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As population increases, one of the factors affecting life is traffic. Efficient traffic management has a direct positive impact on issues such as time, carbon dioxide emissions, and fuel consumption. Today, an important parameter under the heading of traffic is the signalling systems for intersections, which are operated with fixed-time, semi-actuated, fully actuated, and fully adaptive control methods. In this study, an adaptive traffic management model is developed for signalised intersections. The adaptive traffic management model developed includes phase extension with minimum and maximum time intervals dependent on density and phase skip features. Additionally, the most distinctive feature of the model is its flexible phase structure rather than a sequential phase. The Heybe intersection, located within the boundaries of Antalya province, is modelled one-to-one in the simulation of urban mobility (SUMO) simulation programme with real intersection data. The developed adaptive traffic management model is applied to the Heybe intersection, and the effects of the model are revealed. Improvements obtained from the SUMO simulation programme were verified through visual inspection, and high-accuracy results were determined. As a result of the studies, it was found that the application of the adaptive traffic management model developed at Heybe intersection, which has approximately 50,000 vehicles passing daily, resulted in a 27.2 % improvement in the average delay per vehicle parameter, a 32.4 % improvement in the average waiting time per vehicle parameter, and a 16.7 % improvement in the average speed per vehicle parameter.

Improving the performance at signalized intersections can be achieved through different management styles or sensor technologies. It is crucial that we measure the real-time impact of these variables on intersection performance. This study introduces a Bluetooth-based real-time performance measurement system applicable to all signalized intersections. Additionally, the developed method serves as a feedback tool for adaptive intersection management systems, providing valuable data input for performance optimization. The method developed in the study is applied at the Refik Cesur Intersection in the Polatlı district of Ankara where delay values are calculated based on traffic flows and data from Bluetooth sensors positioned at strategic locations. Initially, the intersection operated under a fixed-time signaling system, followed by a fully adaptive signaling system the next day. The performance of these two systems is compared using the Bluetooth-based application. The results show that the average delay per vehicle per day is 58.1 seconds/vehicle for the fixed-time system and 45.3 seconds/vehicle for the adaptive system. To validate the Bluetooth-based performance measurement system, the intersection is modeled and simulated using Aimsun Simulation Software Next 20.0.4. The simulation results confirm the findings of the Bluetooth-based analysis, demonstrating the effectiveness of the adaptive signaling system in reducing delays.

Batılılar tarafından fiili olarak sömürgeleştirilememiş tek Afrika ülkesi olan Etiyopya, 4. yüzyıl gibi erken bir tarihte Hıristiyanlığı benimsemiş ve Ortodoks bir geleneğe sahip olmuştur. Rakımının yüksek, etrafının Müslüman ülkeler ile çevrili olmasından dolayı Etiyopya, dış dünya ile bağını koparmak zorunda kalmış, bu sayede zamanla kendine has bir kimlik ve gelenek inşa etmeyi başarmıştır. Kadim bir Hıristiyan geleneğe sahip olan Etiyopya, Batı Hıristiyanlığı ile ise ilk defa 16. yüzyılda ülkeye gelen misyonerler yoluyla tanışmıştır. Batılı misyonerler, 16. yüzyıldan 20. yüzyıla kadar faaliyetlerde bulunmasına rağmen Etiyopya Kilisesi karşısında başarılı olamamışlardır. Ancak Etiyopya, 20. yüzyılın ilk yarısından itibaren modernleşme düşüncesi ile kapılarını özellikle Protestan misyonerlere açmak zorunda kalmıştır. Ülkedeki Protestan nüfus 1950 yılına kadar yüzde bir civarında iken günümüzde bu oran yüzde yirmilere çıkmıştır. Bu hızlı değişimin arka planında ülkeye akın eden misyonerlerin çeşitli yöntemlerle yapmış olduğu faaliyetler, modernleşme süreci, ekonomik ve siyasi sebeplerden ötürü ülke yöneticilerinin misyonerlere olan bakış açısının değişmesi yatmaktadır. Bu çalışmada, Protestan misyonerlerin nasıl ve hangi aşamalar sonucunda başarılı oldukları sosyal, siyasi ve tarihsel açıdan incelenmektedir. Bu açıdan Etiyopya Ortodoks Tevhid Kilisesi’nin karakteristik yapısı, misyonerlik faaliyetlerinin ne zaman başladığı, Etiyopya Kilisesi’nin misyonerler ve misyonerlik faaliyetlerine karşı tavrı, misyonerlik faaliyetlerinin önünü açan unsurlar, misyonerlik faaliyetlerinde sağlık ve eğitim faktörlerinin rolü, Pentekostalizm’in ülke içindeki seyri gibi tartışmalar makalenin konusunu oluşturmaktadır. Ayrıca bu çalışma, Etiyopya’daki misyonerlik faaliyetleri açısından 1950’li yılları esas almaktadır. Öncesinde misyonerlik faaliyetleri olmasına rağmen niçin başarılı olunamadığı, sonrasında hangi sebep ve motivasyonlardan dolayı ve hangi Protestan grubunun önemli derecede artış gösterdiği ele alınmaktadır. Ülkede faaliyet gösteren bütün misyoner cemiyetlerin dahil edilmesi bu çalışmanın sınırları içinde mümkün olmadığı için bu araştırmanın kapsamı Protestanlık içinde öne çıkan isimlerden bazıları verilerek Pentekostalizm ile sınırlandırılmıştır. Etiyopya’da Protestan misyonerliğinin mahiyetini ortaya koyabilmek için tarihsel süreç içinde teşekkülüne ve gelişimine değinmek gerekmektedir. Bu bağlamda özellikle Etiyopya Kilisesi’nin karakteristik yapısı hakkında bilgi verilmiş ve bu amaçla da betimleyici metot kullanılmıştır. Devamında bireysel olarak ortaya çıkan misyonerlik faaliyetleri tarihi bir seyir içinde incelenmiş, birbirleri arasındaki benzerlik ve farklılıklar ele alınmıştır. Söz konusu faaliyetlerin tarihsel analizi yapılırken misyonerlerin tutum ve tavırları, Etiyopya Kilisesi ile olan ilişkileri, sanayi ve teknoloji alanındaki gelişmelerin misyonerlik faaliyetlerindeki etkisi gibi hususlar analitik bir gözle incelenmiştir. Bu çalışmanın hazırlanış aşamasında literatür taraması yapılmış, konu ile alakalı kitap ve makale gibi eserler incelenmiş ve konunun kavramsal çerçevesi oluşturulmuştur. Etiyopya, fiili olarak sömürgeleştirilmemiş bir ülke olması hasebiyle ülkedeki misyonerlik faaliyetleri kıtadaki diğer ülkelere nazaran farklı yönlere sahiptir. Zira Etiyopya’da misyonerler sömürge güçlerinin sağladığı ekonomik ve siyasi güçten mahrum bir şekilde faaliyetlerini yürütmek zorunda kalmıştır. Bu durum ise misyonerlerin başarı oranlarını doğrudan etkilemiştir. Ancak 20. yüzyıldan sonra yaşanan tecrübe ve değişimlerin misyonerlik faaliyetleri üzerinde kalıcı etkisi olmuştur. Bu cihetle misyonerler Afrika kıtasında sadece sömürge gücü ile değil teknolojik ve sanayi alanındaki gelişmelerle de faaliyetlerini etkin bir şekilde yapmaya başlamıştır. Hatta ikinci faktörün etkisinin birincisinden daha güçlü olduğu iddia edilebilir. Zira elli yıllık bir zaman diliminde bile Protestan nüfusun yüzde birlerden yirmilere çıkması bu iddiayı kanıtlar niteliktedir. Bu açıdan çalışmamız misyonerlik faaliyetlerinde özellikle sağlık ve eğitim faktörüne dikkat çekmekte, ayrıca Afrika kültürü ve geleneğine yakın olan Pentekostalizm’e vurgu yapmaktadır.

