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BACKGROUND: Cortical dysplasia (CD) is associated with several behavioral disorders in both the pediatric and the adult population. The effect of melatonin on behavioral disorders in rats generated CD has not been investigated so far. AIM: To investigate the effects of melatonin administration on activity and anxietic behavior of neonatal rats in a model of CD. MATERIALS AND METHODS: Newborn Sprague-Dawley rats (n=21) were randomized into three groups. On postnatal day 1, one freeze lesion was carried out in 14 rats between bregma and lambda to create a CD model. Another group of neonatal rats served as control group (n=7). Those 14 rats were either administered melatonin (n=7) or vehicle solution (n=7). Melatonin treatment (4 mg/kg/day, i.p.) was initiated ten days after induction of cold injury and continued for three weeks. Animal activity and anxiety were analyzed by using open field and elevated plus maze tests 24h after the last melatonin administration (day 32) in a blind manner. RESULTS: It was observed that CD induced animals spent significantly less time in the open field area when compared to the other groups (p < 0.01). Additionally, the time spent in the open field area was significantly elevated in the melatonin-treated animals compared to both the control and the CD groups (p < 0.01). Accordingly, anxiety scores in the CD group was significantly increased (p < 0.01), and this effect could be reversed by administration of melatonin. CONCLUSIONS: Melatonin exerts protective behavioral effects against cortical dysplasia in newborn rats. Further clinical investigations may prove melatonin as a useful therapeutic adjunct to prevent from possible behavioural damages of cortical dysplasia. [ABSTRACT FROM AUTHOR]

Purpose: This study aims to assess the diagnostic power of brain asymmetry indices and neuropsychological tests for differentiating mesial temporal lobe epilepsy (MTLE) and schizophrenia (SCZ)., Methods: We studied a total of 39 women including 13 MTLE, 13 SCZ, and 13 healthy individuals (HC). A neuropsychological test battery (NPT) was administered and scored by an experienced neuropsychologist, and NeuroQuant (CorTechs Labs Inc., San Diego, California) software was used to calculate brain asymmetry indices (ASI) for 71 different anatomical regions of all participants based on their 3D T1 MR imaging scans., Results: Asymmetry indices measured from 10 regions showed statistically significant differences between the three groups. In this study, a multi-class linear discriminant analysis (LDA) model was built based on a total of fifteen variables composed of the most five significantly informative NPT scores and ten significant asymmetry indices, and the model achieved an accuracy of 87.2%. In pairwise classification, the accuracy for distinguishing MTLE from either SCZ or HC was 94.8%, while the accuracy for distinguishing SCZ from either MTLE or HC was 92.3%., Conclusion: The ability to differentiate MTLE from SCZ using neuroradiological and neuropsychological biomarkers, even within a limited patient cohort, could make a substantial contribution to research in larger patient groups using different machine learning techniques., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Lithium-metal batteries with a solid electrolyte separator are promising for advanced battery applications, however, most electrolytes show parasitic side reactions at the low potential of lithium metal. Therefore, it is essential to understand how much (and how fast) charge is consumed in these parasitic reactions. In this study, a new electrochemical method is presented for the characterization of electrolyte side reactions occurring on active metal electrode surfaces. The viability of this new method is demonstrated in a so-called anode-free stainless steel ∣ Li 6 PS 5 Cl ∣ Li cell. The method also holds promise for investigating dendritic lithium growth (and dead lithium formation), as well as for analyzing various electrolytes and current collectors. The experimental setup allows easy electrode removal for post-mortem analysis, and the SEI's heterogeneous/layered microstructure is revealed through complementary analytical techniques. We expect this method to become a valuable tool in the future for solid-state lithium metal batteries and potentially other cell chemistries., (© 2023. The Author(s).)

With the current global projection of over 130 million electric vehicles (EVs), there soon will be a need for battery waste management. Especially for all-solid-state lithium-ion batteries (lithium ASSBs), aspects of waste management and circular economy have not been addressed so far. Within such ASSBs, the use of solid-electrolytes like garnet-type Li 6.5 La 3 Zr 1.5 Ta 0.5 O 12 (LLZTO) may shift focus on strategies to recover not only the transition metal elements but also elements like La/Zr/Ta. In this work, we present a two-step recycling approach using citric acid as the leaching agent to separate and recover the individual components of a model cell comprising of Li 4 Ti 5 O 12 (LTO) anode, Li 6.5 La 3 Zr 1.5 Ta 0.5 O 12 (LLZTO) garnet electrolyte and LiNi 1/3 Mn 1/3 Co 1/3 O 2 (NMC) cathode. We observe that by adjusting the concentration of citric acid, it was possible to separate the materials from each other without strong mixing of individual phases and also to maintain their principle performance characteristics. Thus, the process developed has a potential for upscaling and can guide towards considering separation capability of battery components in the development of lithium ASSBs., (© 2023 The Authors. ChemSusChem published by Wiley-VCH GmbH.)

