Is epileptogenesis a key to treatment of childhood epileptic encephalopathies?

Comments to “Is epileptogenesis a key to treatment of childhood epileptic encephalopathies?”

Eyelid myoclonia with absences (Jeavons syndrome): still an overlooked epilepsy syndrome. Comments to Covanis review in this issue of <italic>Journal of Epileptology</italic>

Radovici or Jeavons syndrome? Reply to comments

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2015-0031_s_001"><h3>Background</h3><p>Cerebral palsy (CP) is the most common childhood motor impairment. Epilepsy affects approximately one third of patients with CP. It is characterized by earlier disclosure, it is more severe and shows greater resistance than that of the general epilepsy treatment, associated with necessity for polytherapy. Its presence can result in gradual loss of function, loss of posture in non-ambulant individuals with severe disabilities and cognitive impairment risk, behavioural disorders and reducing probability of walking.</p></sec><sec id="j_joepi-2015-0031_s_002"><h3>Aim</h3><p>The aim of the study was to evaluate the functioning of people with CP with and without epilepsy.</p></sec><sec id="j_joepi-2015-0031_s_003"><h3>Material and Methods</h3><p>The study included 210 patients with a diagnosis of CP, aged 0–18 years. The study was conducted among the patients using the physiotherapy services in centres in southern Poland. The study used the Paediatric Evaluation of Disability Inventory (PEDI) and the classification systems: GMFCS, MACS, and CFCS.</p></sec><sec id="j_joepi-2015-0031_s_004"><h3>Results</h3><p>There were significant differences with regards to social functioning (53.7/67.4; W = 179, p = 0.006) and support in the social functioning (65.4/89.9; W = 185.5, p = 0.007) in patients with diplegia. However, mobility (19.55/29.00; W = 392, p = 0.018) and the social functioning (36.95/44.1; W = 418.5, p = 0.042) were lower in epileptic patients with tetraplegia. In patients with hemiplegia, there were no significant differences, although each domain with epilepsy subgroup had a lower rating than the subgroup without epilepsy.</p></sec><sec id="j_joepi-2015-0031_s_005"><h3>Conclusion</h3><p>The presence of epilepsy is associated with lower levels of social function in patients with cerebral palsy; particularly, with regard to mobility and selfservice.</p><p>Assessment of epilepsy impact on the level of social functioning of people with CP (diplegia, tetraplegia, hemiplegia) is difficult because ambiguous relationship with mental retardation. The assessment should be undertaken separately for each group of spastic CP.</p></sec></abstract>

The effect of seizures on functional status of people with spastic forms of cerebral palsy

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2015-0032_s_001"><h3>Introduction</h3><p>The correct diagnosis of epileptic seizures and non-epileptic attacks has a decisive influence on treatment, counseling and duration of possible work limitations.</p><p>Diagnostic efforts should aim towards classifying the seizure as precisely as possible. For risk assessments, e.g. at the workplace, a close cooperation and networking of all professionals involved in the epilepsy treatment, care and consultation processes is required.</p></sec><sec id="j_joepi-2015-0032_s_002"><h3>Aim</h3><p>To present guidelines for assessment of occupational capacity of persons with epilepsy and to discuss their value in clinical practice.</p></sec><sec id="j_joepi-2015-0032_s_003"><h3>Method and Material</h3><p>The German employer’s liability insurance association has recently published the new revised BGI 585 Risk Assessment Guidelines (DGUV information 250-001) framework and assistance in epilepsy in view of protection against unfair dismissal. These guidelines provide information on safety and health in the workplace.</p><p>Throughout all the German federal states, 24 Network teams were established. During the period January 2010 and December 2013, 374 employees with epilepsy were consulted by an expert member of Network Epilepsy and Work (NEA) Team, of which 80 were prospectively included in a study and scientifically evaluated.</p></sec><sec id="j_joepi-2015-0032_s_004"><h3>Guidelines and discussion</h3><p>While conducting the risk assessment, a special medical fact check in accordance with the guidelines was used. In addition to medical aspects, the individual vocational and occupational situation was considered. Based on this assessment an individual recommendation was made relating to continuation of employment. The project NEA established regional teams of physicians, therapists, consultants from social services, employment offices and rehabilitation authorities across Germany in order to link by networks the complex medical and social aspects of reducing the risk of people with epilepsy losing their job.</p></sec><sec id="j_joepi-2015-0032_s_005"><h3>Results</h3><p>It was shown that support and consultation through the NEA team led to an endangered position of employment being maintained in 70% of cases.</p></sec><sec id="j_joepi-2015-0032_s_006"><h3>Conclusion</h3><p>In many cases, loss of employment can be prevented by consequent application of DGUV information 250-001 (recently revised from BGI 585) for risk assessment of epilepsy in employment, together with improved networking between medical professionals, occupational health professionals and social services.</p></sec></abstract>

