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Background: This study investigates the effects of erbium, chromium:yttrium-scandium-gallium-garnet (Er,Cr:YSGG) laser irradiation and hand instrumentation on the attachment of periodontal ligament (PDL) fibroblasts to periodontally involved root surfaces.Methods: Twenty-four single-rooted periodontally involved human teeth (test groups), and six healthy premolar teeth extracted for orthodontic reasons (control group) were included in this study. A total of 45 root slices were obtained from all selected teeth and assigned to the following five groups: 1) untreated healthy group (+control); 2) untreated periodontally diseased group (-control); 3) hand instrumentation group (scaled Gracey); 4) laser I, Er,Cr:YSGG laser irradiation setting-I (short pulse); and 5) laser II, Er,Cr:YSGG laser irradiation setting-II (long pulse). All of the root slices were autoclaved in phosphate buffered saline and slices were placed onto cell culture inserts. PDL fibroblasts were placed at the density of 80,000 cells on the root plate (5 x 6 mm) and incubated for 48 hours and transferred to 24-well plates. The attachment PDL fibroblasts on the root plates were observed using confocal microscopy (at 12 hours and on days 3 and 7) and scanning electron microscopy (at 12 hours and day 3). 3-(4,5-dimethyl-thiazol-2-yl)-2,5-diphenyl-tetrazolium bromide assay was performed on day 5 for PDL fibroblast survival.Results: 3-(4,5-dimethyl-thiazol-2-yl)-2,5-diphenyl-tetrazolium bromide assay shows that whereas laser-treated specimens showed a significantly higher cell density, the Gracey-treated group showed a lower cell density compared to the positive control group (P <0.05). Based on confocal microscopy, apparent reduction was observed in the attachment of PDL cells to the periodontally diseased root surfaces. In the laser and Gracey groups, cells looked well-oriented to the root surfaces. Laser-treated groups provided suitable environment for cell adhesion and growth. Laser I treatment was more favorable for the attachment of PDL compared to scaled Gracey, laser II, and even healthy root surfaces.Conclusion: The results of the study indicate that short-pulse laser setup (laser I) looks more promising regarding the attachment, spreading, and orientation of PDL cells. [ABSTRACT FROM AUTHOR]

The PLEKHG5 gene encodes a protein that induces the nuclear factor kappa B (NFjB) signaling pathway. Biallelic pathogenic variants in PLEKHG5 have been linked to distal spinal muscular atrophy IV and intermediate axonal neuropathy. We present a six-year-old girl complaining of difficulties climbing stairs and getting up from crouching. Neurological examination revealed a symmetrical proximal limb weakness with milder distal weakness. While the patellar reflexes were absent, the biceps reflexes were hyporeflexic. Electromyography showed a mild denervation pattern in the proximal muscle groups with normal nerve conduction studies. MLPA in SMN1 showed no abnormalities. Exome sequencing revealed a homozygous missense variant in PLEKHG5 (c.1399G>A: NM_020631.4 [p.Glu467Lys]). Their unaffected mother and father were both carriers. [ABSTRACT FROM AUTHOR]

The rewarding taste of food is critical for motivating animals to eat, but whether taste has a parallel function in promoting meal termination is not well understood. Here, we show that hunger-promoting agouti-related peptide (AgRP) neurons are rapidly inhibited during each bout of ingestion by a signal linked to the taste of food. Blocking these transient dips in activity via closed-loop optogenetic stimulation increases food intake by selectively delaying the onset of satiety. We show that upstream leptin-receptor-expressing neurons in the dorsomedial hypothalamus (DMH LepR ) are tuned to respond to sweet or fatty tastes and exhibit time-locked activation during feeding that is the mirror image of downstream AgRP cells. These findings reveal an unexpected role for taste in the negative feedback control of ingestion. They also reveal a mechanism by which AgRP neurons, which are the primary cells that drive hunger, are able to influence the moment-by-moment dynamics of food consumption., Competing Interests: Declaration of interests The authors declare no competing interests., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: critically ill cirrhotic patients may present a serious clinical condition defined as acute-on-chronic liver failure with high mortality. While established scoring systems like Child-Pugh and Model for End-stage Liver Disease (MELD) offer prognostic insights, their limitations warrant exploration of alternative markers. The lactate/albumin ratio (LAR) serves as a potential prognostic indicator in critical care settings, yet its utility in cirrhotic patients remains underexplored., Methods: one hundred and seventy-five critically ill cirrhotic patients were assessed in this retrospective cohort study. Clinical severity scores, including Acute Physiology and Chronic Health Evaluation II (APACHE II), Sequential Organ Failure Assessment (SOFA), and Chronic Liver Failure-Organ Failure Score (CLIF-OF) were compared with LAR along with traditional liver failure scoring systems. Logistic regression and receiver operating characteristic (ROC) curve analysis were used to evaluate prognostic performance., Results: Intensive Care Unit (ICU) nonsurvivors had significantly higher scores in all liver failure and clinical severity scores compared to survivors (p < 0.001). Median LAR was significantly higher in nonsurvivors (p < 0.001). ROC analysis revealed comparable prognostic accuracy between LAR, APACHE II, SOFA, and CLIF-OF scores in predicting ICU mortality. Logistic regression identified SOFA score at 48th hour, LAR, and requirement of mechanical ventilation as independent predictors of ICU mortality., Conclusion: LAR demonstrates promising prognostic utility in predicting ICU mortality among critically ill cirrhotic patients, complementing established scoring systems. Early reassessment using SOFA score at 48th hour may guide therapeutic interventions and improve patient outcomes. Further prospective studies are warranted to validate these findings and optimize clinical management strategies.