New molecular therapies are available for the treatment of spinal muscular atrophy (SMA) but early intervention is required. We report two cases that were diagnosed prenatally, where treatment with nusinersen was initiated within 7 h and three days respectively. The children were followed up for 13 months and almost six years respectively. Both children have developed within entirely normal centiles, indicating that initiating treatment immediately after birth, as in these cases, is essential for a good outcome., Competing Interests: Declaration of Competing Interest NONE, (Copyright © 2022. Published by Elsevier B.V.)

The unusual physical and chemical properties of electrolytes with excessive salt contents have resulted in rising interest in highly concentrated electrolytes, especially for their application in batteries. Here, we report strikingly good electrochemical performance in terms of conductivity and stability for a binary electrolyte system, consisting of lithium bis(fluorosulfonyl)imide (LiFSI) salt and ethylene carbonate (EC) solvent. The electrolyte is explored for different cell configurations spanning both high-capacity and high-voltage electrodes, which are well known for incompatibilities with conventional electrolyte systems: Li metal, Si/graphite composites, LiNi 0.33 Mn 0.33 Co 0.33 O 2 (NMC111), and LiNi 0.5 Mn 1.5 O 4 (LNMO). As compared to a LiTFSI counterpart as well as a common LP40 electrolyte, it is seen that the LiFSI:EC electrolyte system is superior in Li-metal-Si/graphite cells. Moreover, in the absence of Li metal, it is possible to use highly concentrated electrolytes (e.g., 1:2 salt:solvent molar ratio), and a considerable improvement on the electrochemical performance of NMC111-Si/graphite cells was achieved with the LiFSI:EC 1:2 electrolyte both at the room temperature and elevated temperature (55 °C). Surface characterization with scanning electron microscopy (SEM) and X-ray photoelectron spectroscopy (XPS) showed the presence of thicker surface film formation with the LiFSI-based electrolyte as compared to the reference electrolyte (LP40) for both positive and negative electrodes, indicating better passivation ability of such surface films during extended cycling. Despite displaying good stability with the NMC111 positive electrode, the LiFSI-based electrolyte showed less compatibility with the high-voltage spinel LNMO electrode (∼4.7 V vs Li + /Li)., Competing Interests: The authors declare no competing financial interest., (© 2022 The Authors. Published by American Chemical Society.)

BACKGROUND Encephalocraniocutaneous lipomatosis (ECCL) was first announced as a new type of ectomesodermal dysgenesis in 1970 by Haberland and Perou. ECCL was first described in 1970, and approximately 60 cases have been reported since then. The classic triad of ECCL are skin, ocular, and central nervous system involvement, including conditions such as unilateral porencephalic cyst, ipsilateral lipomatous hamartoma of the scalp-eyelids-eye globe, cortical atrophy, cranial asymmetry, developmental delay, seizures, mental retardation, and spasticity of the contralateral limbs. The dermatological hallmark is a hairless fatty tissue nevus of the scalp called nevus psiloliparus. CASE REPORT An 11-year-old right-handed boy, born at full term, was referred to our clinic. His family had no consanguinity or history of neurocutaneous disease. The patient's physical examination revealed a large hairless lesion on the right frontoparietal scalp called nevus psiloliparus. Beginning from the birth, a dermolipoma (an uncommon benign tumor) was reported to have occurred on the conjunctiva, mostly ipsilateral in his right eye and present on the ipsilateral side of the neurological abnormalities shown on magnetic resonance imaging and computed tomography. The patient had muscle weakness in left upper and lower extremities. He had a mild form of mental retardation. CONCLUSIONS There is no specific treatment for ECCL. Management of ECCL is usually symptomatic. Surgical correction of a cutaneous lesion can be performed for cosmetic improvement. An early diagnosis of ECCL allows for early symptom treatment and improved patient quality of life.