Diagnosis of epilepsy - consequences for work and professional activities

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2015-0033_s_001"><h3>Introduction</h3><p>Eyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy.</p></sec><sec id="j_joepi-2015-0033_s_002"><h3>Aim and method</h3><p>The aim is to consider the updated electro-clinical pathophysiology and to discuss terminology, classification and differential diagnosis. The review includes our own research and relevant papers on the subject of Jeavons syndrome (JS).</p></sec><sec id="j_joepi-2015-0033_s_003"><h3>Review and differential diagnosis</h3><sec id="j_joepi-2015-0033_s_004"><h3>Definition</h3><p>eavons syndrome is a generalized idiopathic (genetic) epilepsy syndrome (IGE) characterized by eyelid myoclonia, other seizures (absences, myoclonic and or generalized tonic-clonic) and EEG paroxysms induced by voluntary or on command eye closure, in the light and photosensitivity.</p></sec></sec><sec id="j_joepi-2015-0033_s_005"><h3>Demographical data</h3><p>The prevalence of JS has been reported to vary from 7.3% to 12.9% among idiopathic generalized epilepsies and 2.5% to 2.7% among all patients with epileptic disorders.</p></sec><sec id="j_joepi-2015-0033_s_006"><h3>Etiology</h3><p>JS, as is the case for all idiopathic generalized epilepsies, is genetic and the familial preponderance and concordance is high.</p><sec id="j_joepi-2015-0033_s_007"><h3>Pathophysiology</h3><p>Three factors are important in order for JS to manifest clinically; the genetic predisposition, the voluntary or on command eye closure and the light input.</p></sec></sec><sec id="j_joepi-2015-0033_s_008"><h3>Clinical forms of JS</h3><p>we have identified four forms of JS; early onset (&lt; 4 years), mild form, classical form and an ELMA-JME form.</p></sec><sec id="j_joepi-2015-0033_s_009"><h3>Diagnosis</h3><p>the diagnosis of JS is based on the history, clinical observation and provocation and the confirmation with an EEG.</p></sec><sec id="j_joepi-2015-0033_s_010"><h3>Differential diagnosis</h3><p>is easily made from tics, other idiopathic generalized or focal cryptogenic/symptomatic epilepsies.</p></sec><sec id="j_joepi-2015-0033_s_011"><h3>Conclusion</h3><p>JS is characterized by unique electro-clinical features evoked by voluntary or on command eye closure in the light and photosensitivity.</p></sec></abstract>

Jeavons syndrome – updated review

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2015-0034_s_001"><h3>Introduction</h3><p>The treatment of epilepsy is still a major challenge. Despite the introduction of many new antiepileptic drugs, approximately 30% of patients still remain drug resistant. In the absence of a satisfactory therapy outcome, which is sometimes associated with numerous side effects, there is a need for new and effective drugs with low toxicity. Cannabinoids have been shown in preliminary animal model studies and in studies of patients with epilepsy to have antiepileptic activity.</p></sec><sec id="j_joepi-2015-0034_s_002"><h3>Aim</h3><p>The aim of this paper is to review current reports on the role of the endocannabinoid system and cannabinoids in the treatment of epilepsy.</p></sec><sec id="j_joepi-2015-0034_s_003"><h3>Methods</h3><p>Articles from PubMed and Scopus published up to 2015 concerning the role of cannabinoids and the endocannabinoid system in the treatment of epilepsy are reviewed.</p></sec><sec id="j_joepi-2015-0034_s_004"><h3>Review and Discussion</h3><p>Cannabis has been used for thousands of years in the treatment of various diseases. They contain cannabinoids, which act on the endocannabinoid system which regulates many biochemical and physiological processes. By affecting glutamate and gamma-aminobutyric acid (GABA) neurotransmission cannabinoids have the ability to affect seizure threshold. The best known cannabinoid is cannabidiol, which inhibits the occurrence of seizures without causing significant side effects in humans and animals. However, only a small number of double blind, randomized and placebo controlled studies have been published to date.</p></sec><sec id="j_joepi-2015-0034_s_005"><h3>Conclusions</h3><p>The role of cannabinoids in the treatment of epilepsy is not well defined because these substances have shown pro-convulsive actions in some animal studies and also there are not many randomized trials performed to date. The existing human data do not support the conclusion that cannabinoids are effective and safe in the treatment of epilepsy, but do encourage further studies on a larger group of patients.</p></sec></abstract>