Objective: Immaturity of the digestive tract and enteric nervous system is a widely accepted theory for infantile colic (IC) etiopathogenesis. The study aimed to show whether neurotrophins that are necessary for normal functioning and development of the gastrointestinal system have a role in the pathogenesis of IC. Materials and Methods: The IC group ( n  = 75) comprising the mothers of infants with IC and the control group ( n  = 75) were included to this cross-sectional case-control study. Brain-derived neurotrophic factor (BDNF), glial cell-derived neurotrophic factor (GDNF), ciliary neurotrophic factor (CNTF), and nerve growth factor (NGF) levels of breast milk samples were evaluated by immunosorbent analysis method. Results: The mean age of infants with IC was 7.3 ± 2.8 weeks, while the mean age of the control group was 8.1 ± 2.9 weeks ( p  = 0.110). No significant difference was found between the breast milk BDNF, GDNF, CNTF, and NGF levels of two groups ( p  = 0.941, p  = 0.510, p  = 0.533, p  = 0.839, respectively). Conclusions: This is the first report comparing the neurotrophin levels of the breast milk samples taken from the mothers of infants with and without IC. The study demonstrated that breast milk neurotrophin levels of the mothers did not differ significantly between the infants with and without IC.

The termination of a meal is controlled by dedicated neural circuits in the caudal brainstem. A key challenge is to understand how these circuits transform the sensory signals generated during feeding into dynamic control of behaviour. The caudal nucleus of the solitary tract (cNTS) is the first site in the brain where many meal-related signals are sensed and integrated 1-4 , but how the cNTS processes ingestive feedback during behaviour is unknown. Here we describe how prolactin-releasing hormone (PRLH) and GCG neurons, two principal cNTS cell types that promote non-aversive satiety, are regulated during ingestion. PRLH neurons showed sustained activation by visceral feedback when nutrients were infused into the stomach, but these sustained responses were substantially reduced during oral consumption. Instead, PRLH neurons shifted to a phasic activity pattern that was time-locked to ingestion and linked to the taste of food. Optogenetic manipulations revealed that PRLH neurons control the duration of seconds-timescale feeding bursts, revealing a mechanism by which orosensory signals feed back to restrain the pace of ingestion. By contrast, GCG neurons were activated by mechanical feedback from the gut, tracked the amount of food consumed and promoted satiety that lasted for tens of minutes. These findings reveal that sequential negative feedback signals from the mouth and gut engage distinct circuits in the caudal brainstem, which in turn control elements of feeding behaviour operating on short and long timescales., (© 2023. The Author(s).)

Anti N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease that often presents with various neurological and neuropsychiatric symptoms. Although most reported cases occur in children, only a limited number of studies on children are available. The subject of this case report is an 8-month-old female who presented with fever, vomiting, and seizure. She was diagnosed with encephalitis and treated with acyclovir. After 21 days, she showed irritability, seizure, orolingual-facial dyskinesias, choreodystonic movements, hemiparesis, dysphagia, strabismus, lack of interest in light and objects. Clinical signs, neuroimaging findings, and serum analysis of anti-NMDAR antibodies confirmed the diagnosis of anti-NMDAR encephalitis. After the first line of treatment, she showed full recovery. We update the infants with anti-NMDAR encephalitis in the literature. Clinical outcomes suggest that patients with anti-NMDAR encephalitis are mostly poor in the infants, excluding our case. We propose that early and appropriate treatments are critical for timely diagnosis and rapid improvement.

