Which terms should be used to describe medications used in the treatment of seizure disorders? An ILAE position paper.

A variety of terms, such as "antiepileptic," "anticonvulsant," and "antiseizure" have been historically applied to medications for the treatment of seizure disorders. Terminology is important because using terms that do not accurately reflect the action of specific treatments may result in a misunderstanding of their effects and inappropriate use. The present International League Against Epilepsy (ILAE) position paper used a Delphi approach to develop recommendations on English-language terminology applicable to pharmacological agents currently approved for treating seizure disorders. There was consensus that these medications should be collectively named "antiseizure medications". This term accurately reflects their primarily symptomatic effect against seizures and reduces the possibility of health care practitioners, patients, or caregivers having undue expectations or an incorrect understanding of the real action of these medications. The term "antiseizure" to describe these agents does not exclude the possibility of beneficial effects on the course of the disease and comorbidities that result from the downstream effects of seizures, whenever these beneficial effects can be explained solely by the suppression of seizure activity. It is acknowledged that other treatments, mostly under development, can exert direct favorable actions on the underlying disease or its progression, by having "antiepileptogenic" or "disease-modifying" effects. A more-refined terminology to describe precisely these actions needs to be developed.

Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions.

Epilepsy syndromes have been recognized for >50 years, as distinct electroclinical phenotypes with therapeutic and prognostic implications. Nonetheless, no formally accepted International League Against Epilepsy (ILAE) classification of epilepsy syndromes has existed. The ILAE Task Force on Nosology and Definitions was established to reach consensus regarding which entities fulfilled criteria for an epilepsy syndrome and to provide definitions for each syndrome. We defined an epilepsy syndrome as "a characteristic cluster of clinical and electroencephalographic features, often supported by specific etiological findings (structural, genetic, metabolic, immune, and infectious)." The diagnosis of a syndrome in an individual with epilepsy frequently carries prognostic and treatment implications. Syndromes often have age-dependent presentations and a range of specific comorbidities. This paper describes the guiding principles and process for syndrome identification in both children and adults, and the template of clinical data included for each syndrome. We divided syndromes into typical age at onset, and further characterized them based on seizure and epilepsy types and association with developmental and/or epileptic encephalopathy or progressive neurological deterioration. Definitions for each specific syndrome are contained within the corresponding position papers.

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions.

In 2017, the International League Against Epilepsy (ILAE) Classification of Epilepsies described the "genetic generalized epilepsies" (GGEs), which contained the "idiopathic generalized epilepsies" (IGEs). The goal of this paper is to delineate the four syndromes comprising the IGEs, namely childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic-clonic seizures alone. We provide updated diagnostic criteria for these IGE syndromes determined by the expert consensus opinion of the ILAE's Task Force on Nosology and Definitions (2017-2021) and international external experts outside our Task Force. We incorporate current knowledge from recent advances in genetic, imaging, and electroencephalographic studies, together with current terminology and classification of seizures and epilepsies. Patients that do not fulfill criteria for one of these syndromes, but that have one, or a combination, of the following generalized seizure types: absence, myoclonic, tonic-clonic and myoclonic-tonic-clonic seizures, with 2.5-5.5 Hz generalized spike-wave should be classified as having GGE. Recognizing these four IGE syndromes as a special grouping among the GGEs is helpful, as they carry prognostic and therapeutic implications.

International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age: position statement by the ILAE Task Force on Nosology and Definitions.

The goal of this paper is to provide updated diagnostic criteria for the epilepsy syndromes that have a variable age of onset, based on expert consensus of the International League Against Epilepsy Nosology and Definitions Taskforce (2017-2021). We use language consistent with current accepted epilepsy and seizure classifications and incorporate knowledge from advances in genetics, electroencephalography, and imaging. Our aim in delineating the epilepsy syndromes that present at a variable age is to aid diagnosis and to guide investigations for etiology and treatments for these patients.

ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology.