Narrowband power line communication has become a major focus of research with its applicability extending to residential facilities. This article examines data transmission over flexible AC power line cables stretching from the elevator control panel to the cabin. In previous studies, there has been no significant attempt to characterize the Power Line Communication (PLC) channel and noise of an elevator system. In this article, a vector network analyzer and mixed domain oscilloscope along with coupling devices are used to conduct power line channel noise and response measurements for an elevator system. The acquisition of channel transfer functions corresponding to both idle and moving states of the elevator is carried out for the 9 kHz–500 kHz frequency range. A mathematical model is provided for the average frequency response of the PLC channel. The main source of noise is the inverter that supplies power to the motor. The channel exhibits notable variations for frequencies less than 281 kHz while staying relatively consistent for the 281 kHz to 500 kHz frequency range as the elevator changes from idle to moving state. Reed Solomon and convolutional codes along with interleaving to spread erroneous bits are utilized in this work to simulate an orthogonal frequency division multiplexing transceiver. By systematically selecting subcarrier frequencies that do not overlap with the harmonic noise components, simulation results have demonstrated reliable data recovery for signal-to-noise ratio greater than 4 dB.

An electrical plan drawing–sometimes called a wiring diagram or electrical drawing–consists of lines and symbols. Electrical plan drawings are prepared on 2D architectural floor plans using Computer-Aided Design and/or Drafting (CAD) programs. The placement/drawing of electrical power symbols–such as sockets, lights, and switches–is the first step of an electrical plan drawing. For this purpose, a smart system has been developed in this study to automatically draw/place electrical power symbols in appropriate locations. The system is based on the detection and classification/recognition of furnishing (decorative) symbols in the floor plans. We have created a furnishing symbol dataset drawing on dozens of architectural plan drawings that contain symbols of the most commonly used tools in floor plans, such as furniture, appliances, plumbing, doors, and windows. We used a Deep Convolutional Neural Network (D-CNN) with transfer learning–Inception-v3 model– to classify furnishing symbols. We tested the model on 20 real floor plans and achieved a very satisfactory accuracy of 97.05% in furnishing symbol classification. The symbol drawing step, which is the first step of drawing the electrical plan, was automated using the work developed, thus achieving the aim of saving time and labour. Experimental studies show the effectiveness of the proposed automated system.

This study examines the rural tourism experiences of the tourists visiting the Lake Salda and Lisinia Doğa of Burdur province, which has shown an important development in rural tourism in recent years. Data has been collected from the comments of the tourists who have visited Lake Salda and Lisinia Doğa on the online platform www.tripadvisor.com and content analysis has been used to examine data. Frequency and word cloud analysis have been performed in MAXQDA Analytics Pro program. Research results indicate that rural tourism experiences are versatile and heterogeneous, based on authentic (unique) experience, activity experience, local food & beverage experience, accommodation experience, local souvenir experience, testify wildlife rehabilitation, transportation experience and negative experience. Tourism businesses may be offered to design and manage their products and services for rural tourism experiences.

Garîbü’l-Kur’ân, âyetlerdeki anlamı izaha muhtaç kelimeleri konu edinen Kur’ân ilimlerinde bir bilgi alanıdır. Bu ilmin temelini atarak ilk mümessili olan kişi, bilgin sahâbî Abdullah b. Abbas’tır (ö. 68/687-88). En seçkin eseri ortaya koyarak doruk noktasına taşıyan kişi ise Râgıb el-İsfahânî’dir (ö. 502/1108-1109). Makalede, iki müfessirin eserleri çerçevesinde alana katkıları, garîb kelimeyi açıklarken âyet, hadis, şiir gibi istişhad unsuru olarak kullandıkları yaklaşımları esas alınmak suretiyle ortak ve farklı yönleri mukayeseli olarak tespit edilmeye çalışılacaktır. Böylelikle bidayeti ve yaklaşık beş asır sonraki dönemi bakımından garîbü’l-Kur’ân alanında yaşanan gelişim ve kelimelerin açıklanmasındaki değişim, müşahhas örneklerle ortaya konulmaya çalışılacaktır. Bu vesileyle başlangıçta sayı ve kapsam olarak sınırlı kelimeyi içeren garîbü’l-Kur’ân ilminin ilerleyen süreçte bütün Kur’ân kelimelerini kapsar hale gelmesine yol açan ana etkenlere kısmen temas edilecektir. İbn Abbas ve İsfahânî’yi garîbü’l-Kur’ân çerçevesinde mukayeseli bir biçimde ele alan yönüyle çalışmanın özgün bir nitelik arz ettiği değerlendirilmektedir. Çok sayıda İslâmî ilimler alanında etki ve telîfi bulunan her iki bilginin yalnız garîbü’l-Kur’ân’ları esas alınmak suretiyle çalışmanın kapsamı sınırlandırılmıştır. Konunun özelliği gereği ağırlıklı olarak deskriptif ve mukayeseli tahlil yöntemleri kullanılmıştır.

