome and early-onset dystonia-parkinsonism with cognitive impairment. Phenotypic spectrum continues to evolve and genotype-phenotype correlations are currently limited. Due to the overlapping phenotypes and heterogeneity of clinical findings characterization of the syndrome is not always achievable. We reviewed the most recent clinical and neuroradiological information in the way to make easier differential diagnosis with other degenerative disorders in the paediatric age. Recognizing subtle signs and symptoms is a fascinating challenge to drive towards better diagnostic and genetic investigations.



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Timing of maternal exposure and fetal sex determine the effects of low-level chemical mixture exposure on the fetal neuroendocrine system in sheep

Abstract

We have shown that continuous maternal exposure to the complex mixture of environmental chemicals (ECs) found in human biosolids (sewage sludge), disrupts mRNA expression of genes crucial for development and long-term regulation of hypothalamo-pituitary gonadal (HPG) function in sheep. This study investigated whether exposure to ECs only during preconceptional period or only during pregnancy perturbed key regulatory genes within the hypothalamus and pituitary gland and whether these effects were different from chronic (life-long) exposure to biosolid ECs. The findings demonstrate that the timing and duration of maternal EC exposure influences the subsequent effects on the fetal neuroendocrine system in a sex-specific manner. Maternal exposure prior to conception or during pregnancy only, altered the expression of key fetal neuroendocrine regulatory systems such as GnRH and kisspeptin to a greater extent than when maternal exposure was 'life-long'. Furthermore, hypothalamic gene expression was affected to a greater extent in males than in females, and following EC exposure, male fetuses expressed more "female-like" mRNA levels for some key neuroendocrine genes. This is the first study to show that "real-life" maternal exposure to low levels of a complex cocktail of chemicals prior to conception can subsequently affect the developing fetal neuroendocrine system. These findings demonstrate that the developing neuroendocrine system is sensitive to EC mixtures in a sex-dimorphic manner likely to predispose to reproductive dysfunction in later life.

This article is protected by copyright. All rights reserved.



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Tolosa-Hunt syndrome and comorbidity of obsessive compulsive disorder and aortic aneurysm

Description

A 59-year-old man with obsessive compulsive disorder (OCD) was on antidepressant treatment for years. Six months ago, he got a severe headache on the left side of his head, reported pain behind his left eye, diplopia and ptosis displayed on his left eyelid. The patient had mild migraine attacks occasionally. However, his previous headache was very intense when compared with earlier ones. By cranial MRI, a lesion (22x7 mm horizontally, 13x8 mm vertically) adjacent to carotid segment 4 (C4) of the left internal carotid artery was found. The lesion showed a hyperintense signal in contrast-enhanced T2-weighted images, which was the result of enhanced abnormal soft tissue extending through the orbital fissure and into the orbital apex. This did not cause compression on the optic nerve and there was slight bulging into the left cavernous sinus (figure 1). The patient's blood and cerebrospinal fluid biochemical parameters were within...



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A call for neurologists to take up stroke intervention

Vasantha M Padma, Rohit Bhatia, Gigy Kuruttukulam, Anand Alurkar, Kewal Krishan Talwar, Dheeraj Khurana, Subhash Kaul, Vinit Suri, Gagandeep Singh, Vikram Huded

Annals of Indian Academy of Neurology 2016 19(4):429-432

Recent data have provided overwhelming evidence in favor of benefits of emergent endovascular intervention in large vessel acute ischemic stroke (AIS). India with its large population has a huge burden of AIS. Hence, neurologists need to gear up to the new challenge of providing interventional care to huge populations of AIS in the country. The best way to cover this unprecedented unmet need is to encourage neurologists to take up interventional subspecialty interests through new but sound training pathways.