Background: Knobloch syndrome is a rare, autosomal recessive, developmental disorder characterized by stereotyped ocular abnormalities with or without occipital skull deformities (encephalocele, bone defects, and cutis aplasia). Although there is clear heterogeneity in clinical presentation, central nervous system malformations, aside from the characteristic encephalocele, have not typically been considered a component of the disease phenotype., Methods: Four patients originally presented for genetic evaluation of symptomatic structural brain malformations. Whole-genome genotyping, whole-exome sequencing, and confirmatory Sanger sequencing were performed. Using immunohistochemical analysis, we investigated the protein expression pattern of COL18A1 in the mid-fetal and adult human cerebral cortex and then analyzed the spatial and temporal changes in the expression pattern of COL18A1 during human cortical development using the Human Brain Transcriptome database., Results: We identified two novel homozygous deleterious frame-shift mutations in the COL18A1 gene. On further investigation of these patients and their families, we found that many exhibited certain characteristics of Knobloch syndrome, including pronounced ocular defects. Our data strongly support an important role for COL18A1 in brain development, and this report contributes to an enhanced characterization of the brain malformations that can result from deficiencies of collagen XVIII., Conclusions: This case series highlights the diagnostic power and clinical utility of whole-exome sequencing technology-allowing clinicians and physician scientists to better understand the pathophysiology and presentations of rare diseases. We suggest that patients who are clinically diagnosed with Knobloch syndrome and/or found to have COL18A1 mutations via genetic screening should be investigated for potential structural brain abnormalities even in the absence of an encephalocele., (Copyright © 2014 Elsevier Inc. All rights reserved.)

In the light of the latest knowledge acquired from clinical and laboratory research dealing with genetic, molecular biology and neuroimaging, existing classifications were successively revised by the International League Against Epilepsy (ILAE) in 2001, 2006, and 2010. In the latest classification established in 2010, proposals articulated radical changes in terms of concepts and definitions of the previously published classifications and put forward new classifications for epileptic seizures, epilepsies and electroclinical syndromes. This review refers to the changes of the new classification with their reasons and criticisms., Competing Interests: Conflict of interest: The authors reported no conflict of interest related to this article. Çıkar çatışması: Yazarlar bu makale ile ilgili olarak herhangi bir çıkar çatışması bildirmemişlerdir.

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a common medically intractable epilepsy syndrome. Although pathogenesis of HS still remains highly controversial, genetics may play a role as a predisposing factor. Previous evidence in a Japanese population revealed that the homozygotes for allele T at position -511 of the interleukin (IL)-1β gene promoter region (IL-1β-511 T/T) confers susceptibility to the development of HS. However, whether this polymorphism has an effect on IL-1β levels in MTLEHS patients was not demonstrated. This study aimed to analyze the distribution of this particular polymorphism in a group of Turkish HS patients and correlate the polymorphism with IL-1β secretion from the lymphocytes, thus revealing a functional role for IL-1β in the etiopathogenesis of HS. A single base pair polymorphism at position -511 in the promoter region of the IL-1β gene was analyzed. The spontaneous and 1 ng/mL lipopolysaccharidestimulated production of IL-1β by peripheral blood mononuclear cells after 4 and 24 h of incubation were measured by ELISA method. The heterozygous type (-511 C/T) was the most common genotype. There was no difference in frequency of allele -511 T between patients and controls. Analysis of IL-1β levels, genotype and allele distributions showed no significant difference among the groups (P>0.05). Nevertheless, it was seen that patients who carry a T allele at position -511 of the IL-1β gene had increased IL-1β levels. T-allele carriage may be important. Only IL-1β secretion from the lymphocytes has been assessed in this study. Considering the importance of IL-1β in the etiopathogenesis of HS, further studies are needed to evaluate locally produced IL-1β levels.