Mavi Büyüme Stratejisi balıkçılık, deniz turizmi, mavi enerji, deniz tabanı madenciliği ve mavi biyoteknoloji gibi çeşitli denizcilik faaliyetlerini ayrı ayrı yönetmek yerine birlikte yönetilmesinden daha fazla değer elde edilebileceği felsefesine dayanmaktadır. Bu çalışmanın amacı, mavi büyüme stratejisini Türkiye’nin kıyı ve deniz turizmi açısından değerlendirmektir. Araştırma nitel araştırma yöntemlerinden arşiv/doküman taraması tekniğine dayanmaktadır. Sonuç olarak, 2019 yılında Türkiye’nin, Akdeniz ve Ege Denizi kıyılarına yaklaşık 20 milyon turist gelmiş ve deniz turizmi tesisleri 300.000 kurvaziyer turisti ağırlamış ve deniz turizminden 5,9 milyar $ gelir elde etmiştir. Türkiye’de mavi büyüme sektörlerinden deniz turizmi ve su ürünleri yetiştiriciliği faaliyetleri yapılırken, mavi enerji, mavi bioteknoloji ve deniz tabanı madenciliği alanında sınırlı faaliyetler söz konusudur. Yarımada şeklindeki Türkiye, mavi büyüme potansiyelinden daha fazla ekonomik katma değer ve istihdam sağlayabilir.

This study presents a new sex pheromone trap for European Grapevine Moth, Lobesia botrana, to monitor its field population without direct field observations in Turkey. The study was conducted in Hadim and Taşkent (Konya) districts in 2016. Viticulture has been carried out for many years in both districts where the maximum vineyards are located. Due to the distance to the center and the difficulty of transportation to these districts, there has not been conducted any detailed studies until today. Therefore, this study about L. botrana, which is harmful in these vineyards, was planned. In order to monitor the pest population, the cameras were placed onto the pheromone traps, and it was tried to get information about the population development of the pest via internet without going to the vineyards. For this purpose, four locations in Hadim and one location in Taşkent district were selected and one pheromone trap was established in each location before the bud burst in vineyards. The camera was placed on each pheromone trap and was linked with vMEyeIPC program via internet. The time of first adult flight and the population development of adult L. botrana were determined. The first adult flight began at the beginning (7/4/2016) and in the middle of April (15/4/2016) in Hadim and Taşkent, respectively. The number of offspring and the maximum number of pest individuals caught in traps were recorded in vineyards in both districts.

İslam'da musikinin hükmü konusunda bugüne kadar lehte ve aleyhte pek çok eser kaleme alınmıştır. bu eserler içerisinde İbn Hazm el- Endülisi'nin konuya yaklaşımı musiki ile ilgili rivayet edilen hadislere bakışı, konuyla ilgili çalışma yapan diğer alimlerden çok farklıdır. ancak İbn Hazm'ın musiki yönüyle ilgili Türkçe esere rastlanmamıştır. Makale bu konudaki eksikliği görerek kaleme alınmış; çalışmada, İbn Hazm'ın konuyla ilgili müstakil çalışması olan "Risaletün fi Ğınai'l-Mülhi e Mübahun hüve em Mahsurun" (Eğlendirici musikinin haram mı yoksa mübah mı olduğuna dair bir risale) adlı eseri incelenmiştir. Müellif hakkında kısaca bilgi verilerek onun musiki ile ilgili hadislere bakışı incelenmiştir.

Tarihte her topluluğun kendine özgü dini gelenekleri olmuştur. Gelenekler milletlerin varlığını sürdürebilmesi ve kültürlerinin bekası açısından çok önemlidir. İslâm geleneği içerisinde en önemli uygulamalardan birisi de Kırk Hadis demeti oluşturma çabalarıdır. Söylediği, uyguladığı ve tasdik ettiği her faaliyeti hadis olarak zikredilen Hz. Muhammed s.a.v. ’in dini günlük hayatla ilgili hadislerinden kırk tanesini seçerek bir hadis demeti oluşturup bunu insanların öğrenmesine yardımcı olacak şekilde düzenleme ve sunma gayreti İslâm toplumlarında yüzyıllar boyu devam edegelmiştir. “ Ümmetimden kim kırk hadis hıfzederse, Allah onu âlimler ve fakihler arasında diriltsin.” Şeklinde ifadeler dini kaynaklarda geçmektedir. Dolayısıyla biz de bu çalışmamızda özellikle mûsiki ve eğlenmeye dair kırk hadisi düzenleyerek bir buket oluşturmaya çalıştık. Hemen hemen her branştan ve meslekten çeşitli âlimlerce bu konu ele alınmış ve ilgili oldukları alanda kırk hadis demeti oluşturmuşlardır. Araştırmalarımızda mûsikî alanında kırk hadis çalışmasının yapılmadığını gördük. Bu yüzden mûsikî alanlında kırk hadis toplayarak ve şerh ederek bu çalışmayı oluşturmaya karar verdik

Religious rituals such as the tradition of visiting shrines and tree cult have been in existence for thousands of years in the different regions of Anatolia. Clear examples of such still- existing rituals are the visits paid to “Türbe” Quarter in Yaka Village of Fethiye District in the Province of Muğla and making wishes and hoping for heals from the holly tree. The data, which has been obtained from the research that has been launched the scope of a TÜBİTAK (The Scientific and Technological Research Council of Turkey) Project in 2011 and the study that have been on the drawing of historical ruins and documentations in Türbe Quarter, have been transferred to the Geographic Information Systems. Although the certain date of the historical ruins is not exactly known, it has been thought that the ruins may belong to Menteşoğulları Seigniory of Türkmen Tribes. In the scope of project, the study have also been done to determine the age of 3 Cupressus sempervirens var. horizantalis (cypress) that are within the ancient ruins and their environs. The oldest one has been understood to be about 910 years old. As a result, in this study, historical ruins and monumental trees within the boundaries of Tlos Antic City being one of the important settlements in the Lycian Region have been assessed in termsof cultural landscape.