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Approach to a tremor patient

Soumya Sharma, Sanjay Pandey

Annals of Indian Academy of Neurology 2016 19(4):433-443

Tremors are commonly encountered in clinical practice and are the most common movement disorders seen. It is defined as a rhythmic, involuntary oscillatory movement of a body part around one or more joints. In the majority of the population, tremor tends to be mild. They have varying etiology; hence, classifying them appropriately helps in identifying the underlying cause. Clinically, tremor is classified as occurring at rest or action. They can also be classified based on their frequency, amplitude, and body part involved. Parkinsonian tremor is the most common cause of rest tremor. Essential tremor (ET) and enhanced physiological tremor are the most common causes of action tremor. Isolated head tremor is more likely to be dystonic rather than ET. Isolated voice tremor could be considered to be a spectrum of ET. Psychogenic tremor is not a diagnosis of exclusion; rather, demonstration of various clinical signs is needed to establish the diagnosis. Severity of tremor and response to treatment can be assessed using clinical rating scales as well as using electrophysiological measurements. The treatment of tremor is symptomatic. Medications are effective in half the cases of essential hand tremor and in refractory patients; deep brain stimulation is an alternative therapy. Midline tremors benefit from botulinum toxin injections. It is also the treatment of choice in dystonic tremor and primary writing tremor.

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An elderly male with tingly legs and a heavy heart: Persisting with the diagnosis!

Mudassar Kharadi, Sreenath Vedula Harsha, Gayathri Gopalakrishnan, GA Karthick, Vimal Raj, Subramanian Kannan

Annals of Indian Academy of Neurology 2016 19(4):444-447

Pursuing a diagnosis despite a negative nerve biopsy report requires a strong clinical conviction. Paying attention to clinical details and a diligent review of medical records is needed to solve cases in which the diagnosis is not obvious despite numerous tests. We present once such case of an elderly man with chronic diarrhea, weight loss, and peripheral neuropathy and elaborate on a step by step basis on how the clinical thought process evolved after sifting through the reports of scan and tests done during his work up.

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Godfrey Newbold Hounsfield (1919-2004): The man who revolutionized neuroimaging

Kalyan B Bhattacharyya

Annals of Indian Academy of Neurology 2016 19(4):448-450

Godfrey Hounsfield, a biomedical engineer contributed enormously towards the diagnosis of neurological and other disorders by virtue of his invention of the computed axial tomography scan for which he was awarded the Nobel Prize in 1979. Working for the Electrical and Musical Industry (EMI) Limited and in collaboration with two radiologists, James Ambrose and Louis Kreel, he introduced the use of this machine in 1971 at the Atkinson Morley's Hospital in Wimbledon. He continued to improve the quality of the devise and the human head was scanned for the first time in 1972. He continued his work on imaging of the human body was later concentrated on the next step in diagnostic radiology namely, magnetic resonance imaging.

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Assessment of fatigue in Parkinson's disease: Indian perspective

Birinder Singh Paul, Amandeep Singh, Dinesh Jain, Gagandeep Singh, Sandeep Kaushal, Gunchan Paul, Karan Kaura

Annals of Indian Academy of Neurology 2016 19(4):451-455

Context: Fatigue is one of the most frequent nonmotor manifestations in Parkinson's disease (PD), having a major effect on quality of life but is not reported in Indian patients. Aims: To evaluate the frequency of fatigue in a cohort of PD population and its correlation with disease. Settings and Design: Fatigue Severity Scale (FSS) was translated and validated in local vernacular language. All patients of PD visiting neurology outpatient department of a tertiary care hospital. Subjects and Methods: A total of 150 patients were screened, and 104 were included in this study. They were divided into - Group I with fatigue (score of >4 in each item) and Group II without fatigue. Statistical Analysis: Data were analyzed by SPSS software version 20.0. Spearman correlation was used to evaluate the convergent validity of the FSS-Ind score with PD-related variables. The principal components analysis was applied to detect the domain structure of the FSS. Results: Of the total 104 patients, 68 (65.3%) patients experienced fatigue. The duration of disease was significantly more (P = 0.021) in Group I (4.39 ± 3.8 years) than in the Group II (3.13 ± 1.6 years). The severity of disease also showed a positive correlation with fatigue with 50.9% patients in H and Y stage >3 experiencing fatigue. 69.1% patients of tremor phenotype experienced fatigue as compared to 32.3% of rigid phenotype. There was no relation of fatigue with age, gender, H and Y stage, levodopa equivalent dose and mean Unified PD Rating Scale motor III score. Conclusions: Translated version of the FSS, FSS-Ind has high internal consistency and validity which supports its application as an effective tool in detecting fatigue in patients with PD. Fatigue in PD was related to duration and phenotype of the disease.