The International League Against Epilepsy (ILAE) Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances that have taken place since the last ratified classification in 1989. As a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies, and communication around the world. The new classification originates from a draft document submitted for public comments in 2013, which was revised to incorporate extensive feedback from the international epilepsy community over several rounds of consultation. It presents three levels, starting with seizure type, where it assumes that the patient is having epileptic seizures as defined by the new 2017 ILAE Seizure Classification. After diagnosis of the seizure type, the next step is diagnosis of epilepsy type, including focal epilepsy, generalized epilepsy, combined generalized, and focal epilepsy, and also an unknown epilepsy group. The third level is that of epilepsy syndrome, where a specific syndromic diagnosis can be made. The new classification incorporates etiology along each stage, emphasizing the need to consider etiology at each step of diagnosis, as it often carries significant treatment implications. Etiology is broken into six subgroups, selected because of their potential therapeutic consequences. New terminology is introduced such as developmental and epileptic encephalopathy. The term benign is replaced by the terms self-limited and pharmacoresponsive, to be used where appropriate. It is hoped that this new framework will assist in improving epilepsy care and research in the 21st century.

The dilemma of arranged marriages in people with epilepsy. An expert group appraisal.

INTRODUCTION: Matrimony remains a challenging psychosocial problem confronting people with epilepsy (PWE). People with epilepsy are less likely to marry; however, their marital prospects are most seriously compromised in arranged marriages. AIMS: The aim of this study was to document marital prospects and outcomes in PWE going through arranged marriage and to propose optimal practices for counseling PWE contemplating arranged marriage. METHODS: A MEDLINE search and literature review were conducted, followed by a cross-disciplinary meeting of experts to generate consensus. RESULTS: People with epilepsy experience high levels of felt and enacted stigma in arranged marriages, but the repercussions are heavily biased against women. Hiding epilepsy is common during marital negotiations but may be associated with poor medication adherence, reduced physician visits, and poor marital outcome. Although divorce rates are generally insubstantial in PWE, divorce rates appear to be higher in PWE undergoing arranged marriages. In these marriages, hiding epilepsy during marital negotiations is a risk factor for divorce. CONCLUSIONS: In communities in which arranged marriages are common, physicians caring for PWE are best-equipped to counsel them about their marital prospects. Marital plans and aspirations should be discussed with the family of the person with epilepsy in a timely and proactive manner. The benefits of disclosing epilepsy during marital negotiations should be underscored.

Research priorities in epilepsy for the Asia-Oceanian region.

The Asia-Oceanian region is the most populous region in the world. Although there has been substantial economic development and improvement in health services in recent years, epilepsy remains generally an underrecognized and understudied condition. To help promote research in the region, the Commission on Asian and Oceanian Affairs (CAOA) of the International League Against Epilepsy (ILAE) appointed the Research Task Force (RTF) to facilitate the development of research priorities for the region. Research that focuses on issues that are unique or of particular importance in the Asia-Oceanian region is encouraged, and that captures the impact of the dynamic socioeconomic changes taking place in the region is emphasized. Based on these considerations, we propose research "dimensions" as priorities within the Asia-Oceanian region. These are studies (1) that would lead to fuller appreciation of the health burden of epilepsy, particularly the treatment gap; (2) that would lead to better understanding of the causes of epilepsy; (3) that would alleviate the psychosocial consequences of epilepsy; (4) that would develop better therapies and improved therapeutic outcomes; and (5) that would improve the research infrastructure.

Sinonasal Schwannoma: A Case Report.

Schwannomas are benign tumours of the peripheral nerve sheath that can develop anywhere in the body. In the nasal cavity, they hardly ever happen. Lesions known as paranasal schwannomas make up fewer than 4% of all head and neck schwannomas. Here, we present a case of sinonasal schwannoma in a 24-year-old male who had been experiencing nasal obstruction and loss of sense of smell for the previous 2 years. The lump was successfully removed without any postoperative complications.

New furanocoumarins and other chemical constituents from Ficus carica root heartwood.

Two new furanocoumarins, 5-(1",1"-dimethylallyl)-8-methyl psoralen (1) and 2"-O-acetyl oxypeucedanin hydrate-3"-methyl ether (2), were isolated from the root heartwood of Ficus carica Linn. together with three known furanocoumarins, two triterpenoids, two long-chain compounds, and a steroid. Their structures and relative configurations were elucidated by spectroscopic methods (IR, HR-ESI-MS, and NMR) and by comparison of their NMR spectral data with those of related compounds.

A study on mechanical and tribological properties of eco-friendly synthesized ZrO2-doped borosilicate glasses.