Objective: The aim of the study described here was to investigate the efficacy, tolerability, and side effects of oxcarbazepine (OXC) monotherapy in newly diagnosed, previously untreated adult and elderly patients with partial epilepsy., Methods: We prospectively analyzed and recorded the efficacy, tolerability, and side effects of OXC monotherapy. The results were analyzed on the basis of etiologic classifications and age distributions. Remission was defined as seizure freedom for at least 1 year., Results: A total of 147 patients were evaluated in a single center for a median of 18 months (range: 14-36 months). Overall, 92 patients (62.6%) were seizure free for at least 12 months and 55 of them (37.4%) were unresponsive despite treatment with the maximum tolerable dose of OXC. There was a significant difference in the outcomes of patients with cryptogenic (75% remission) and symptomatic (51.9% remission) epilepsy (P=0.004). Patients with cerebral tumors did worse than the remainder of the patients in the symptomatic group (36.7% remission) (P=0.03). Results were favorable for the elderly; 14 patients (73.6%) in the elderly subgroup became seizure free for at least 1 year, and the remission was achieved with low to moderate doses (approximately 900 mg/day). Overall, 13 patients (8.8%) discontinued OXC due to intolerable side effects. Side effects leading to discontinuation were: Stevens-Johnson syndrome (n=2, 1.4%); fatigue and drowsiness (n=2, 1.4%); dizziness, nausea, and vomiting with normal laboratory tests (n=2, 1.4%); dizziness, nausea, and vomiting with serum Na levels <130 mEq/L (n=5, 3.4%); and elevated serum gamma-glutamyl transferase levels (GGT>200mg/dL) (n=1, 0.7%). OXC proved to be a tolerable drug for the elderly; only one patient experienced symptomatic hyponatremia with mild symptoms and responded well to fluid restriction, which did not lead to discontinuation of OXC., Conclusion: Although the limitations of our study include its open-label design, the results suggest that OXC monotherapy may be regarded as an effective first-line monotherapy option for adult and elderly patients with partial epilepsy, but has low efficacy in patients with cerebral tumors.

Behçet's disease (BD) is a multisystemic, recurrent, inflammatory disorder of unknown aetiology. Neurological involvement is characterised either by primary parenchymal lesions or secondary to major vascular involvement. Seizures are rarely seen in BD and their occurrence can be related to seizure provoking factors or exacerbation of the disease. We experienced a case of neuro-BD presenting with subacutely developing mental and behavioral changes, followed by left dominant tetraparesis with bilateral pyramidal signs, fever and left hand focal motor seizures with elementery clonic motor signs which later evolved into right hand epilepsia partialis continua (EPC) of Kojevnikov. The seizures were very resistant to antiepileptic drugs and 8 months after neurological involvement the patient died. The EPC evolving after neurological involvement is associated with high mortality rate.

We aimed to assess the relapse rate of epilepsy, prospectively attributable to antiepileptic drug (AED) withdrawal in seizure-free patients and to determine the risk factors for seizure recurrence. Seventy-nine patients with epilepsy who were seizure-free for at least 4 years were enrolled into the study. The AEDs were tapered by one-sixth every 2 months. The EEG and clinical examination were performed at the beginning; at each visit during discontinuation and 2, 6, 12, 24, and 36 months after the complete drug withdrawal. For each patient, records were obtained of the main demographic and clinical variables. A total of 49 patients completed the discontinuation programme. Twenty-eight patients (57%) relapsed while 21 of those (42.8%) did not suffer a relapse at the end of the study period. In patients discontinuing treatment, the probability of relapse was 21.4% during the tapering period (especially in the last months), 28.6% at 1 month, 14.3% at 3 months, 3.6% at 6 months, 7.1% at 12 months, 17.8% at 24 months, and 7.1% at 36 months. The age at onset of epilepsy and the duration of active disease were found to affect the risk of relapse. Although drug withdrawal could be considered in adult patients free of seizures for 4 years, the final decision should be tailored to the patient's clinical, emotional, and socio-cultural profile.

Purpose: Our aim was to compare three available seizure classifications (SCs), namely, the international classification of epileptic seizures published in 1981 (ICES; Epilepsia 1981;22:489-50); the semiological seizure classification (SSC) by H. Luders, J. Acharya, C. Baumgartner, et al. (Epilepsia 1998;39:1006-13; Acta Neurol Scand 1999;99:137-41); and the proposal of a new diagnostic scheme for seizures (PDSS) by J. Engel, Jr. (Epilepsia 2001;42:796-803) published in 2001. The three SCs were compared with respect to diagnostic success rates, usefulness, and consistency by a large group of neurologists in this preliminary study., Methods: After a training period, 28 blindfed participants with different levels of experience with epilepsy classified videos or written descriptions of 48 randomly selected seizures according to the three SCs. Definite diagnoses of the seizures were established based on all clinical, ictal/interictal EEG, and MRI data. All the participants answered a questionnaire concerning their preferences for SCs after the study., Results: The overall diagnostic success rates were 81.4% for ICES, 80.5% for PDSS, and 87.5%, for SSC. Various parameters concerning experience with epilepsy affected success rates positively, without reaching statistical significance, whereas experience with epilepsy surgery appeared to be a parameter significantly affecting the success rate in all SCs. In reliability analysis, Cronbach's alpha was 0.94 for ICES, 0.88 for PDSS, and 0.70 for SSC, all showing good agreement in the group. Nineteen reviewers chose SSC, eight chose ICES, and one chose PDSS as their preference in the questionnaire, completed after the end of the study., Conclusion: The results of this preliminary study demonstrate that with proper training, physicians treating epilepsy patients can handle new SCs, and emphasize the need for revision of the current classification.