Purpose: The aim of this study was to determine the clinical, laboratory, and radiological factors related with posttraumatic epilepsy (PTE)., Methods: The study is a multicenter descriptive cross-sectional cohort study. Children who followed up for TBI in the pediatric intensive care unit between 2014 and 2021 were included. Demographic data and clinical and radiological parameters were recorded from electronic case forms. All patients who were in the 6-month posttraumatic period were evaluated by a neurologist for PTE., Results: Four hundred seventy-seven patients were included. The median age at the time of trauma was 66 (IQR 27-122) months, and 298 (62.5%) were male. Two hundred eighty (58.7%) patients had multiple traumas. The mortality rate was 11.7%. The mean duration of hospitalization, pediatric intensive care unit hospitalization and mechanical ventilation, Rotterdam score, PRISM III score, and GCS at admission were higher in patients with epilepsy (p < 0.05). The rate of epilepsy was higher in patients with severe TBI, cerebral edema on tomography and clinical findings of increased intracranial pressure, blood transfusion in the intensive care unit, multiple intracranial hemorrhages, and intubated patients (p < 0.05). In logistic regression analysis, the presence of intracranial hemorrhage in more than one compartment of the brain (OR 6.13, 95%CI 3.05-12.33) and the presence of seizures (OR 9.75, 95%CI 4.80-19.83) were independently significant in terms of the development of epilepsy (p < 0.001)., Conclusions: In this multicenter cross-sectional study, intracranial hemorrhages in more than one compartment and clinical seizures during intensive care unit admission were found to be independent risk factors for PTE development in pediatric intensive care unit patients with TBI., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

We aimed to identify nonconvulsive seizures (NCS) and nonconvulsive status epilepticus (NCSE) in a pediatric intensive care unit (PICU). A prospective cohort study on 35 patients who underwent continuous electroencephalographic monitoring in the PICU was done. The patients were evaluated to collect data of their demographics, clinical diagnoses, clinical seizures by electroencephalography, and neuroimaging findings. One case with NCSE and 4 cases with NCS were diagnosed among the 35 patients. The etiology of the patient with NCSE showed antiepileptic drug (AED) withdrawal. The etiology of the patients with NCS included electrical injury, head trauma, subarachnoid hemorrhage, and pneumonia. The findings suggest that younger age, epilepsy, acute structural brain abnormalities, abrupt cessation of AED, and clinically overt seizures before NCSE/NCS are associated with significant risk for NCS/NCSE. In addition, the electrical injury may also be considered as a risk factor for electrographic seizure though such a case has not yet been reported.

This report describes a case in which diplopia was developed as a finding of postinfectious anti- N -methyl- d -aspartate receptor encephalitis. Infectious encephalitis, especially herpes simplex virus, is essential as it is one of the triggers of autoimmune encephalitis. Even if the cases present unexpected clinical findings, we should be vigilant in terms of autoimmune processes, such as diplopia seen in our case., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: We aimed to analyze pediatric patients with coronavirus disease 2019 (COVID-19) with a diverse spectrum of neurological manifestations in a single center since neurological involvement in children is still poorly understood., Methods: We performed a retrospective study on 912 children aged between zero and 18 years who had a positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) test result and symptoms of COVID-19 from March 2020 to March 2021 in a single center., Results: Among 912 patients, 37.5% (n = 342) had neurological symptoms and 62.5% (n = 570) had no neurological symptoms. The mean age of patients with neurological symptoms was significantly higher (14.2 ± 3.7 vs 9.9 ± 5.7; P < 0.001). Three hundred and twenty-two patients had nonspecific symptoms (ageusia, anosmia, parosmia, headache, vertigo, myalgia), whereas 20 patients had specific involvement (seizures/febrile infection-related epilepsy syndrome, cranial nerve palsy, Guillain-Barré syndrome and variants, acute disseminated encephalomyelitis, central nervous system vasculitis). The mean age of the patients with nonspecific neurological symptoms was significantly higher (14.6 ± 3.1 vs 7.7 ± 5.7; P < 0.001)., Conclusion: This study presents a large number of patients with a diverse spectrum of neurological manifestations. The rare neurological manifestations reported in our study will contribute to better understanding the neurological involvement of SARS-CoV-2 in children. The study also points out the differences of SARS-CoV-2-related neurological manifestations between patients at different ages. Physicians should be alert about recognizing the early neurological manifestations of the SARS-CoV-2 in children., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Animals associate cues with outcomes and update these associations as new information is presented. This requires the hippocampus, yet how hippocampal neurons track changes in cue-outcome associations remains unclear. Using two-photon calcium imaging, we tracked the same dCA1 and vCA1 neurons across days to determine how responses evolve across phases of odor-outcome learning. Initially, odors elicited robust responses in dCA1, whereas, in vCA1, odor responses primarily emerged after learning and embedded information about the paired outcome. Population activity in both regions rapidly reorganized with learning and then stabilized, storing learned odor representations for days, even after extinction or pairing with a different outcome. Additionally, we found stable, robust signals across CA1 when mice anticipated outcomes under behavioral control but not when mice anticipated an inescapable aversive outcome. These results show how the hippocampus encodes, stores and updates learned associations and illuminates the unique contributions of dorsal and ventral hippocampus., (© 2023. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Since the COVID-19 pandemic began, various severe acute respiratory syndrome coronavirus 2 variants have been identified with different characteristics than the nonvariant strain. We retrospectively evaluated the demographic and clinical differences in the cohort of hospitalized COVID-19 children (1 month-18 years old) between March 11, 2020, and September 31, 2022, by the time the variants identified in our country predominate. Bonferroni post hoc analysis was performed to compare the differences between the periods. Of the 283 children in this study, 142 (50.2%) were females. The median age was 36 (interquartile range [IQR]: 7-132) months. Sixty-three (22.2%) patients were hospitalized in the nonvariant period, 24 (8.5%) in the Alpha period, 93 (32.9%) in the Delta period, and 103 (36.4%) in the Omicron period. Fever was the most common symptom in all groups, with no statistically significant differences (p = 0.25). In the Omicron period, respiratory and gastrointestinal symptoms decreased, and neurological symptoms increased significantly compared to other periods: [respiratory symptoms; nonvariant (65.1%) vs. Omicron (41.7%), (p = 0.024)], [gastrointestinal symptoms; Delta (41.9%) vs. Omicron (22.3%), (p = 0.018), [neurological symptoms; Delta (14.5%) vs. Omicron (31.1%), (p = 0.03]. Altered mental status and seizures were more common during the Omicron period compared to the pre-Omicron (nonvariant, Alpha, and Delta) period (p = 0.017 and p = 0.005, respectively). Although the main symptoms in children with COVID-19 were fever and respiratory symptoms, an increase in severe neurological manifestations was seen throughout the Omicron variant period., (© 2023 Wiley Periodicals LLC.)

