<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2017-0001_s_001"><h3>Introduction</h3><p>The differentiation between the clinical and electroencephalographic changes in noncon-vulsive status epilepticus (NCSE) and those in sporadic Creutzfeldt-Jakob disease (sCJD) is a crucial question.</p></sec><sec id="j_joepi-2017-0001_s_002"><h3>Case report</h3><p>A 77-year old woman was admitted because of fluctuating behavioural chancaseges, adynamia and apraxia since several months for diagnostic. The diagnosis of sCJD was suggested. Subsequently, she had a generalized tonic clonic seizure (GTCS) and the EEG revealed periodic lateralized epileptiform discharges and NCSE was considered.</p></sec><sec id="j_joepi-2017-0001_s_003"><h3>Discussion</h3><p>The presented case illustrates the dilemma in the differential diagnosis of sCJD and (symptomatic) NCSE in the light of the recently published new Salzburg consensus criteria and unified EEG terminology. Concerning these criteria, the patient showed after an initial generalized seizure and substantial clinical improvement after administration of antiepileptic drugs, persisting epileptic discharges and only subtle clinical ictal phenomena during the EEG patterns with typical spatiotemporal evolution as a correlate of a symptomatic NCSE. During the further course of the disease in the presented patient the picture changed into an encephalopathic pattern.</p></sec><sec id="j_joepi-2017-0001_s_004"><h3>Conclusion</h3><p>EEG criteria for the diagnosis of NCSE are complex. In our case the EEG resembled the pattern of NCSE in the postictal phase of a GTCS according to a classification of NCSE in use at this time. After initial responsiveness to antiepileptic medication the patient lost responsiveness to therapy displaying the typical encephalopathic EEG findings in sCJD. These findings may support the hypothesis of initial NCSE and transformation into prion protein induced encephalopathic EEG and demonstrated clinical usefulness of the Salzburg consensus criteria for NCSE.</p></sec></abstract>

The diagnostic dilemma of non-convulsive status epilepticus in sporadic Creutzfeldt-Jakob disease

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2017-0002_s_001"><h3>Introduction</h3><p>There are some genetic disorders with combination of mental retardation, epilepsy and autism in which the abnormal mammalian Target of Rapamycin (m-TOR) signaling is implicated. The most important of them is tuberous sclerosis complex (TSC), but the disturbances of the m-TOR pathway can also be detected in Rett syndrome (RS), Fragile X syndrome and Down syndrome. We describe the rare case of co-occurrence of TSC and RS.</p></sec><sec id="j_joepi-2017-0002_s_002"><h3>Case study</h3><p>The female child was born at term by normal delivery after a non-complicated pregnancy. Family history was negative for epilepsy and mental retardation. The neonatal period was uneventful and psychomotor development was normal before the child became 1.5 years old. At the age of 18 months the girl developed hand-wringing stereotypes, facial hypotonia, ataxia and gait apraxia. She lost eye-to-eye contact and verbal contact with relatives, and became indifferent to the surrounding environment. When she was 2 years old, focal adversive seizures started which were readily controlled with carbamazepine. Cerebral cortical and subcortical tubers, cerebral white-matter radial migration lines and subependymal nodules on brain MRI together with hypomelanotic macules suggested the presence of TSC. Diagnosis was confirmed at age of 3 years by a heterozygous mutation c.5161-2A>G in <italic>TSC2</italic> gene on chromosome 16p13. But the rude regression of psychomotor development and speech, autistic features alongside with characteristic hand-wringing stereotypes were unexplained until at age of 4.5 years RS was diagnosed by finding a heterozygous missense mutation in exon 4 of the <italic>MECP2</italic> gene c.455C>T, resulting in a P152R substitution in the methyl-binding domain of the protein. At age of 5 the patient is not able to walk independently and has no expressive speech, she is autistic, has ataxia, limb rigidity, hyperreflexia, lack of purposeful hand movements, verbal and motor stereotypies.</p></sec><sec id="j_joepi-2017-0002_s_003"><h3>Discussion</h3><p>The presence of two mutations (one characteristic for <italic>TSC2</italic> and one – characteristic for RS) significantly worsened the developmental and motor delay and autistic features in our patient. Dysregulation of m-TOR way is well established in TSC and recently described in RS, Down syndrome and Fragile X syndrome.</p></sec></abstract>

Clinical report: a rare co-occurrence of tuberous sclerosis complex and Rett syndrome in a girl with mental retardation, epilepsy and autism