The role of cannabinoids and endocannabinoid system in the treatment of epilepsy

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2015-0035_s_001"><h3>Introduction</h3><p>Excessive accumulation of amyloid-beta (Aβ) peptides in the brain results initially in mild cognitive impairment (MCI) and finally in Alzheimer’s disease (AD). Evidences from experimental and clinical studies show that pathological hyperexcitability of hippocampal neurons is a very early functional impairment observed in progressive memory dysfunctions. Therefore, antiepileptic drugs (AEDs) whose mechanism of action is aimed at inhibition of such neuronal hyperexcitability, seems to be an rationale choice for MCI and AD treatment.</p></sec><sec id="j_joepi-2015-0035_s_002"><h3>Aim</h3><p>To provide data from experimental and clinical studies on: 1. The unfavorable impact of neuronal hyperexcitability, mainly within the hippocampus, on cognitive processes. 2. Efficacy of AEDs against such abnormally elevated neuronal activity for the prevention of progressive cognitive impairment.</p></sec><sec id="j_joepi-2015-0035_s_003"><h3>Methods</h3><p>A literature review of publications published within the last fifteen years, was conducted using the PubMed database.</p></sec><sec id="j_joepi-2015-0035_s_004"><h3>Review</h3><p>The authors describe Aβ-induced hyperexcitability of hippocampal nerve cells as the cause of cognitive deficits, the connection of such activity with an increased risk of seizures and epilepsy in patients with MCI/AD, and finally the efficacy of AEDs: valproic acid (VPA), phenytoin (PHT), topiramate (TPM), lamotrigine (LTG), ethosuximide (ESM) and levetiracetam (LEV) in the prevention of cognitive impairment in experimental models and patients with MCI/AD.</p></sec><sec id="j_joepi-2015-0035_s_005"><h3>Conclusions</h3><p>The majority of the studied AEDs improve cognitive dysfunction in various experimental models of Aβ-induced brain pathology with accompanied neuronal hyperexcitability. The promising results achieved for LEV in animal models of cognitive impairment were also confirmed in patients with MCI/AD. LEV was well-tolerated and it’s beneficial antidementive effect was confirmed by memory tests and fMRI examination. In conclusion, the use of AEDs could be a novel therapeutic concept for preventing cognitive impairment in patients with Aβ-associated brain pathology.</p></sec></abstract>

Antiepileptic drugs as a new therapeutic concept for the prevention of cognitive impairment and Alzheimer’s disease. Recent advances

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2015-0036_s_001"><h3>Introduction</h3><p>Epidemiological studies on epilepsy were long based, with few exceptions, on hospital and institution patients with a subsequent bias toward more difficult cases and the reported prevalence and incidence rates were often obviously too low. Few data are available on the temporal changes in the incidence of epilepsy.</p></sec><sec id="j_joepi-2015-0036_s_002"><h3>Aim</h3><p>To study the prevalence and incidence in an unselected child population including all the children living either in the society or in the institution, temporal changes in the incidence and mortality through five decades.</p></sec><sec id="j_joepi-2015-0036_s_003"><h3>Methods</h3><p>The most important personal data were reviewed and compared with the relevant data of other investigators.</p></sec><sec id="j_joepi-2015-0036_s_004"><h3>Results and discussion</h3><p>The prevalence of epilepsy in our study was 3.2/1000, quite obviously true for the contemporary methodology and well comparable with 3.4-4.2/1000 of other relevant studies published about two decades later and using a more advanced methodology. Similarly, the incidence of 35/100 000, ascertained in two Finnish studies, was comparable with the relevant contemporary literature data. Another study of ours shows that, probably associated with the people “coming from the shadows” and an improved diagnostic methodology, the incidence of childhood epilepsy has increased and is now 60-70/100 000. However, the incidence of childhood epilepsy shows an obvious decreasing trend in the first two decades of the 2000s.</p></sec><sec id="j_joepi-2015-0036_s_005"><h3>Conclusions</h3><p>The incidence of childhood epilepsy, in all probability true for the contemporary methodology, was lower than it is now, but it now again shows a decreasing trend.</p></sec></abstract>