Nails have been found to be a non-invasive and readily available tissue whose mineral content can change because of a change in dietary mineral intake. Thus, this study was undertaken to determine whether boron (B) supplementation would change the concentrations of some mineral elements in nails and whether these changes correlated with changes induced in bone. Female New Zealand White rabbits (aged 8 months, 2-2.5 kg weight) were fed a grain-based, high-energy diet containing 3.88 mg B/kg. The rabbits were divided into four treatment groups: controls receiving no supplemental B (N: 7; C) and three groups supplemented with 30 mg B/L in drinking water as borax decahydrate (Na 2 B 4 O 7 ∙10H 2 O, N: 10; BD), borax anhydrous (Na 2 B 4 O 7 , N: 7; Bah), and boric acid (H 3 BO 3 , N: 7; BA). Boron, calcium (Ca), copper (Cu), iron (Fe), magnesium (Mg), phosphorus (P), potassium (K), sodium (Na), sulfur (S), and zinc (Zn) concentrations in nails were determined by inductively coupled plasma atomic emission spectroscopy. Parametric and non-parametric multiple group comparisons and post hoc tests were performed and whether a correlation between nail and tibia and femur mineral elements concentrations were determined. A p-value of < 0.05 was considered statistically significant. Boron was not detectable in control nails but was found in the nails of the three B supplemented groups. Boron supplementation markedly increased the Ca concentration in nails with the effect greatest in the BA and BD groups. The P and Mg concentrations also were increased by B supplementation with the effect most marked in the BA group. In contrast, B supplementation decreased the Na concentration with the effect most noticeable in the BD and Bah groups. The Zn concentration in nails was not affected by BA and BD supplementation but was decreased by Bah supplementation. Boron supplementation did not significantly affect the concentrations of Cu, Fe, Mo, K, and S in nails. No meaningful significant correlations were found between nail mineral elements and tibia and femur mineral elements found previously. Nails can be an indicator of the response to boron supplementation but are not useful to indicate changes in mineral elements in bone in response to B supplementation., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Children with chronic neurological diseases, including cerebral palsy (CP), are especially susceptible to vaccine-preventable infections and face an increased risk of severe respiratory infections and decompensation of their disease. This study aims to examine age-appropriate immunization status and related factors in the CP population of our country. This cross-sectional prospective multicentered survey study included 18 pediatric neurology clinics around Turkey, wherein outpatient children with CP were included in the study. Data on patient and CP characteristics, concomitant disorders, vaccination status included in the National Immunization Program (NIP), administration, and influenza vaccine recommendation were collected at a single visit. A total of 1194 patients were enrolled. Regarding immunization records, the most frequently administrated and schedule completed vaccines were BCG (90.8%), hepatitis B (88.9%), and oral poliovirus vaccine (88.5%). MMR was administered to 77.3%, and DTaP-IPV-HiB was administered to 60.5% of patients. For the pneumococcal vaccines, 54.1% of children received PCV in the scope of the NIP, and 15.2% of children were not fully vaccinated for their age. The influenza vaccine was administered only to 3.4% of the patients at any time and was never recommended to 1122 parents (93.9%). In the patients with severe (grades 4 and 5) motor dysfunction, the frequency of incomplete/none vaccination of hepatitis B, BCG, DTaP-IPV-HiB, OPV, and MMR was statistically more common than mild to moderate (grades 1-3) motor dysfunction (p = 0.003, p < 0.001, p < 0.001, p < 0.00, and p < 0.001, respectively). Physicians' influenza vaccine recommendation was higher in the severe motor dysfunction group, and the difference was statistically significant (p = 0.029).Conclusion: Children with CP had lower immunization rates and incomplete immunization programs. Clinicians must ensure children with CP receive the same preventative health measures as healthy children, including vaccines. What is Known: • Health authorities have defined chronic neurological diseases as high-risk conditions for influenza and pneumococcal infections, and they recommend vaccines against these infections. • Children with CP have a high risk of incomplete and delayed immunization, a significant concern given to their increased healthcare needs and vulnerability to infectious diseases. What is New: • Influenza vaccination was recommended for patients hospitalized due to pneumonia at a higher rate, and patients were administered influenza vaccine more commonly. • Children with CP who had higher levels of motor dysfunction (levels 4 and 5) were more likely to be overdue immunizations., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Aim: To examine the regional spinal curvatures and movements in the sagittal and frontal planes during sitting position, and the ability to act independently in patients with CP and to compare the differences between children and adolescents with minimal-to-moderate functional limitations., Method: Twenty-one participants diagnosed with CP aged 5-16 years were included. The participants' Gross Motor Function Classification System (GMFCS) levels were determined and those at levels I (minimal functional limitation group: minFLG) or II-III (moderate functional limitation group: modFLG) were included. Spinal curvatures, mobilities, and inclinations in the sagittal and frontal planes were evaluated in the sitting position using a hand-held, computer-assisted non-invasive electromechanical device. Participants' functional independence levels were assessed with the Functional Independence Measure (WeeFIM)., Results: In the sagittal plane, there were no differences in terms of spinal curvatures between the minFLG and modFLG (p > 0.05). Spinal mobility degrees for flexion (thoracic and lumbar regions and total spine), extension (sacral region), and total spine mobility scores were significantly greater in the minFLG (p < 0.05). In the frontal plane, lumbar spinal curvature significantly increased, and total spine mobility in the right/left lateral motions and functional independence decreased in the modFLG (p < 0.05)., Interpretation: The children/adolescents with minimal functional limitations had greater spinal mobility during flexion, extension, and lateral flexions. Spinal curvatures were similar between groups in the sagittal plane. The lumbar region posture scores in the frontal plane observed as lordoscoliosis were higher, and functional independence was lower in the modFLG., (Copyright © 2021 Elsevier B.V. All rights reserved.)