Progressive facial hemiatrophy (PFH), Parry-Romberg syndrome, is a rare disorder frequently associated with epilepsy. We describe a 28-year-old man who had PFH and partial epilepsy that was easily controlled with antiepileptic drugs. In accordance with this patient's benign course of seizures, the cortical silent period was prolonged in the symptomatic hemisphere. This finding may represent compensatory interictal mechanisms in epilepsy.

Trace element content of different tissues might be altered by both age and exercise training. We aimed to determine the effects of a 1-yr swimming protocol (60 min/d, 5 day/wk) on tissue levels and the distribution of zinc (Zn), magnesium (Mg), and copper (Cu) in aging rats. Three groups were formed: sedentary and trained old groups and a young control group. Tissue Zn, Mg, and Cu concentrations were measured in the kidney, heart, liver, lungs, and gastrocnemius and soleus muscles. Kidney zinc concentration significantly decreased in the sedentary old group compared to the young control group (p<0.01) and was significantly higher in the trained old group compared to the sedentary old group (p<0.01), whereas Zn levels in the soleus muscle significantly increased in the sedentary old group in comparison to young controls (p<0.05). Tissue Mg concentrations remained unchanged. The sedentary old group exhibited a significant decrease in kidney Cu concentration compared to the young control group (p<0.01). Although kidney Cu levels also decreased in trained old rats in comparison to young controls (p<0.05), they were significantly higher than in sedentary old rats (p<0.01). The decrease in kidney Zn and Cu content as a result of aging was partly prevented by long-term swimming exercise.

The effect of sulfur dioxide (SO2) on brain antioxidant status, lipid peroxidation, and somatosensory evoked potentials (SEPs) was investigated in diabetic rats. A total of 40 rats were divided into 4 equal groups: control (C), SO2 + C (SO2), diabetic (D), and SO2 + D (DSO2). Experimental diabetes mellitus was induced by i.v. injection of alloxan at a dose of 50 mg/kg body weight. Ten ppm SO2 was administered to the rats in the sulfur dioxide groups (SO2 and DSO2) in an exposure chamber. Exposure occurred 1 hr/day, 7 days/wk, for 6 wk; control rats were exposed to filtered air during the same time periods. Although SO2 exposure markedly increased copper, zinc Superoxide dismutase activity, it significantly decreased glutathione peroxidase activity in both the diabetic and nondiabetic groups, compared with the C group. Brain catalase activity was unaltered; however, brain thiobarbituric acid reactive substances (TBARS) were elevated in all experimental groups with respect to the C group. SEP components P1, N1, P2, and N2 were significantly increased in all experimental groups, compared with the C group, and these components were also prolonged in the DSO2 group with respect to the other groups. The authors' findings suggest that exposure to SO2, because it increases lipid peroxidation, can change antioxidant enzyme activities and affect SEP components in diabetic rats.

Brain stem interneuronal excitability can be assessed by recording the recovery cycle of the blink reflex and exteroceptive suppression of temporalis muscle activity. Abnormal endogenous pain control mechanisms due to disturbed brain stem interneuronal activity have been implicated in the pathogenesis of tension-type headaches. The blink reflex, exteroceptive suppression of temporalis muscle activity, and the recovery curve of both the R2 component of the blink reflex and the ES2 component of the exteroceptive suppression of the temporalis muscle activity were studied in 20 patients with migraine without aura, 32 patients with tension-type headache, and 20 normal controls. In our study, the blink reflex was elicited by stimulation of the supraorbital nerve; the exteroceptive suppression of the temporalis muscle activity was elicited by applying electrical shocks to the labial commissure, both on the lower and upper sides. The recovery cycle was established by delivering paired shocks at different interstimulus intervals. Comparisons were made between normal control subjects, patients with migraine without aura, and patients with tension-type headache. The latency of R1, R2, and R2', the amplitude and size of the R2 and R2' components of the blink reflex, the latency and duration of the ES1 and ES2 components, and the recovery curve of the ES2 component of the temporalis muscle activity did not differ between groups. However, the recovery curve of the R2 component of the blink reflex diminished in patients with tension-type headache compared with the other groups. Our findings indicate reduced excitability of the brain stem interneurons in patients with tension-type headache.