Epidemiology and long-term Turku outcome of childhood-onset epilepsy and mortality. Personal experiences. Part I<sup>*</sup>

AHEAD OF PRINT

Volume 32 (2024): Issue 1 (May 2024)

Volume 31 (2023): Issue 1 (November 2023)

Volume 29 (2021): Issue 1 (July 2021)

Volume 28 (2020): Issue 1 (December 2020)

Volume 27 (2019): Issue 1 (February 2019)

Volume 26 (2018): Issue 1-2 (June 2018)

Volume 25 (2017): Issue 1-2 (December 2017)

Volume 24 (2016): Issue 2 (December 2016)

Volume 24 (2016): Issue 1 (June 2016)

Volume 23 (2015): Issue 2 (December 2015)

Volume 23 (2015): Issue 1 (June 2015)

Volume 22 (2014): Issue 2 (December 2014)

Volume 22 (2014): Issue 1 (June 2014)

Volume 21 (2013): Issue 2 (December 2013)

Volume 21 (2013): Issue 1 (June 2013)

Journal of Epileptology

 The Journal of Epileptology – published since 2013 - is an international, open access peer reviewed scientific journal that publishes articles of clinical and experimental epilepsy.   The following categories of epilepsy related topics are accepted for publications: medical hypotheses, basic research, clinical research, reviews and special reports, product investigation, diagnostic and medical technology, case reports, letters to Editor, reports from international and local activities.   The aim of the journal is to widen the international community of readers, research and clinicians interested in various aspects of epilepsy and disseminate interdisciplinary knowledge in all fields related to epilepsy.   The Journal of Epileptology is owned by the Foundation of Epileptology which was established in 1993 by Professor Jerzy Majkowski. The Foundation of Epileptology is the first non-governmental organization in Poland whose priority is to improve the quality of life of persons with epilepsy by improving the diagnostic and therapeutic skills of doctors and by shaping a positive attitude to epilepsy among the public. The Foundation, led by this idea, brings together physicians, psychologists, scientists and academics, social activists and other people who wish to contribute to the development of epileptology and thus provide medical assistance to persons with epilepsy.     About Society   The Foundation of Epileptology was established in 1993 by Professor Jerzy Majkowski who also founded the Department of Neurology and Epileptology at the Medical Centre for Postgraduate Education (CMKP) in 1988. The Foundation of Epileptology is the first non-governmental organisation in Poland to have as its priority the improvement of quality of life of epileptic patients by means of furthering doctors' diagnostic and therapeutic mastery and developing a positive approach to epilepsy among the general public. The Foundation, led by this idea, attracts physicians, psychologists, researchers and academics, social activists and other persons who wish to contribute to the development of epileptology and thereby bring medical assistance to people suffering from epilepsy. The Foundation of Epileptology was registered in the Foundation Register of the District Court for the Municipality of Warsaw on 16 March 1993 and thereby acquired legal status. The Founder appointed the Foundation Council and the Foundation Executive Committee.   The Foundation of Epileptology co-operates with the Polish Society of Epileptology, the Warsaw-based Society for Victims of Epilepsy and their Friends "Calm Head" (Spokojna Głowa) and other domestic and foreign organisations with similar goals. The Foundation of Epileptology is a member of the European Epilepsy Academy (EUREPA) and Professor Majkowski is co-organiser and member of the first Executive Committee and is now a member of the EUREPA Research Advisory Committee. The Executive Committee of the Polish Society of Epileptology shares its headquarters with the Epilepsy Foundationv   Archiving   The archiving of issues published from 2013 to 2017 will occur, and thus all issues published in the current form of the Journal will become available on-line. However, the issues published quarterly under the journal’s earlier name of Epileptologia during 1993 to 2012 will be successively attached and published on journal’s website www.journal-epileptology.com.   Sciendo archives the contents of this journal in Portico - digital long-term preservation service of scholarly books, journals and collections.   Plagiarism Policy   The editorial board is participating in a growing community of Similarity Check System's users in order to ensure that the content published is original and trustworthy. Similarity Check is a medium that allows for comprehensive manuscripts screening, aimed to eliminate plagiarism and provide a high standard and quality peer-review process. 