The reported beneficial effects of boron on mineralized tissues in animals and humans vary. Thus, a study was performed to assess whether the variability was the result of different forms of boron supplementation, method of supplementation, and increased adiposity of the rabbit experimental model. Thirty-one female New Zealand White rabbits, (aged 8 months, 2-2.5 kg weight) were fed a grain-based high energy diet containing 11.76 MJ/kg (2850 kcal/kg) and 3.88 mg boron/kg. The rabbits were randomly divided into four treatment groups: Control group was not supplemented with boron (n:7; C), and three groups supplemented with 30 mg boron/L in drinking water in the forms of borax decahydrate (Na2O4B7 10H2O, n:10; BD), borax anhydrous (Na2O4B7, n:7; Bah) or boric acid (H2BO3, n:7; BA). Cone beam micro computed tomographic (micro-CT), histological and elemental analysis was used to evaluate the bones/teeth. Results of the experiments demonstrated that boron supplementation had beneficial effects on mineralized tissue but varied with the type of treatment. Mineral density of the femur was increased by the Bah and BA treatments (p < 0.001), but only BA increased mineral density in the tibia (p = 0.015). In incisor teeth, mineral density of dentin was increased by all boron treatments (p < 0.001), and mineral density of enamel was increased by the BD and Bah treatments. Mineral analysis found that all boron treatments increased the boron concentration in tibia and femur. In the tibia, both the BD and Bah treatments decreased the iron concentration, and the BD treatment decreased the magnesium concentration. Sodium and zinc concentrations in the tibia were decreased by the Bah and BA treatments. The boron treatments did not significantly affect the calcium, copper, molybdenum, potassium phosphorus, and sulfur concentrations. The findings show that boron supplementation can have beneficial effects on mineralized tissues in an animal model with increased adiposity, which is a model of increased inflammatory stress. However, this effect varies with the form of boron supplemented, the method of supplementation, and the mineralized tissue examined., (Copyright © 2021 Elsevier GmbH. All rights reserved.)