Background: The cingulate gyrus (CG), a brain structure above the corpus callosum, is recognised as part of the limbic system and plays numerous vital roles. However, its full functional capacity is yet to be understood. In recent years, emerging evidence from imaging modalities, supported by electrical cortical stimulation (ECS) findings, has improved our understanding. To our knowledge, there is a limited number of systematic reviews of the cingulate function studied by ECS. We aim to parcellate the CG by reviewing ECS studies., Design/methods: We searched PubMed and Embase for studies investigating CG using ECS. A total of 30 studies met the inclusion criteria. We evaluated the ECS responses across the cingulate subregions and summarised the reported findings., Results: We included 30 studies (totalling 887 patients, with a mean age of 31.8±9.8 years). The total number of electrodes implanted within the cingulate was 3028 electrode contacts; positive responses were obtained in 941 (31.1%, median percentages, 32.3%, IQR 22.2%-64.3%). The responses elicited from the CG were as follows. Simple motor (8 studies, 26.7 %), complex motor (10 studies, 33.3%), gelastic with and without mirth (7 studies, 23.3%), somatosensory (9 studies, 30%), autonomic (11 studies, 36.7 %), psychic (8 studies, 26.7%) and vestibular (3 studies, 10%). Visual and speech responses were also reported. Despite some overlap, the results indicate that the anterior cingulate cortex is responsible for most emotional, laughter and autonomic responses, while the middle cingulate cortex controls most complex motor behaviours, and the posterior cingulate cortex (PCC) regulates visual, among various other responses. Consistent null responses have been observed across different regions, emphasising PCC., Conclusions: Our results provide a segmental mapping of the functional properties of CG, helping to improve precision in the surgical planning of epilepsy., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background/aim: Obstructive sleep apnea (OSA) is a common sleep-related breathing disorder in children. Determination of risk factors for the development of OSA is essential for early diagnosis and treatment of the disease and decreases the risk of negative consequences. This study aimed to investigate the predictive values of Mallampati score, tonsillar size, and BMI z-score in the presence and severity of OSA in children., Materials and Methods: This prospective cross-sectional study included 114 children with OSA symptoms. All children were assessed by BMI z-score, Mallampati score, and tonsillar size and underwent overnight polysomnography. They were consecutively selected and assigned to 4 groups as follows: Group 1 included normal-weight with a low Mallampati score; Group 2 involved normal-weight with a high Mallampati score; Group 3 included obese with a low Mallampati score; and Group 4 involved obese with a high Mallampati score., Results: Of the 114 included children, 58 were female and 56 were male, with a mean age of 13.1 ± 2.9 years. OSA frequency and apnea-hypopnea index were significantly higher in group 4 compared with other groups (p = 0.003 and p < 0.0001, respectively), whereas average and minimum spO 2 were significantly lower (for both, p = 0.001). Mallampati score and BMI z-score were found to be significant for predicting OSA (odds ratio = 4.147, 95% CI: 1.440-11.944; p = 0.008 and odds ratio = 1.760, 95% CI: 1.039-2.980; p = 0.035, respectively). Among OSA patients, the Mallampati score, tonsillar size, and BMI z-score were found to be significant for predicting OSA severity (odds ratio = 4.520, 95% CI: 1.332-15.335, p = 0.015, odds ratio = 9.177, 95% CI: 2.513-33.514, p = 0.001, and odds ratio = 2.820, 95% CI: 1.444-5.508; p = 0.002, respectively)., Conclusion: The coexistence of the Mallampati score and BMI z-score significantly increases the presence of OSA in children. Mallampati score, tonsillar size, and BMI z-score are promising parameters for predicting OSA severity., (© TÜBİTAK.)

Purpose: It has been suggested that the cause of the balance disorder seen in adolescent idiopathic scoliosis (AIS) originates from the central nervous system. However, the extent of the balance problem and the dysfunction of which part of the central nervous system has not been investigated in detail. This study aimed to correlate the values obtained by balance analysis and cerebellum volume measurement in female individuals with AIS with healthy individuals., Methods: Cerebellum volume was calculated via the cloud-based software " https://volbrain.upv.es " using brain magnetic resonance images of 27 healthy and 26 individuals with AIS. The duration of stay in the test positions, the movement strategy used during this time and the amount of postural sway were analyzed by using a computer-assisted force platform and compared statistically., Results: Significant differences were found between the AIS and control groups in cerebellum total volume, vermis cerebelli volume (cm 3 ), and trunk oscillation velocity (mm/s) parameters (p < 0.05). Cerebellum and vermis cerebelli volumes were found to be lower and trunk oscillation velocity was found to be greater in patients with AIS., Conclusion: Balance problems in patients with AIS are correlated with decreased cerebellum volume and increased trunk oscillation velocity., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Glossokinetic artifact (GKA) is a well-known scalp EEG artifact characterized by deflections within the delta to low-theta frequency bands and dynamic polarity typically attributed to the direction of tongue movement. This study aims to investigate intracranial EEG correlations of scalp-GKA. If the tongue is a dipole, per the conventional view, then volume-conducted deflections are expected in the nearest frontal intracranial EEG contacts., Materials and Methods: Simultaneous scalp and intracranial EEG recordings were evaluated in five consecutive medically resistant epilepsy patients at Yale Epilepsy Center in 2017 and 2018, who had classic GKA deflections on scalp EEG. The EEG was sampled at 2,048 to 4,096 Hz and analyzed visually, using a reference placed in the diploic space or over the convexity, and confirmed quantitatively by a statistical framework. Ten GKA deflections were analyzed per case., Results: The medians of age at the time of recording, contacts per case, and amplitude of scalp GKA deflections were 35 years (range: 20-41 years), 171 contacts (range: 165-241 contacts), and 56 μV (range: 51-72 μV), respectively. There were no slow discharges in the frontal intracranial EEG contacts synchronized with the scalp GKA, either in the delta (1-3 Hz) or in the sub-delta (0.1-1 Hz) bands. However, the expected physiologic attenuation of alpha and beta rhythms and the emergence of high-gamma activity were observed over the peri-Rolandic regions in the invasive recordings., Conclusions: The traditional view of the tongue as a dipole generator of scalp GKA is simplistic and does not account for the findings reported herein. The tongue most probably shunts other scalp and soft-tissue currents. Knowledge of tongue potentials is of interest in the education and the design of tongue-computer interfaces., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 by the American Clinical Neurophysiology Society.)

Purpose: Walker-Warburg syndrome (WWS) is a rare autosomal recessive disorder characterized by congenital muscular dystrophy and severe brain and eye malformations. This study aims to analyze genotype-phenotype correlations in WWS with a novel cytidine diphosphate-l-ribitol pyrophosphorylase A ( CRPPA ) mutation in different clinical manifestations., Case Description: We report a girl with a presentation of multiple brain and ocular anomalies. Her ophthalmological evaluation showed a shallow anterior chamber, cortical cataract, iris hypoplasia, persistent hyperplastic primary vitreous in the right eye, punctate cataract, iris hypoplasia, primary congenital glaucoma, and a widespread loss of fundus pigmentation in the left eye. She was hypotonic, and her deep tendon reflexes were absent. Laboratory investigations showed high serum levels of serum creatine kinase. Brain magnetic resonance imaging demonstrated hydrocephalus, agenesis of the corpus callosum, retrocerebellar cyst, cerebellar dysplasia and hypoplasia, cobblestone lissencephaly, and hypoplastic brainstem. Whole exome sequencing revealed a novel homozygous nonsense mutation in the first exon of the CRPPA gene (NM&#95;001101426.4, c.217G>T, p.Glu73Ter)., Conclusions: The study findings expand the phenotypic variability of the ocular manifestations in the CRPPA gene-related WWS. Iris hypoplasia can be a part of clinical manifestations of the CRPPA gene-related WWS. The uncovering of the genes associated with ocular features can provide preventative methods, early diagnosis, and improved therapeutic strategies.