The research article aims to investigate the mechanical and tribological characteristics of bioactive glass specimens comprising 31B2O3-20SiO2-24.5Na2O-(24.5-x) CaO and xZrO2 (mol%). This glass system was partially derived from bio-waste, with varying concentrations of Zirconia (ZrO2) represented x (x = 0, 1, 3, and 5). The specimens were fabricated using the traditional melt-quench method. Mechanical studies like hardness and compressive strength were measured using Vickers hardness tester and universal tensile machine respectively, while a pin-on-disk tribometer was used to analyze the tribological characteristics. All the specimens were soaked in SBF for a week to assess in-vitro bioactivity. The research findings indicate that Zirconia inclusion resulted in a significant reduction in the intensity of hydroxyapatite peaks of FTIR and XRD spectra, suggesting a decrease in bioactivity. However, it concurrently resulted in increased glass hardness, with the highest value (∼7.55 GPa) observed in the BSG-5 glass sample. Similarly, compressive strength results demonstrated maximum strength in BSG-5 glass specimen, with a value of approximately ∼132 MPa. Moreover, the tribological properties of the glass system were enhanced, evident from the reduced coefficient of friction and specific wear rate. Notably, the BSG-5 glass specimen exhibited the least wear coefficient of 0.018 mm3/N-m at a track radius of 40 mm and a load of 15N. These findings were further supported by SEM images of the worn-out ZrO2-Doped Borosilicate Glass surface. Overall, the results suggest that the addition of Zirconia to borosilicate glass holds promise for improving its mechanical and tribological characteristics. However, this enhancement comes at the expense of its bioactivity. Consequently, the modified glass system presents a cost effective viable option for various applications, particularly in load-bearing and dental applications.

Cervical Sympathetic Chain Schwannoma-Our Experience.

Tumours arising from the parapharyngeal space are uncommon. The most common tumour arising primarily from this region is a neurogenic tumour, especially Schwannoma. The most common site of origin of a Schwannoma in parapharyngeal space is Vagus nerve. Cervical sympathetic chain (CSC) schwannomas are extremely rare. Here, we discuss a case of a 30-year old female with cervical sympathetic chain schwannoma, the clinical presentation, diagnosis and management.

Genetic testing in the epilepsies--report of the ILAE Genetics Commission.

In this report, the International League Against Epilepsy (ILAE) Genetics Commission discusses essential issues to be considered with regard to clinical genetic testing in the epilepsies. Genetic research on the epilepsies has led to the identification of more than 20 genes with a major effect on susceptibility to idiopathic epilepsies. The most important potential clinical application of these discoveries is genetic testing: the use of genetic information, either to clarify the diagnosis in people already known or suspected to have epilepsy (diagnostic testing), or to predict onset of epilepsy in people at risk because of a family history (predictive testing). Although genetic testing has many potential benefits, it also has potential harms, and assessment of these potential benefits and harms in particular situations is complex. Moreover, many treating clinicians are unfamiliar with the types of tests available, how to access them, how to decide whether they should be offered, and what measures should be used to maximize benefit and minimize harm to their patients. Because the field is moving rapidly, with new information emerging practically every day, we present a framework for considering the clinical utility of genetic testing that can be applied to many different syndromes and clinical contexts. Given the current state of knowledge, genetic testing has high clinical utility in few clinical contexts, but in some of these it carries implications for daily clinical practice.

Microscopic Thyroidectomy: The Way We Do It.

The present study was conducted with an intent to document the reduced morbidity in terms of postoperative hypocalcemia, injury to recurrent laryngeal and external branch of superior laryngeal nerve, in patients undergoing microscope assisted thyroidectomy. The present study enrolled a total of 878 patrients who underwent hemi, total and completion thyroidectomies, over a period of 3 years at Jain ENT Hospital, Jaipur. In the present study, out of 1118 RL nerves dissected temporary paresis was found in 1.52% and permanent palsy in only 0.36%. Temporary hypocalcemia was seen in 8.12% while permanent hypocalcemia in 0.6% patients. EBSLN could be identified in 1082 of the 1118 nerves dissected. We recommend the use microscope routinely for all thyroid surgeries, starting from the very first step in view of the reduced morbidity that it offers.

Early Intratympanic Methylprednisolone in Sudden SNHL: A Frequency-wise Analysis.