Objective: Celiac disease may present with one or more neurological signs and/or symptoms. We aimed to define the incidence of accompanying neurological manifestations in children diagnosed as having celiac disease., Methods: The prospective study included 146 children diagnosed as having celiac disease. The medical records (presentation symptoms, clinical findings, serological test, duodenal biopsy results, lack/deficiency of vitamin, tissue type, accompanying autoimmune disorders) and demographic data of all patients were also reviewed., Results: Thirty-five (23.9%) of the 146 celiac patients exhibited one or more neurological findings. Headache (11.6%) and dizziness (6.1%) were the most common symptoms among neurological manifestations. There was a significant difference between the patients with and without neurological manifestations in terms of sex, biopsy result, and tissue type (P < 0.05). Moreover, there was a statistically significant difference between tissue types of the patients with and without headache (P < 0.05). We found that grade 3a by Marsh classification was the most common type among the patients with and without neurological findings in celiac disease. On neuroimaging evaluation of patients, 1 patient with chronic focal ischemic lesion, 1 patient with Chiari type 1 malformation, and 1 patient with subcortical white matter changes were identified., Conclusions: Pathophysiology of neurological involvement in celiac disease is liable for various neurological findings. This study contributes to data suggesting that female sex, mild histopathological form, and human leukocyte antigen DQ2 heterozygosity are related to neurological manifestations, and also human leukocyte antigen DQ2 heterozygosity is associated with headache in celiac disease., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Electrical status epilepticus during slow-wave sleep (ESES) is an epilepsy syndrome with sleep-induced epileptic discharges and acquired impairment of cognition or behavior. Since the disease's original description in 1971, no clear consensus has emerged on diagnostic criteria or optimal treatment. The treatment of ESES can be challenging, often including numerous antiepileptic drugs, immunomodulatory agents, and even surgical interventions. There is little evidence to guide treatment because only retrospective studies and case reports on the efficacy of treatment of ESES are present in literature. In this paper, we aim to analyze the etiopathogenesis of ESES in the new genetic era and to evaluate the treatment modalities in accordance with the genetic data and electroclinic spectrum of ESES., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Pediatric Neurosciences.)

Background: Vitamin B12 deficiency occurs primarily as a result of insufficient dietary intake in children in developing countries. Vitamin B12 deficiency produces a cluster of neurological symptoms in children., Aim: The aim of this study was to describe the vitamin B12 status of patients who were admitted with neurological symptoms and to evaluate the clinical response to vitamin B12 treatment., Materials and Methods: This study was conducted on children who had vitamin B12 deficiency presented with neurological findings from January 2014 to October 2016. Patients with serum vitamin B12 levels lower than 300 pg/mL received intramuscular or oral vitamin B12 treatment., Results: Three hundred and fifty-one patients presenting with neurologic symptoms and who had low serum vitamin B12 deficiency were analyzed. Our study population was composed mainly of adolescent age. The most common symptom with respect to age was headache. In infant patients, most common symptoms were seizure and developmental delay., Conclusion: Early diagnosis and vitamin B12 treatment are advocated to avoid long-term injury. Our study shows that patients with serum vitamin B12 levels lower than 300 pg/mL showed clinical improvement of neurological symptoms after receiving vitamin B12 treatment., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Pediatric Neurosciences.)

Objective: The aim of this clinical trial was to evaluate the levels of Neuregulin-4 (Nrg4), Erb-b2 receptor tyrosine kinase 4 (ErbB4), interleukin (IL)-6, IL-10, nitric oxide synthase (NOS)-2, and arginase (Arg)-1 in periodontal health and disease., Materials and Methods: This study includes systemically healthy 20 periodontally healthy (H), 20 gingivitis (G), 20 stage II periodontitis (P1), and 20 stage III periodontitis (P2) subjects. Periodontal clinical measurements and samples of gingival crevicular fluid (GCF) and serum were obtained at baseline and 4 weeks after non-surgical periodontal treatment (NSPT). Enzyme-linked immunosorbent assay (ELISA) was used to determine ErbB4, Nrg4, IL-6, IL-10, NOS2, and Arg1 levels in all samples., Results: GCF ErbB4 and Nrg4 total amounts and IL-6/IL-10 ratio were significantly higher in G, P1, and P2 groups than H group. Serum NOS2 levels were significantly lower, whereas serum Arg1 levels were higher in H group than the others. The GCF levels of ErbB4 and Nrg4 were significantly decreased after NSPT in G, P1, and P2 groups. Additionally, the GCF levels of ErbB4 and Nrg4 were positively correlated with all clinical parameters and IL-6/IL-10 ratio., Conclusions: Nrg4 and its receptor ErbB4 might have crucial roles in the pathogenesis of periodontal disease. These results should be verified with future prospective studies to further clarify the exact role of those biomarkers., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. All rights reserved.)