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Diagnosis and management of narcolepsy in the Indian scenario

Sachin Sureshbabu, Abdul Muniem, Manvir Bhatia

Annals of Indian Academy of Neurology 2016 19(4):456-461

Introduction: The diagnosis and management of narcolepsy in the Indian context needs to be revisited especially in the wake of concerns raised by sleep medicine experts that the entity could be formidably underdiagnosed, as well as undertreated in our setting. Materials and Methods: The history, clinical records, polysomnographic/multiple sleep latency test data, and treatment records of five hundred consecutive patients attending a dedicated sleep clinic between the years 2013 and 2016 were retrospectively analyzed. The response to treatment measures and improvement in daytime functioning were periodically assessed by personal/telephonic interview and E-mail communication. Results: Thirteen patients were diagnosed with narcolepsy based on the standard criteria of which three had cataplexy. The mean age of presentation was 23.23 years and the male:female ratio was 2.25:1. The mean duration from the onset of symptoms to diagnosis was 4.2 years. Two patients responded to nonpharmacological interventions alone, and six to modafinil, while two patients remained symptomatic and required treatment with methylphenidate. One patient was lost to follow-up, while two others are due for their first follow-up. Conclusion: A refurbished outlook of the diagnostic methodology and treatment paths tailored to our clinical scenario can potentially impact the future of narcolepsy management and research in our country.

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Role of brain natriuretic peptide as a novel prognostic biomarker in acute ischemic stroke

Bindu Menon, Krishnana Ramalingam, Jyoti Conjeevaram, K Munisusmitha

Annals of Indian Academy of Neurology 2016 19(4):462-466

Aim: We investigated to study the prognostic importance of brain natriuretic peptide (BNP) in ischemic stroke. Materials and Methods: We prospectively enrolled 100 patients with acute ischemic stroke and measured plasma BNP levels and compared with age- and sex-matched healthy controls. Risk factors, biochemical parameters, lipid profile, carotid and vertebral Doppler, imaging, and cardiac evaluation were done. Stroke severity was assessed by the National Institutes of Health Stroke Scale (NIHSS) score on admission and functional disability by Barthel Index (BI) at 3 months. Ischemic stroke subtype was classified according to the Oxfordshire Community Stroke Project (OCSP). Data were entered in MS Excel, and appropriate statistical analysis was done using the SPSS software version 21.0. A P = 0.05 was considered as significant. Results: Mean age of patients was 55.17 ± 11.37 years with a male:female ratio 3:1. OCSP showed total anterior circulation infarct (TACI) 35, partial anterior circulation infarct 9, lacunar infarct 12, and posterior circulation infarct 44. NIHSS on admission was average 10 ± 7 and BI was 57 ± 30. BNP in patients (435 ng/ml) was very high as compared to controls (<60 ng/ml) (P < 0.001). There was a positive correlation between age and BNP (R2 = 0.34; P < 0.00); NIHSS and BNP (R2 = 0.255; P < 0.01), negative correlation between BI and BNP (R2 = −0.064; P < 0.01). Mean BNP levels across the OCSP showed higher values in TACI (F = 4.609 P = 0.005). Regression analysis showed that BNP can predict BI which was statistically significant. Conclusion: Plasma BNP levels was significantly elevated in patients with ischemic stroke. Our study concludes that high BNP levels are seen in large anterior circulation stroke and is a predictor for the poor functional outcome at 3 months. Determination of BNP levels as a biomarker could be helpful in predicting the outcome in stroke patients.

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Interaction of incidental microbleeds and prior use of antithrombotics with early hemorrhagic transformation: Causative or protective?

Konark Malhotra, Monica Khunger, Bichun Ouyang, David S Liebeskind, Yousef M Mohammad