Background and Purpose: Dueto motion artifacts, optic nerve (ON) findings of idiopathic intracranial hypertension (IIH) can easily be overlooked on T2-weighted (T2w) turbo spin-echo sequence. This study aimed to investigate the contribution of the apparent diffusion coefficient (ADC) map derived from the interleaved multi-shot (IMS) echoplanar imaging (EPI) to the ON findings of IIH in children., Methods: MRIs of 42 pediatric patients aged 3-17 years diagnosed with definite IIH according to modified Dandy criteria were retrospectively re-evaluated, between April 2018 and January 2021. Forty-two age- and sex-matched subjects with no IIH symptoms and reported as normal were included as a control group., Results: ON sheath distance (ONSD) on the ADC map (p = .005) and vertical tortuosity (p = .030) were significant single MRI parameters for predicting IIH. Other single parameters were not statistically significant. Flattening of the posterior sclera (FPS) and ON protrusion (ONP) were observed on ADC maps more frequently than T2w (42.8% vs. 19% and 19% vs. 4.7%, respectively). From combined MRI parameters, the presence of at least one of ONP, FPS, or ONSD on ADC maps (p = .001) showed greater significance than the presence of T2w (p = .048). The predictive values of other MRI findings evaluated together were not statistically significant (p > .05)., Conclusions: This study's results show that due to the short readout time and less sensitivity to motion, the ADC map obtained from IMS-EPI can contribute to orbital findings of IIH, in addition to T2w., (© 2021 American Society of Neuroimaging.)

Background: Agenesis of the corpus callosum (ACC) is the most frequent commissural malformation of the brain. It continues to be an important cause of the pregnancy termination associated with the central nervous system (CNS)., Objective: The aim of the study is to provide a comprehensive assessment of fetuses with diagnosis of complete ACC, as well as postnatal neurodevelopmental outcomes., Methods: The data of 75,843 fetuses were screened for evaluation of complete ACC between 2003 and 2017, and a total of 109 cases with complete ACC were included in the study. ACC was considered isolated when no additional anomalies were detected, and ACC was considered complex when additional anomalies were present., Results: The prevalence of complete ACC was 9.4 per 10,000 live births, and the incidence was ranged from 1.8 to 16.6 per 10,000 person-years. Patients with isolated ACC had a significantly higher survival when compared with patients with complex ACC (97.4%, n = 38/39 vs. 68.8%, n = 22/32, P = 0.001).The most important cause of death were congenital heart disease and/or respiratory failure during neonatal period. Developmental and intellectual disabilities were significantly higher in the complex ACC cases (P < 0.001). Postnatal neurodevelopmental outcomes were completely normal in 79.4% of cases with isolated ACC., Conclusions: Isolated complete ACC is usually associated with a favorable outcome. The most important prognostic factors are the presence or absence of associated congenital anomalies.

Ischemic stroke is a significant health condition, whose frequency in childhood is increasing day by day. Although many factors are effective in development of the stroke, it has been showed that individuals having risk factors have a genetic predisposition. The aim of the study is to determine whether distinct genetic mutations are risk factors for children with history of ischemic stroke. Our sample data is taken from 58 patients (29 male and 29 female) who applied our hospital between 2012 and 2016 with diagnosis of acute or chronic arterial stroke and from 70 healthy children (32 male and 38 female) with similar particularities in the sense of age and sex, who have not any chronical disease. Blood samples are taken from each child participated in the study to conduct genetic analysis. It has been examined whether a mutation exists in gene locations of CDKN2B-AS1 (Rs2383206), HDAC9 (Rs11984041), NINJ2 (Rs12425791), NAA25 (Rs17696736). Moreover, whether there are significant difference between patient and control group has been investigated. In the genetic analysis of patients and control groups, no significant difference has been found for any of the genes. Mutations in gene locations of CDKN2B-AS1 (Rs2383206), HDAC9 (Rs11984041), NINJ2 (Rs12425791), NAA25 (Rs17696736) are not risk factors for ischemic stroke in childhood. However this study showed us, the patients who inherit CDKN2B-AS1 and HDCA9 gene mutations had poor prognosis. However, this study should be replicated for a wider sample of patient population.

Objective: To identify causes of the autosomal-recessive malformation, diencephalic-mesencephalic junction dysplasia (DMJD) syndrome., Methods: Eight families with DMJD were studied by whole-exome or targeted sequencing, with detailed clinical and radiological characterization. Patient-derived induced pluripotent stem cells were derived into neural precursor and endothelial cells to study gene expression., Results: All patients showed biallelic mutations in the nonclustered protocadherin-12 (PCDH12) gene. The characteristic clinical presentation included progressive microcephaly, craniofacial dysmorphism, psychomotor disability, epilepsy, and axial hypotonia with variable appendicular spasticity. Brain imaging showed brainstem malformations and with frequent thinned corpus callosum with punctate brain calcifications, reflecting expression of PCDH12 in neural and endothelial cells. These cells showed lack of PCDH12 expression and impaired neurite outgrowth., Interpretation: DMJD patients have biallelic mutations in PCDH12 and lack of protein expression. These patients present with characteristic microcephaly and abnormalities of white matter tracts. Such pathogenic variants predict a poor outcome as a result of brainstem malformation and evidence of white matter tract defects, and should be added to the phenotypic spectrum associated with PCDH12-related conditions. Ann Neurol 2018;84:646-655., (© 2018 American Neurological Association.)

Aims: The aim of this study is to evalute the effects of methylphenidate and atomoxetine treatments on electroencephalography (EEG) signals in volunteer children diagnosed with Attention Deficit and Hyperactivity Disorder(ADHD)., Methods: The study contained 40 children all of whom were between the ages of 7 and 17. The participants were classified into two groups as ADHD (n=20), which was in itself divided into two groups as ADHD-MPH (ADHD- Metylphenidate treatment) (n=10) and as ADHD-ATX (ADHD-Atomoxetin treatment) (n=10), and one control group (n=20). Following the first EEG recordings of the ADHD group, long-acting methylphenidate dose was applied to one ADHD group and atomoxetine dose was applied to the other ADHD group. The effect of optimal dosage is about for 4-6 weeks in general. Therefore, the response or lack of response to the treatment was evaluated three months after the beginning of the treatment.After methylphenidate and atomoxetine drug treatment, in order to obtain mean and maximum power values for delta, theta, alpha and beta band, the EEG data were analyzed., Results: The EEG power spectrum densities in all the bands yielded similar findings in both methylphenidate and atomoxetine. Although statistically significant frequency values of the electrodes were amplitude and maximally varied, in general, they appeared mostly at both frontal and temporal regions for methylphenidate and atomoxetine., Conclusion: Especially, after atomoxetine treatment, Quantitative Electroencephalography (QEEG) rates at frontal area electrodes were found statistically more significant than methylphenidate QEEG rates. What has been researched in this study is not only whether QEEG is likely to support the diagnosis, but whether changes on QEEG by treatment may be related to the severity of ADHD as well.