Purpose: This study aims to compare the neurocognitive outcome in term infants who were treated using phenobarbital (PB) and levetiracetam (LEV) monotherapy for neonatal clinical seizures., Methods: Term infants who were treated using PB or LEV monotherapy as the first-line anti-epileptic treatment for neonatal clinical seizures and followed-up in a pediatric neurology outpatient clinic were enrolled in this study. Neurodevelopmental outcome assessments were carried out using the Bayley Scales of Infant Development, third edition (BSID-III), including cognitive, receptive language, expressive language, fine motor and gross motor subscales., Results: The study group consisted of 62 infants who received monotherapy with PB monotherapy (n = 22) and LEV (n = 40). The mean duration of monotherapy treatment was 8 ± 6 months. There was no statistically significant difference between PB and LEV monotherapy groups concerning each outcome parameter on the BSID-III. There was also no statistically significant difference between PB and LEV monotherapy subgroups excluding the infants with neurodevelopmental impairment with a BSID-III scale score<7 or a composite score<85., Conclusion: Our findings suggest that both LEV and PB therapy can be equally safe as monotherapy for neonatal clinical seizures for the neurodevelopmental outcome assessment with BSID-III., Competing Interests: Declaration of Competing Interest The authors declare no potential conflicts of interest regarding this research, authorship, and/or publication of this article., (Copyright © 2020 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Objectives: The aim of this present study was to evaluate the levels of Netrin-1 and Unc5b in periodontal health and disease., Background: Netrin-1, acting via its receptor UNC5b, regulates the inflammatory response and takes apart in bone destructive diseases., Methods: Samples of gingival crevicular fluid (GCF), whole saliva, and serum were taken from systemically healthy, nonsmoking 20 periodontitis , 20 gingivitis patients, and 20 periodontally healthy subjects at baseline and 4 weeks after nonsurgical periodontal treatment (NSPT). Whole-mouth and site-specific clinical periodontal parameters were recorded. Netrin-1 and Unc5b levels were measured by enzyme-linked immunosorbent assay. Data were analyzed by nonparametric tests., Results: Total amount of Netrin-1 in GCF was significantly higher in periodontitis than the others, and the levels were significantly reduced after NSPT. Salivary and serum concentrations of Netrin-1 were significantly different among the study groups (P = .000), and NSPT significantly increased the concentration levels of both salivary and serum Netrin-1 (P < .05). Healthy subjects had significantly lower GCF (P = .001) and conversely, higher salivary and serum levels of Unc5b than the other groups (P = .002). The GCF levels of Unc5b were significantly reduced (P < .01), and conversely, serum concentrations were significantly increased after NSPT (P < .05). GCF Netrin-1 and Unc5b total amounts were positively correlated with clinical parameters (P < .01 and P < .05) whereas salivary Netrin-1 and Unc5b concentrations were negatively correlated with clinical parameters (P < .01 and P < .05)., Conclusions: The results of this study indicate that Netrin-1 and its receptor Unc5b may have essential roles in periodontal inflammation and those can be assumed as useful therapeutic agent to control inflammation and periodontal breakdown., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: We explored the clinical and molecular characteristics of molybdenum cofactor deficiency due to MOCS2 muations., Methods: We summarize the genetic and clinical findings of previously reported patients with a MOCS2 mutation. We also present a new patient with novel neuroradiological findings associated with molybdenum cofactor deficiency due to a novel homozygous variant in the 5' untranslated region of the MOCS2 gene., Results: The study population comprised 35 patients with a MOCS2 gene mutation. All reported children had delayed motor milestones. The major initial symptom was seizures in neonatal period. Facial dysmorphism was present in 61% of the patients. Only one patient had ectopia lentis. Agenesis of the corpus callosum and an associated interhemispheric cyst in our case are novel neuroradiological findings., Conclusions: The occurrence of neonatal seizures and feeding difficulties can be the first clinical signs of molybdenum cofactor deficiency. Although there is no effective therapy for this condition, early diagnosis and genetic analysis of these lethal disorders facilitate adequate genetic counseling., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Chromosomal microarray (CMA) analysis for discovery of copy number variants (CNVs) is now recommended as a first-line diagnostic tool in patients with unexplained developmental delay/intellectual disability (DD/ID) and autism spectrum disorders. In this study, we present the results of CMA analysis in patients with DD/ID. Of 210 patients, pathogenic CNVs were detected in 26 (12%) and variants of uncertain clinical significance in 36 (17%) children. The diagnosis of well-recognized genetic syndromes was achieved in 12 patients. CMA analysis revealed pathogenic de novo CNVs, such as 11p13 duplication with new clinical features. Our results support the utility of CMA as a routine diagnostic test for unexplained DD/ID.