Annals of Indian Academy of Neurology 2016 19(4):467-471

Background: Gradient echo (GRE) sequence of magnetic resonance imaging (MRI) is a sensitive tool to detect hemorrhagic transformation (HT) and old cerebral microbleeds (CMBs). Presence of CMBs and prior use of antithrombotics pose a risk of HT in ischemic stroke. We evaluated the association of CMBs and antithrombotic use with resultant HT in acute ischemic stroke (AIS). Methods: This retrospective study included AIS patients admitted to our center between January 2009 and August 2010 who underwent GRE-weighted MRI within 48 h of admission. Demographic and clinical data including diabetes mellitus, hypertension, hyperlipidemia, prior intake of antiplatelets/anticoagulants/statins, and presence of CMBs at admission were collected and compared between patients who developed HT and those who did not. We did a multivariate analysis using logistic regression to assess the effect of CMBs and prior use of antithrombotic agents on the risk of development for early HT in ischemic stroke. Results: Of 529 AIS patients, 81 (15%) were found to have HT during the initial hospital course. CMBs were found in only 9 of 81 patients (11%) with HT and in 40 out of remaining 448 patients (9%) who did not develop HT. The presence of CMBs was not associated with increased risk of HT (P = 0.53). However, prior use of antiplatelets (33% vs. 47% in the patients without HT, P = 0.02) was associated with decreased risk of HT in ischemic stroke. Conclusion: Presence of incidental CMBs was not associated with increased risk for early HT of an ischemic stroke. Interestingly, the prior intake of antiplatelets was found to be protective against HT of ischemic stroke.

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A double-blind, randomized, controlled trial to compare the efficacy and tolerability of fixed doses of ropinirole, bupropion, and iron in treatment of restless legs syndrome (Willis-Ekbom disease)

Kirti Vishwakarma, Juhi Kalra, Ravi Gupta, Mukesh Sharma, Taruna Sharma

Annals of Indian Academy of Neurology 2016 19(4):472-477

Background: We aimed to compare the efficacy of fixed doses of bupropion and ropinirole and iron alone for the treatment of restless legs syndrome (RLS) and to look for the tolerability of these medications. Materials and Methods: Patients diagnosed with RLS were randomly divided into three groups with thirty patients in each group (Group A: Bupropion [300 mg/day], Group B: Ropinirole [0.25-0.5 mg/day], and Group C: Oral iron [150 mg elemental iron] along with folic acid [500 μg]). Each participant was then assessed for severity of RLS, as well as RLS-related quality at the baseline, and thereafter, every 14 th day till 6 weeks based on the International Restless Legs Scale (IRLS) severity rating scale and Restless Legs Syndrome Quality of Life (RLSQoL) Questionnaire, respectively. Results: IRLS scores differed significantly from baseline visit to last (F = 4.85; P = 0.01). The interaction between the time x treatment group was significant (F = 10.37; P < 0.001) showing an improvement with the therapy in all the groups. Pair-wise comparison depicted that ropinirole group differed from other two groups in IRLS score (F = 7.06; P = 0.001), which were comparable to each other. Regarding quality of life of these cases, within each group scores differed among all the four visits (F = 5.12; P = 0.002). Unlike IRLS, there was no significant difference among the RLSQOL scores between groups at any point of time (F = 1.2; P = 0.28). Conclusion: RLS severity decreased across time in all three groups; however, the ropinirole treatment was better than the bupropion and iron-folate therapy. Moreover, RLS-related quality of life although improved among all groups, it was comparable among three groups.

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Clinical correlates of leukoaraiosis: A study of 175 patients

Rustom S Wadia, Sandesh K Ghiya, Joshita Singh, Santosh M Sontakke, Vishwas Bharadwaj, Rahul V Sonawane, Yogesh P Bade, K Shrikanth, Nikhil Goli, Rohit Singh Chauhan, Nilesh A Nadkarni

Annals of Indian Academy of Neurology 2016 19(4):478-481

Background: In India, the correlates of leukoaraiosis (LA) have not been widely reported. This study was designed to investigate the factors which correlate with LA. Materials and Methods: We included patients with LA who consented for the study and graded their severity on the basis of Fazekas scale. We excluded patients with LA who did not consent/cooperate for the study as also patients with other white matter changes which mimic LA. Results: LA is a common and under-rated cause of disability. Presentations include cognitive decline, gait disturbance, dysarthria, bladder/bowel sphincter disturbances, and increased risk of stroke. The comorbidities include hyperhomocysteinemia, hypertension, dyslipidemia, tobacco use, ischemic heart disease, previous stroke, atrial fibrillation, chronic renal failure, and bariatric surgery.