Objectives: Wilson's disease (WD) is characterized with the accumulation of copper in the liver and brain. The objective of this study is to quantitatively measure the susceptibility changes of basal ganglia and brain stem of pediatric patients with neurological WD using quantitative susceptibility mapping (QSM) in comparison to healthy controls., Methods: Eleven patients with neurological WD (mean age 15 ± 3.3 years, range 10-22 years) and 14 agematched controls were prospectively recruited. Both groups were scanned on a 1.5 Tesla clinical scanner. In addition to T 1 - and T 2 -weighted MR images, a 3D multi-echo spoiled gradient echo (GRE) sequence was acquired and QSM images were derived offline. The quantitative measurement of susceptibility of corpus striatum, thalamus of each hemisphere, midbrain, and pons were assessed with the region of interest analysis on the QSM images. The susceptibility values for the patient and control groups were compared using twosample t-test., Results: One patient with WD had T 1 shortening in the bilateral globus pallidus. Another one had hyperintensity in the bilateral putamen, caudate nuclei, and substantia nigra on T 2 -weighted images. The rest of the patients with WD and all subjects of the control group had no signal abnormalities on conventional MR images. The susceptibility measures of right side of globus pallidus, putamen, thalamus, midbrain, and entire pons were significantly different in patients compared to controls (P < 0.05)., Conclusion: QSM method exhibits increased susceptibility differences of basal ganglia and brain stem in patients with WD that have neurologic impairment even if no signal alteration is detected on T 1 - and T 2 -weighted MR images.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Background  Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis. Objective  The purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings. Materials and Methods  Our medical records database for 2003 to 2014 was screened retrospectively. In total, 104 patients with FMF were identified, 22 of whom had undergone neurological examination for CNS symptoms. Results  Neurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), tremor in 2 patients (9.1%), and multiple sclerosis in 1 patient (4.5%). The most common MEFV gene mutation was homozygous M694V (40.9%). Conclusions  Patients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF., (Georg Thieme Verlag KG Stuttgart · New York.)

Traditionally, Morus rubra L. (Moraceae) (red mulberry) and Cornus mas L. (Cornacea) (cornelian cherry) fruits are eaten fresh and are also used in marmalades, juices, jam, natural dyes in Turkey and are believed to have beneficial effects in case of multiple health issues such as antipyretic, diarrhea and intestinal parasites. However, the effects of M. rubra and C. mas on epilepsy has not been known. This study evaluates the effects of M. rubra and C. mas extracts on penicillin-induced epileptiform activity. Sixty Wistar rats randomly divided into ten groups (n=6): control, sham, penicillin, penicillin+M. rubra extract (2.5, 5, 10, 20 mg/kg) and penicillin+C. mas extract (2.5, 5, 10 mg/kg). Epileptiform activity was induced by using penicillin (500 IU, i.c.) and electrocorticogram records (150 min) were obtained. Also, biochemical analysis in blood samples were evaluated. According to the electrocorticogram analysis, the effective dose was detected as 10 mg/kg for both C. mas and M. rubra. This dose decreased the spike frequencies of convulsions while amplitude wasn't changed by both substances. In erythrocyte studies, there were significant differences regarding nitric oxide in the control, sham and penicillin groups. There were significant differences regarding malondialdehyde in all groups. In the plasma, there were significant differences among groups regarding xanthine oxidase in the penicillin‑C. mas and penicillin‑M. rubra groups. There were differences regarding malondialdehyde in the penicillin-C. mas and M. rubra-C. mas groups. Both extracts reduced the frequency of epileptiform activity. After administration of the extracts malondialdehyde levels decreased also in both erythrocytes and plasma.

Background: The skin is one of the most affected organs in tuberous sclerosis complex and angiofibromas are seen in almost 80% of such patients. These benign tumors impose a great psycho-social burden on patients., Objective: The aim of the study was to evaluate the effectiveness and tolerability of topical sirolimus for facial angiofibromas in patients with tuberous sclerosis complex., Methods: This was a prospective, single-blinded, cross-over study which involved twelve patients. We investigated the effect and safety of topical 0.1% sirolimus, which was obtained by crushing sirolimus tablets and mixing it with petrolatum. The patients were asked to apply the cream to one side of their face, and vaseline to the other side. The effect of topical sirolimus was evaluated using the "facial angiofibroma severity index.", Results: There was a significant improvement in the redness and extension of the tumors on the sides to which the active ingredient was applied. Some side effects such as itching and irritation occurred in three patients, which were treated with topical hydrocortisone cream., Conclusion: Topical sirolimus appears to be a promising, fairly well tolerated treatment for facial angiofibromas in patients with tuberous sclerosis complex. Although its efficacy diminishes with time, repetitive usage is effective.

The aim of this study was to investigate the relationship between autism spectrum disorders development and exposure to mono-(2-ethylhexyl)-phthalate (MEHP), di-(2-ethylhexyl)-phthalate (DEHP), and bisphenol A (BPA), 1 of the endocrine disruptors, among phthalates. The study included 48 children with autism spectrum disorder (27 boys, 21 girls) and 41 healthy subjects (24 boys, 17 girls) as controls. Serum MEHP, DEHP, and BPA levels were measured by using high-performance liquid chromatography. Children with autism spectrum disorder had significantly increased serum MEHP, DEHP, and BPA concentrations (0.47 ± 0.14 µg/ml, 2.70 ± 0.90 µg/ml, 1.25 ± 0.30 ng/ml) compared to healthy control subjects (0.29 ± 0.05 µg/ml, 1.62 ± 0.56 µg/ml, 0.88 ± 0.18 ng/ml) respectively (P = .000). The fact that higher serum MEHP, DEHP, and BPA were found levels in the autism spectrum disorder group compared to healthy controls suggests that endocrine disruptors may have a role in the pathogenesis of autism spectrum disorders., (© The Author(s) 2015.)