The CDKL5 disorder is characterized by early onset epilepsy, stereotypical hand movement, absent speech and severe hypotonia. Herein, we report epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) in apatient with CDKL5 disorder. She admitted with complaints of frequently recurring generalized tonic and myoclonic seizures. The diagnoses were confirmed by de novo CDKL5 mutation, c.197&#95;198delCT (p.L67QfsX23). Interictal EEG revealed generalized spike and slow-wave activity, occurring intermittently in wakefulness but present for at least 85% of non-REM sleep, consistent with the diagnosis of CSWS. To our knowledge, this is the first report of CSWS associated with CDKL5 disorder.

Congenital myasthenic syndromes (CMS) are neuromuscular transmission disorders caused by mutations in genes encoding neuromuscular junction proteins. CMS due to choline acetyltransferase (CHAT) gene is characterized by episodic apnea. We report a case of a 12-month-old female patient presented with recurrent episodic apnea carrying a mutation in CHAT gene, p.I336T. Furthermore, we describe the genetic and clinical findings in 44 CMS patients due to CHAT mutations in the literature up to date. Episodes of apnea and respiratory insufficiency are the hallmarks of CHAT mutations. Clinical manifestations usually provoked by infections and fever. CMS due to CHAT mutations are rare, but it is important to diagnosis. Early diagnosis and appropriate treatment can improve morbidity and mortality., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (Georg Thieme Verlag KG Stuttgart · New York.)

The goal of the study was to evaluate the effectiveness of analgesia nociception index (ANI) monitoring during intraoperative period for patients with thoracic paravertebral block (TPVB) undergoing breast surgery under general anesthesia. This prospective randomized trial was performed after receiving ethics committee approval in 44 patients who were scheduled to undergo breast surgery under general anesthesia. TPVB was performed in the preoperative period using 20 mL of bupivacaine 0.25% at T4 level. Anesthesia maintenance was provided with sevoflurane in O 2 : air mixture and remifentanil infusion. Intraoperative concentration of sevoflurane was adjusted according to BIS monitoring keeping the values between 40-60. In a randomized manner patients were divided into two groups. In Group control (n:22) intraoperative remifentanil infusion rate was regulated according to hemodynamic parameters, in Group ANI (n:22) remifentanil infusion rate was titrated to keep ANI monitoring values between 50-70. Total remifentanil consumption was recorded as micrograms. Demographic data, anesthesia and surgery time, intraoperative hemodynamic parameters, post-anesthesia recovery time and requirement of additional analgesic in the recovery drug were recorded. There were no significant difference in demographic data, intraoperative hemodynamic parameters, post-anesthesia recovery time and requirement of additional analgesic drug. There was a statistically significant difference between groups in total remifentanil consumption (Group ANI: 629.6 ± 422.4 mcg, Group control: 965.2 ± 543.6 mcg) (p = 0.027). In patients under general anesthesia ANI monitorisation can help optimisation of opioid consumption and provide data about nociception/antinociception intraoperatively but further experimental and clinical trials in a large scale are needed.

Purpose: Levetiracetam is a broad-spectrum anti-epileptic drug that is effective against both focal and generalized epilepsies. In this study, we aimed to evaluate the efficacy, tolerability and safety of levetiracetam monotherapy in the management of different seizure types in children with epilepsy under the age of two., Method: This retrospective study was conducted on children with a diagnosis of epilepsy from January 2014 to January 2017. To be included in the study, patients were required to be less than two years of age at the time levetiracetam was initiated as initial monotherapy and to be followed clinically for at least 6 months., Results: Of the 92 patients, 61 (66%) patients were seizure free. Fifty-eight percent of the patients (31 of 53) with focal epilepsy were seizure free and 77% (30 of 39) generalized epilepsy were seizure free. We found that levetiracetam monotherapy was effective in both focal and generalized epilepsy. Levetiracetam monotherapy was significantly more effective in patients with unknown etiologies (p = 0.004). Seizure freedom rate under levetiracetam monotherapy was significantly higher in patients with normal psychomotor development (p = 0.000). Seizure freedom rate under levetiracetam monotherapy was significantly higher in patients with unknown etiologies and normal psychomotor development. Normal psychomotor development was the strongest predictor of seizure control under levetiracetam monotherapy (OR = 6; 95% CI = 2.3-16.0; p < 0.001). Five children (1%) reported irritability. No hematological or biochemical, or behavioral adverse side effects except irritability were reported in any children. No patient discontinued levetiracetam therapy because of treatment-related side effects., Conclusion: Our study showed levetiracetam to be an effective, well tolerated and safe agent for the treatment of a variety of seizure types and etiologies seen in infants., (Copyright © 2018 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)