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Retrospective analysis of multiplex polymerase chain reaction-based molecular diagnostics (SES) in 70 patients with suspected central nervous system infections: A single-center study

Rama Krishnan Tiruppur Chinnappan Ramalingam, Dipanjan Chakraborty

Annals of Indian Academy of Neurology 2016 19(4):482-490

Background: Central nervous system (CNS) infections present a grave health care challenge due to high morbidity and mortality. Clinical findings and conventional laboratory assessments are not sufficiently distinct for specific etiologic diagnosis. Identification of pathogens is a key to appropriate therapy. Aim: In this retrospective observational study, we evaluated the efficacy and clinical utility of syndrome evaluation system (SES) for diagnosing clinically suspected CNS infections. Materials and Methods: This retrospective analysis included inpatients in our tertiary level neurointensive care unit (NICU) and ward from February 2010 to December 2013. Cerebrospinal fluid (CSF) samples of 70 patients, clinically suspected of having CNS infections, were subjected to routine laboratory tests, culture, imaging, and SES. We analyzed the efficacy of SES in the diagnosis of CNS infections and its utility in therapeutic decision-making. Results: SES had a clinical sensitivity of 57.4% and clinical specificity of 95.6%. Streptococcus pneumoniae and Pseudomonas aeruginosa were the top two bacterial pathogens, whereas Herpes simplex virus (HSV) was the most common viral pathogen. Polymicrobial infections were detected in 32.14% of SES-positive cases. SES elicited a change in the management in 30% of the patients from initial empiric therapy. At discharge, 51 patients recovered fully while 11 patients had partial recovery. Three-month follow-up showed only six patients to have neurological deficits. Conclusion: In a tertiary care center, etiological microbial diagnosis is central to appropriate therapy and outcomes. Sensitive and accurate multiplex molecular diagnostics play a critical role in not only identifying the causative pathogen but also in helping clinicians to institute appropriate therapy, reduce overuse of antimicrobials, and ensure superior clinical outcomes.

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CD6 gene polymorphism rs17824933 is associated with multiple sclerosis in Indian population

Mary Anitha D'Cunha, Lekha Pandit, Chaithra Malli

Annals of Indian Academy of Neurology 2016 19(4):491-494

Background: Multiple sclerosis (MS) prevalence has increased worldwide. The known genetic association for MS in the west has not been studied in detail in nonwhite populations and particularly Indians. Objective: The objective of this study was to evaluate some known genetic variations outside the major histocompatibility complex (MHC) region associated with MS in patients of Indian origin. Materials and Methods: We investigated 10 gene-associated single nucleotide polymorphisms (SNP's) outside the MHC region in 300 patients and 720 unrelated controls. Genotyping was performed on an ABI7500 real-time polymerase chain reaction genotyping platform using predesigned TaqMan SNP genotyping assays. Results: CD6 gene associated SNP (rs17824933) showed significant association with MS (P = 4.2 × 10−5 , odds ratio [OR] = 2.24, confidence interval (CI) = 1.51-3.33). A modest association was also noted for TMEM39A rs1132200 (P = 0.023, OR = 1.41, CI = 1.05-1.91) and IL2RA rs2104286 (P = 0.04, OR = 1.3, CI = 1.006-1.67). In the remaining SNPs, the allele frequencies were overexpressed in patients when compared to healthy controls. Conclusion: Our data illustrate the similarity in risk association between Indian and European populations for MS.

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The clinical significance of brain microbleeds in patients with Alzheimer's disease: Preliminary study

Jae-Hyeok Heo, Dong-Gyu Im, Seung-Hyeon Lee, Jin-Young Ahn

Annals of Indian Academy of Neurology 2016 19(4):495-498

Background: Microbleeds (MBs) are observed frequently in Alzheimer's disease (AD) and suggested to play a crucial role in the pathophysiology, but their clinical significance remains unclear. Materials and Methods: The study recruited 100 patients with AD who were diagnosed at the memory clinic in Seoul Medical Center in 2014. For each patient, baseline characteristics, neuropsychological tests, cerebrovascular risk factors, medial temporal lobe atrophy (MTLA), and severity of small vessel disease (SVD) according to the existence of MBs were evaluated. Results: The prevalence of MBs in patients with AD was 33%. The percentage of male gender, the severity of SVD and MTLA were significantly increased in MB(+) group. The MB(+) group showed more severe MTLA and SVD than MB(−) group. Conclusions: These results suggested that MBs might reflect the burden of amyloid and ischemic vascular pathology.