Background: Moyamoya disease is an uncommon, progressive, and occlusive cerebrovascular disorder, predominantly affecting the terminal segment of the internal carotid arteries and its main branches. This occlusion results at the formation of a compensatory collateral arterial network (moyamoya vessels) developing at the base of the brain. The c.14576G>A variant in ring finger protein 213 (RNF213) was recently reported as a susceptibility gene for moyamoya disease., Methods: We describe two Turkish pediatric siblings with moyamoya disease born to consanguineous, unaffected Turkish parents., Results: The first patient (proband) is a 2-year-old boy who presented with afebrile focal seizures, moderate psychomotor retardation, paresis in the left upper and lower extremity, multiple infarctions of the brain, stenosis of the bilateral internal carotid artery and the middle cerebral artery, and stenosis of the right posterior cerebral artery. The second patient is a 10-year-old girl who is an elder sister of proband. She showed normal psychomotor development, millimetric signal enhancement without diffusion limitation of the brain, and stenosis of the bilateral internal carotid artery., Conclusion: We herein report pediatric sibling patients of moyamoya disease who have homozygous wild-type c.14576G>A variant in RNF213, showing different clinical course and disease severity. This is the first report of pediatric siblings with moyamoya disease from Turkey validating the genetic background of most frequent variant in East Asian patients with moyamoya disease.

Aim: Wilson's disease is an autosomal recessive disorder of copper metabolism which leads to copper overload in different tissues of the body. The aim of this study was to present the neurologic features of Wilson's disease and to assess the clinical course of neurological findings in children receiving anti-copper treatment., Material and Methods: Twelve children with a diagnosis of Wilson's disease and findings of central nervous system involvement who were followed up in the Department of Pediatric Neurology and Pediatric Gastroenterology of the School of Medicine at Erciyes University were enrolled in the study., Results: The study cases consisted of five boys (42%) and seven girls (58%). The mean age at the time of diagnosis was 9.9±3.4 years (5-15 years). The mean duration of follow-up was 49.0±36.4 months (15-128 months). Neurological findings at presentation included headache in seven cases (58%), tremor in seven cases (58%), dystonia in three cases (25%), ataxia in two cases (17%), dizziness in two cases (17%), numbness in the hands and acute weakness in one case (8%) and syncope in one case (8%). Headache, dizziness, syncope, numbness in hands and acute weakness symptoms resolved completely within six months after receiving treatment. Movement disorders either decreased or remained stable in seven of the eight cases. However, one patient developed progressively worsening dystonia despite to all treatments., Conclusions: Wilson's disease can be manifested with signs and symptoms of central nervous system in the childhood. Wilson's disease should be considered in all children presenting with movement disorders. A complete neurological assessment should be carried out in all cases with Wilson's disease.

Introduction: Syncope is one of the most common clinical problem in children. This disorder is characterized by transient, spontaneously self-terminating loss of consciousness with brief duration and complete recovery. This situation is usually alarming for the families of patients. The mechanism of syncope is transient global brain hypoperfusion to levels below those tolerated by cerebrovascular autoregulation. Syncope can occur with many different etiologies in the pediatric population., Classification: Syncopes are divided into three major categories as neurally mediated syncope, cardiovascular-mediated syncope, and non-cardiovascular syncope., Clinical Features: The major challenge in the assessment of children with syncope is that most children are asymptomatic at the time of their presentation, therefore making a careful and detailed history and a comprehensive physical examination essential in all patients. A trigger stimulus is detected in some cases, and this is an important clinical clue for the diagnosis. Cardiac causes of syncope in children are rare but can be life threatening and have the highest risk of morbidity and mortality. Misdiagnosis of epilepsy is common in patients presenting with syncope; therefore, the differential diagnosis between epileptic seizures and syncope is very important. It should be remembered that the evaluation of syncope in children is costly and diagnostic workup has a limited diagnostic yield., Conclusion: The aim of this article is to present different types of syncope and to provide new practical clinical approaches to the diagnosis, investigation, and management in the pediatric population.

Background: Gastroesophageal reflux disease (GERD) can mimic epileptic seizure, and may be misdiagnosed as epilepsy. On the other hand, GERD can be more commonly seen in children with neurological disorders such as cerebral palsy (CP); this co-incidence may complicate the management of patients by mimicking refractory seizures., Objective: The purpose of our study was to evaluate the clinical features, definite diagnoses and treatment approaches of the patients with clinically suspected GERD who were referred to the division of pediatric neurology with a suspected diagnosis of epileptic seizure. We also aimed to investigate the occurrence of GERD in children with epilepsy and/or CP., Methods: Fifty-seven children who had a final diagnosis of GERD but were initially suspected of having epileptic seizures were assessed prospectively., Results: All patients were assigned to 3 groups according to definite diagnoses as follows: patients with only GERD who were misdiagnosed as having epileptic seizure (group 1: n=16; 28.1%), those with comorbidity of epilepsy and GERD (group 2: n=21; 36.8%), and those with the coexistence of GERD with epilepsy and CP (group 3: n=20; 35.1%). Five patients (8.8%) did not respond to anti-reflux treatment and laparoscopic reflux surgery was performed. The positive effect of GERD therapy on paroxysmal nonepileptic events was observed in 51/57 (89.5%) patients., Conclusions: GERD is one of the important causes of paroxysmal nonepileptic events. In addition, GERD must be kept in mind at the initial diagnosis and also in the long-term management of patients with neurological disorders such as epilepsy and CP., (Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, life-threatening hypersensitivity drug reaction. Patients present with cutaneous rash, fever, lymphadenopathy, hematologic abnormalities with eosinophilia and atypical lymphocytes, and visceral organ involvement. The prognosis of DRESS syndrome is related to the degree of end-organ damage, and the mortality rate is approximately 10%., Case Report: We report a 9-year-old girl treated with only levetiracetam because of intracranial space occupying mass-related seizures. The patient developed pharyngitis accompanied by exudative membrane, bilateral cervical lymphadenopathy, tender hepatomegaly, skin rash, and fever after 19 days of levetiracetam therapy. Laboratory findings revealed leukocytosis, lymphocytosis with an atypical lymphocytosis, eosinophilia, thrombocytopenia, and elevated serum transaminases. Serologic studies of viruses were negative. The patient was diagnosed with DRESS syndrome and antiepileptic therapy was ceased immediately. The systemic signs and symptoms of the patient were improved after systemic steroid and antihistamine therapy. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: It is important that emergency physicians be aware of the possibility of DRESS syndrome when attending children that present with clinical viral infections. We would like to emphasize that obtaining a careful and detailed medication history is an essential part of clinical assessment for the diagnosis of DRESS syndrome., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To evaluate diffusion characteristics of tubers and white matter lesions in children with tuberous sclerosis (TS) using diffusion tensor imaging (DTI)., Materials and Methods: Eighteen children (11 male, 7 female; mean age 9.3years, age range 1-16years) with a definite diagnosis of TS were recruited in this study. Apparent diffusion coefficient (ADC), fractional anisotropy (FA), radial diffusivity (RD), and axial diffusivity (AD) values in 89 tubers and 37 white matter lesions were measured and compared with those of contralateral normal regions., Results: ADC, AD, and RD values were significantly higher and FA values were lower in lesions, than the ones measured in contralateral normal regions for tubers (P<0.001). Similarly RD values were significantly higher and FA values were lower in white matter lesions (P<0.05). ADC and AD measures were detected to increase in white matter lesions, however no statistically significant difference was observed. The increase in the mean values of RD was significantly greater than the increase in the AD values for tubers and white matter lesions (P<0.05)., Conclusion: DTI can provide valuable information about the cytoarchitectural changes in TS lesions beyond morphologic MRI findings alone., (Copyright © 2015 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Objective: Autism spectrum disorders (ASDs) are a severe group of neurodevelopmental disorders that are characterized by impairment in social communication, and imagination and social interaction. The aetiology of autism is complex, but some studies suggest autoimmunity to the central nervous system in the pathogenesis. The aim of this study is to investigate the positivity of antineuronal antibodies including anti-glutamic acid decarboxylase antibodies (anti-GAD), anti-glutamate receptor (anti-GluR) antibodies and seven types of anti-ganglioside antibodies, in children with autism., Methods: We conducted the study over a period of one year from May 2012 to December 2013. Human anti-GAD in serum were investigated with ELISA; human autoantibodies against the N-methyl-D-aspartate subtype of GluR were investigated with indirect immunofluorescence test; class IgG antibodies against the seven gangliosides were investigated with immunoblot assay., Results: Serum antineuronal antibodies were measured in 42 children (24 male, 18 female) with autism in comparison to 21 (13 male, 8 female) healthy-matched children aged between 2-12 years. There was no seropositivity of antineuronal antibodies in either of the groups., Conclusion: There is no evidence to support an association between autism and antibodies positivity of anti-GAD, anti-GluR and anti-gangliosides (Ref. 26).