Sudden sensorineural hearing loss is a dire medical emergency which must be treated at the earliest to get better long term hearing results. Our study aims to determine the efficacy of intratympanic steroid (Methylprednisolone) on auditory outcomes in patients of sudden sensorineural hearing loss and study the relation between time of onset of hearing loss to start of therapy and frequency-wise recovery of hearing loss. A prospective cohort clinical study with 33 patients with sudden hearing loss of 30 dB or more were treated with the intratympanic injection of methylprednisolone and the effect of the drug was observed. In this study, 33 patients with sudden onset (unilateral or bilateral) of hearing loss were treated with intratympanic methylprednisolone. The duration at which the drug was administered and the age of the participants was taken into consideration. Main outcome measures included audiometry results at low, medium and high hearing loss frequencies. The specific frequency at which the hearing improvement took place was tabulated. It was observed that hearing improved significantly if the steroid is injected within the first 4 days of onset (p < 0.05) at all the frequencies. A gain of 15 dB or more was achieved in more than 78% patients after injecting methylprednisolone intratympanically. A statistically significant association was found between recovery rate and frequency of hearing loss with patients showing greater improvement at low hearing loss frequency in comparison to mid and high frequencies (p < 0.05). The drug efficacy does not change with the age of the patient.

Vibration induced refrigeration and energy harvesting using piezoelectric materials: a finite element study.

In this study, the bi-functional performance of a small-scale piezoelectric cantilever, which coupled piezoelectric and elastocaloric phenomena in a single device to produce energy harvesting as well as refrigeration effects due to vibration, has been investigated. Finite element modeling has been used to examine the performance of the device. The basic structure of the device is a cantilever that vibrates between two thermal bodies (hot and cold). The properties of BaTiO3 (single crystal) were used to examine the bi-functional performance of piezoelectric cantilevers. In this study, different cases have been investigated, which are based on a number of cantilevers between hot and cold thermal bodies. When the number of cantilevers is one, the net cooling is 0.3 K and the power is 0.03 µW, while for four cantilevers, the net cooling is 1.2 K and 0.13 µW of power is produced. The results show that as we increase the number of cantilevers, a greater refrigeration effect is produced and higher power across the electrical load is achieved.

Association of cutaneous adverse drug reactions due to antiepileptic drugs with HLA alleles in a North Indian population.

PURPOSE: Aromatic antiepileptic drugs (AEDs) are frequently implicated in cutaneous adverse drug reactions (cADRs), a few of which are associated with certain human leukocyte antigen (HLA) alleles in some populations. We aimed to find HLA-associations with AED-related cADRs among North Indians. METHODS: North Indian subjects with cADR due to an AED, and those who were AED-tolerant were recruited as cases and controls, respectively. Genotyping for HLA-A, B and DRB1 were performed. Statistical analysis to compare carrier-rates and allele-frequencies between cases and controls (and healthy population, where necessary), was done for HLA-alleles occurring more than twice in either group. RESULTS: 120 cases {11 - Lamotrigine (LTG), 14 -Valproic acid (VPA), 8 -Levetiracetam (LEV), 35 -Carbamazepine (CBZ) and 52 - Phenytoin (PHT)}, and 250 controls were recruited. Presence of HLA-A*31:01 and HLA-B*51:01 were found to increase the risk of Maculopapular exanthema (MPE) due to CBZ and PHT (OR = 6.38; 95% CI: 1.46-27.75; OR = 4.60; 95% CI: 1.54-13.72, respectively). Among the severe cADRs, HLA-B*57:01(OR = 11.00 95% CI: 1.41-85.81) and HLA-DRB1*07:01 (OR = 7.25; 95% CI: 1.09-48.18) were noted to be significantly associated with CBZ-induced Stevens Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN); HLA-B *51:01 was associated with drug reaction eosinophilia and systemic symptoms (DRESS) caused by PHT (OR = 6.90; 95% CI: 1.38-34.29). CONCLUSIONS: We found significant associations of some HLA alleles with specific cADRs to CBZ and PHT in North Indians, which may need to be tested before AED-initiation; only screening for HLA-B*15:02 may not help in this population.

Association of Child Neurology-Indian Epilepsy Society Consensus Document on Parental Counseling of Children with Epilepsy.

When a child is diagnosed with epilepsy, counseling regarding the same is done by the treating doctor. Most parents are frightened and have poor knowledge about epilepsy. Therapeutic advice including drug dosage, administration and side effects takes up the major part of physician's time, thereby neglecting important issues like home seizure management, follow up and others. These lacunae in knowledge require systematic patient and family education. To address these issues, an expert group meeting of pediatric neurologists and epileptologists in India along with social workers/epilepsy educators, legal experts, parents, and teachers was held. The various aspects regarding parental counseling in children with epilepsy were discussed and a consensus document was formulated. Here authors present the group consensus statement on counseling parents and caregivers of children with epilepsy. This document is intended to help physicians and pediatricians counsel the families when a child is diagnosed with epilepsy.