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Validation of a modified Hindi version of the Epworth Sleepiness Scale among a North Indian population

Geetika Bajpai, Garima Shukla, Ravindra M Pandey, Anupama Gupta, Mohammed Afsar, Vinay Goyal, Achal Srivastava, Madhuri Behari

Annals of Indian Academy of Neurology 2016 19(4):499-504

Background: Since a majority of population in India does not drive automobiles, one item on the Epworth Sleepiness Scale (ESS) requires modification and validation. In addition, data collected by us indicated that a majority of rural and urban Indians regularly spend time in prayer/spiritual activity. The main purpose of this study was to develop a cross-cultural adaptation of the ESS for a North Indian population, in Hindi language (ESS-I). The study also provides evidence of reliability and validity of the modified version. Methodology: The subjects included were normal volunteers aged 18-75 years (Group 1) (n = 70), compared with patients with complaints of excessive daytime sleepiness, who had undergone polysomnography (Group 2) (n = 22) and patients who had undergone multiple sleep latency test (Group 3) (n = 10). The study was carried out in four phases: Translation and retranslation of the original scale with modification of item 8 (mainly addition of option of question on "while offering prayers or in spiritual activity"); reliability (test-retest) (n = 30); internal consistency (using Cronbach's alpha index) (n = 102); and sensitivity to change (n = 8). Results: Group 1 showed spiritual activity as a significantly more commonly practiced activity than driving. The Cronbach's alpha for the modified version was 0.892 (excellent), and this was not improved by removing the modified item. The alpha value for Group 1 versus Groups 2 and 3 was 0.667 and 0.892, respectively. The scale was reliable over time (test-retest), and it was sensitive to sleepiness change in patients with obstructive sleep apnea during treatment. Conclusion: The ESS-I, is comparable to the original scale. It is reliable, valid, and change-sensitive. It is proposed that the modified version can be very useful for detecting sleepiness among Indian population, especially those who do not drive their own vehicles.

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Serological markers associated with neuromyelitis optica spectrum disorders in South India

Lekha Pandit, Douglas Kazutoshi Sato, Sharik Mustafa, Toshiyuki Takahashi, Anitha D'Cunha, Chaithra Malli, Akshatha Sudhir, Kazuo Fujihara

Annals of Indian Academy of Neurology 2016 19(4):505-509

Background: Neuromyelitis optica spectrum disorders (NMOSDs) represent 20% of all demyelinating disorders in South India. No studies have determined the seroprevalence to both antibodies against aquaporin-4* and antimyelin oligodendrocyte glycoprotein antibody (anti-MOG+) in this population. Objective: To identify and characterize seropositive patients for anti-aquaporin-4 antibody (anti-AQP4+) and anti-MOG+ in South India. Materials and Methods: We included 125 consecutive patients (15 children) who were serologically characterized using live transfected cells to human M23-AQP4 or full-length MOG. Results: Among a total of 125 patients, 30.4% of patients were anti-AQP4+, 20% were anti-MOG+, and 49.6% were seronegative. No patient was positive for both. Anti-MOG+ patients represented 28.7% (25/87) of seronegative NMOSD. In comparison to anti-AQP4+ patients, anti-MOG+ patients were commonly male, had less frequent attacks and milder disability on expanded disability status score scale. Seronegative patients were also predominantly male, 36% (9/25) had monophasic longitudinally extensive transverse myelitis and disability was comparable with anti-AQP4+ patients. Lumbar cord involvement was common in anti-MOG+ and seronegatives, whereas anti-AQP4+ patients had more cervical lesions. Conclusion: Anti-AQP4+/anti-MOG + patients accounted for nearly half of the patients suspected of having NMOSD in South India, indicating that antibody testing may be useful on the management of subgroups with different prognosis.

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Bilateral abducens and facial nerve palsies as a localizing sign due to reduction in intracranial pressure after fourth ventriculoperitoneal shunting

Boby Varkey Maramattom, Dilip Panikar

Annals of Indian Academy of Neurology 2016 19(4):510-511

A trapped fourth ventricle often requires fourth ventriculoperitoneal shunting (4VP). Complications of this procedure include shunt blockage, infection, shunt migration, and overdrainage. Cranial nerve palsies are very rare after 4VP shunting and have been described with over drainage and brainstem distortion. We present an unusual case of bilateral abducens and facial nerve palsies after 4VP shunting after normalization of 4 th ventricular parameters. Measurement of various brainstem angles presented us with a plausible hypothesis to explain the cranial nerve dysfunction.