Tangier disease (TD) is a rare, autosomal recessive inherited disorder caused by a mutation in the adenosine triphosphate-binding cassette transporter 1 (ABCA1) gene, which results in a decrease in plasma high-density lipoprotein (HDL) levels. Peripheral neuropathy can be seen in approximately 50% of patients with TD, which usually occurs after the age of 15 years, and is characterized by relapsing-remitting mono- or polyneuropathy or syringomyelia-like neuropathy. Herein, we report a 16-year-old female patient who was initially diagnosed with peripheral neuropathy at the age of 13 years. Whole exome sequencing was performed, and a nonsense mutation (p.Arg1817X) in ABCA1 was identified. The patient was investigated for systemic findings of TD after the genetic diagnosis was made, and low (< 5 mg/dL) levels of HDL cholesterol were detected by lipid electrophoresis. Other family members were reexamined after the diagnosis of the proband, and asymptomatic sister of the proband was diagnosed with TD. We would like to emphasize that TD should be considered in the differential diagnosis of pediatric patients presenting with peripheral neuropathy; furthermore detection of HDL levels by lipid electrophoresis is a simple but indicative diagnostic test., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: The purpose of this study was to characterize patients who were diagnosed with glucose transporter protein 1 deficiency syndrome (Glut1D), and also to assess the efficacy of ketogenic diet (KD) therapy on seizure control, cognitive functions, and other neurological disorders., Patients and Methods: We studied six unrelated patients with the classical phenotype of Glut1D, focusing on clinical and laboratory features, the KD therapy and outcome over the 25-month follow-up period., Results: Five patients became seizure-free with the onset of ketosis, and anticonvulsants were discontinued. Other neurological features such as ataxia, spasticity, and dystonia showed a less striking improvement than seizure control. There was no significant change in the intelligence quotient (IQ) level or microcephaly. In all patients, alertness, concentration, motivation, and activity resulted in a moderate improvement of variable degree. The early-onset adverse effects of KD were observed in five patients. The KD regimen failed in one patient, therefore, his diet was changed with an alternative to KD., Conclusions: Treatment with KD resulted in a marked improvement in seizures and cognitive functions but its effect appeared to be less striking on the other neurological disorders of the patients. When the classic KD is not tolerated, an alternative to KD may be helpful., (Georg Thieme Verlag KG Stuttgart · New York.)

The aim of this study was to compare the efficacy and safety of different oral chloral hydrate and dexmedetomidine doses used for sedation during electroencephalography (EEG) in children. One hundred sixty children aged 1 to 9 years with American Society of Anesthesiologists physical status I-II who were uncooperative during EEG recording or who were referred to our electrodiagnostic unit for sleep EEG were included to the study. The patients were randomly assigned into 4 groups. In groups D1 and D2, patients received oral dexmedetomidine doses of 2 and 3 µg/kg, respectively. In group C1 and C2, patients received oral chloral hydrate doses of 50 and 100 mg/kg, respectively. The induction time was significantly shorter in group C2 compared with other groups (P = .000). The rate of adverse effects was significantly higher in group C2 compared with the dexmedetomidine groups (D1 and D2; P = .004). In conclusion, dexmedetomidine can be used safely for sedation during EEG in children., (© The Author(s) 2014.)

SUMMARY - 'Idiopathic' herniation of the brain is a rare entity previously reported in 13 cases. It may be incidentally encountered in neuroimaging studies acquired for various clinical indications. We herein describe two cases of idiopathic brain herniation that were incidentally diagnosed. A 12-year-old boy presented with a six-month history of daytime sleepiness and sudden spells of sleep. Herniation of the left inferior temporal gyrus was revealed in MRI acquired with the suspicion of epilepsy. His overnight polysomnogram and multiple sleep latency tests were compatible with the diagnosis of narcolepsy. The other case, a two-year-old girl, was transferred from an outside hospital due to partial seizures with the fever. Herniation of the precuneal gyrus was encountered in MRI acquired after controlling her seizures with the initiation of phenytoin. The brain herniations of both patients were considered to be inconsistent with their medical conditions, so that they were symptom-free with only medical treatment for following three and six months, respectively. This is a rare presentation of idiopathic brain herniation as an incidental finding that accompanied narcolepsy and epilepsy. Awareness of this entity would avoid excessive surgical and medical treatments.