Bioactivities of polyphenolics from the roots of Bauhinia racemosa.

Various extracts of Bauhinia racemosa Lamk. (roots) and the isolated polyphenolics, viz. 1,7,8,12b-tetrahydro-2,2,4-trimethyl-10-methoxy-2H-benzo [6,7] cyclohepta[1,2,3-de][1]benzopyran-5,9-diol (racemosol) (1), 1,7,8,12b-tetrahydro-2,2,4-trimethyl-2H-benzo[6,7]cyclohepta[1,2,3-de][1]benzopyran-5,10,11-triol (2), and 1,7,8,12b-tetrahydro-2,2,4-trimethyl-2H-benzo[6,7] cyclohepta [1,2,3-de][1]benzopyran-5,9,10-triol (de-O-methyl racemosol) (3) were screened for antibacterial, antifungal, and antiviral activities. The isolated compounds exhibited profound antibacterial and antifungal activities while methanol extract exhibited potential efficacy against Herpes simplex virus.

Surgical outcome of cortical dysplasias presenting with chronic intractable epilepsy: a 10-year experience.

BACKGROUND: There has been sparse description of cortical dysplasias (CDs) causing intractable epilepsy from India. AIM: Clinical retrospective study of CDs causing intractable epilepsy that underwent surgery. MATERIALS AND METHODS: Fifty-seven cases of CDs reviewed (1995 till July 2006) are presented. All patients had intractable epilepsy, and underwent a complete epilepsy surgery workup (inter ictal electroencephalography (EEG), video EEG, MRI as per epilepsy protocol, SPECT {interictal, ictal with subtraction and co-registration when required}, and PET when necessary). Surgical treatment included a wide exposure of the pathology with a detailed electrocorticography under optimal anesthetic conditions. Mapping of the sensori-motor area was performed where indicated. Procedures included resection either alone or combined with multiple subpial transactions when extending into the eloquent areas. RESULTS: Our study had 28 (49.12%) cases of isolated focal CDs, and 29 (50.67%) with dual pathology. Average age at the time of onset of seizures in our series was 7.04 years (three months to 24 years), and average age at the time of surgery was 10.97 years (eight months to 45 years). Among coexistent pathologies, one had associated MTS, 16 had coexistent gangliogliomas and 12 (dysembryonic neuroepithelial tumor) DNTs. At an average follow-up of 3.035 years (range 5-10 years), three patients were lost to follow-up. Fifty-one per cent (29/57) patients had a good outcome (Engel Grade I) and 26%(15/57) had a Grade II outcome. CONCLUSION: Cortical dysplasias have a good outcome if evaluated and managed with concordant electrical and imaging modalities.

Neuropathological spectrum of lesions associated with intractable epilepsies: a 10-year experience with a series of 153 resections.

BACKGROUND: Surgical management of intractable epilepsies is currently an established mode of therapy in various clinical settings. AIMS: To retrospectively evaluate the neuropathological findings in both temporal and extratemporal lobe resections in such patients. MATERIALS AND METHODS: The study included resected specimens from patients with intractable epilepsy managed at a tertiary care hospital of India, during a 10-year period (1995-2004). RESULTS: A total of 153 patients, with mean age of 19.4 years and male predominance (73.2%) were included in the study. Overall, there was a predilection for the temporal lobe (73.2%), while 41cases were extratemporal in location. On histopathology, mesial temporal sclerosis (MTS) (24.8%) was the commonest lesion, followed by tumors (19.6%) and isolated focal cortical dysplasia (FCD - 15.11%). Other less common findings included Rasmussen encephalitis, non-specific gliosis and vascular malformations. In addition, 20.9% (32 cases) had dual lesions, majority of which included FCD with ganglioglioma (15 cases) or with dysembryoplastic neuroepithelial tumor (12 cases). In the temporal lobe, neoplasms and dual lesions formed the majority (apart from MTS), unlike dual lesions followed by neoplasms and FCD, in the extratemporal location. CONCLUSION: This series demonstrates that most patients with chronic intractable epilepsy have significant histopathological findings and highlights the neuropathological spectrum of such lesions, in the Indian context. This was similar to that reported from the West, but different from the single Indian series available in the literature. Further, the overall profile of temporal lobe lesions was not different from the extratemporal ones.