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Idiopathic intracranial hypertension with spontaneous cerebrospinal fluid leak into the orbit

Joe James, James Jose, V Abdul Gafoor

Annals of Indian Academy of Neurology 2016 19(4):512-513



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Bilateral "Swirl Sign": A predictor of rebleed

Biplab Das, Dheeraj Khurana, Chiraj Kamal Ahuja

Annals of Indian Academy of Neurology 2016 19(4):514-515



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The brightening splenium: An imaging hallmark of dengue encephalopathy?

Sachin Sureshbabu, Laxmi Khanna, Sudhir Peter, Elisheba Patras, Gaurav Kumar Mittal

Annals of Indian Academy of Neurology 2016 19(4):516-517



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Brainstem dysfunction in patients with late-onset Lennox-Gastaut syndrome: Voxel-based morphometry and tract-based spatial statistics study

Kang Min Park, Yun Jung Hur, Sung Eun Kim

Annals of Indian Academy of Neurology 2016 19(4):518-522

Background: There have been a few reports of patients who developed Lennox-Gastaut syndrome (LGS) in the second decades of their life. Objectives: The aim of this study was to investigate electroclinical presentation in patients with late-onset LGS. In addition, we evaluated structural abnormalities of the brain, which may give some clue about the common pathogenic pathway in LGS. Materials and Methods: We enrolled the patients with late-onset LGS. We collected electroclinical characteristics of the patients and evaluated structural abnormalities using voxel-based morphometry (VBM) and tract-based spatial statistics (TBSS) analysis. Results: The three subjects were diagnosed with late-onset LGS. The patients have no mental retardation and normal background activities on electroencephalography (EEG), and they had generalized paroxysmal fast activities on EEG, especially during sleep. The TBSS analysis revealed that fractional anisotropy values in the patients were significantly reduced in the white matter of brainstem compared with normal controls. However, VBM analysis did not show any significant difference between the patients and normal controls. Conclusions: Patients with late-onset LGS have different clinical and EEG characteristics from those with early-onset LGS. In addition, we demonstrated that brainstem dysfunction might contribute to the pathogenesis of late-onset LGS.

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Mesenchymal Stem Cells Attenuate the Adverse Effects of Immunosuppressive Drugs on Distinct T Cell Subopulations.

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Mesenchymal Stem Cells Attenuate the Adverse Effects of Immunosuppressive Drugs on Distinct T Cell Subopulations.

Stem Cell Rev. 2016 Nov 19;

Authors: Hajkova M, Hermankova B, Javorkova E, Bohacova P, Zajicova A, Holan V, Krulova M

Abstract
Immunosuppressive drugs are widely used to treat undesirable immune reaction, however their clinical use is often limited by harmful side effects. The combined application of immunosuppressive agents with mesenchymal stem cells (MSCs) offers a promising alternative approach that enables the reduction of immunosuppressive agent doses and simultaneously maintains or improves the outcome of therapy. The present study aimed to determinate the effects of immunosuppressants on individual T cell subpopulations and to investigate the efficacy of MSC-based treatment combined with immunosuppressive drugs. We tested the effect of five widely used immunosuppressants with different action mechanisms: cyclosporine A, mycophenolate mofetil, rapamycin, and two glucocorticoids - prednisone and dexamethasone in combination with MSCs on mouse CD4(+) and CD8(+) lymphocyte viability and activation, Th17 (RORγt(+)), Th1 (T-bet(+)), Th2 (GATA-3(+)) and Treg (Foxp3(+)) cell proportion and on the production of corresponding key cytokines (IL-17, IFNγ, IL-4 and IL-10). We showed that MSCs modulate the actions of immunosuppressants and in combination with immunosuppressive drugs display distinct effect on cell activation and balance among different T lymphocytes subpopulations and exert a suppressive effect on proinflammatory T cell subsets while promoting the functions of anti-inflammatory Treg lymphocytes. The results indicated that MSC-based therapy could be a powerful strategy to attenuate the negative effects of immunosuppressive drugs on the immune system.

PMID: 27866327 [PubMed - as supplied by publisher]



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