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2017-0001_s_001"><h3>Introduction</h3><p>An increasing body of evidence points to an important role of neuroinflammatory processes in the pathomechanism of epilepsy. This hypothesis is mainly supported by data showing an increase of pro-inflammatory cytokine levels and glia activation in animal models of epilepsy and in brain tissue of epileptic patients. On the other hand, less emphasis has been put on pharmacological verification of this hypothesis.</p></sec><sec id="j_joepi-2017-0001_s_002"><h3>Aim</h3><p>The aim of this review is to summarize current knowledge on potential usefulness of microglia regulators and anti-inflammatory agents in designing antiepileptic/antiepileptogenic drugs, with the primary mechanism of action based on the inhibition of neuroinflammation.</p></sec><sec id="j_joepi-2017-0001_s_003"><h3>Methods</h3><p>We reviewed PubMed and MEDLINE databases to select publications in the topic: epilepsy, neuroinflammation, microglia and microglia regulators with antiepileptic properties. We searched the databases up to April 2017 with no date restrictions.</p></sec><sec id="j_joepi-2017-0001_s_004"><h3>Review and Discussion</h3><p>In the present paper, we will discuss new concepts of epileptogenesis which focus not only on changes in neurons but also take into consideration the role of activation of glial cells: microglia and astrocytes. Neuroinflammation, mainly through increased production of pro-inflammatory factors such as cytokines or chemokines, may play an important role in the development of epilepsy. Drugs regulating glial cells activation and consequently inflammatory status in the central nervous system have beneficial effects in different animal models of epilepsy as well as in clinical study in patients. The most promising compound seems to be minocycline which in some studies has been shown to possess antiepileptogenetic action. On the other hand, some antiepileptic drugs exhibit marked anti-inflammatory potency.</p></sec><sec id="j_joepi-2017-0001_s_005"><h3>Conclusions</h3><p>There are much data to suggest that there is significant opportunity for designing new antiepileptic drugs whose primary mechanism of action entails the inhibition of neuroinflammatory processes.</p></sec></abstract>

Do minocycline and other suppressors of microglia reactivity have a future in prevention or treatment of epilepsy?

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2017-0004_s_001"><h3>Background</h3><p>Epilepsy is among the most frequent neurological conditions and it is estimated that approximately 8% of the population experience a seizure at some time in their lives.</p></sec><sec id="j_joepi-2017-0004_s_002"><h3>Aim</h3><p>To examine the characteristics of patients referred to a First Seizure Clinic (FSC) at a University Hospital in South-West London.</p></sec><sec id="j_joepi-2017-0004_s_003"><h3>Methods</h3><p>All subjects referred to the FSC at St George’s University Hospitals between January and December 2015 were included in this audit.</p></sec><sec id="j_joepi-2017-0004_s_004"><h3>Results</h3><p>From a total of 257 patients, males 49.5%, age range 16–90, 30% referred by General Practices (GPs), 59.1% by the Accident &amp; Emergency Department (A&amp;E) and 10.9% by other hospital wards, 24.5% did not attend (DNA) the clinical appointment. Females who did not attend were significantly older than males (49.8 years old vs 39.7; p = 0.007). Among those who attended the clinical appointment, 17% were diagnosed first unprovoked seizure, 12.4% acute symptomatic seizure and 28.9% epilepsy. These patients were referred mainly by A&amp;E while GPs referred seizure mimics especially non-epileptic attack disorder (NEAD) and syncope. Patients with NEAD were significantly younger than those with seizures (29.4 years old vs 44.2; p &lt; 0.001) and had a previous psychiatric history (72.7% vs 16.8%; p &lt; 0.001). The proportion of seizure mimics was similar in the older sample group (> 65 years). Regarding acute symptomatic seizures, 33.3% were alcohol-related, 20.8% acute brain insults and 12.5% drug-related (always overdose).</p></sec><sec id="j_joepi-2017-0004_s_005"><h3>Conclusions</h3><p>1 in 4 patients referred to a FSC does not attend the clinical appointment, especially older females. More than 1 in 3 cases represent seizure mimics and are referred mainly by GPs.</p></sec></abstract>

Diagnoses and referral pattern at a first seizure clinic in London

<abstract xmlns="http://www.w3.org/1999/xhtml"><p>The International Bureau for Epilepsy (IBE) Executive Committee for the term 2013-2017 began in June 2013 during the 30<sup>th</sup> International Epilepsy Congress in Montreal. From the beginning, our primary goals were to fulfil the mission of our organisation and address problems such as awareness, education, and social issues, while promoting and protecting the human rights of persons with epilepsy (PWE) and improving trans-regional equity in access to health care services, improved prevention, diagnosis and treatment and as a consequence, a reduction in the treatment gap and alleviation of stigma worldwide. By so doing, the quality of life of PWE and those who care of them will be significantly improved. In order to achieve these aims, the IBE joined forces with the International League Against Epilepsy (ILAE) and also the World Health Organisation (WHO), regional and national IBE organisations and other stakeholders.</p><p>In addition, the participation of the IBE President in many national epilepsy events worldwide has helped to promote care and human rights of PWE nationally. A major awareness event accomplished during our term in office was the launch of the International Epilepsy Day in 2015. An IBE-ILAE event celebrated the 2<sup>nd</sup> Monday in February each year at the European Parliament with the participation of many stakeholders, PWE and advocates for epilepsy MEPs.</p><p>In 2014 in order to improve access to care, treatment, appropriate treatment for PWE worldwide, particularly in developing countries, we developed our strategy plan becoming IBE’s roadmap up to 2019 and with strategic priorities to establish epilepsy as a health priority worldwide. In June 2014, in Troina, Italy, WHO, ILAE, IBE and the Global Outreach Research Task Force organised a workshop to discuss how to improve access to antiepileptic medicines in low- and middle-income countries and, as a consequence, to produce a white paper. A unique historical achievement during our term in office was the approval from WHO and subsequently from World Health Assembly (WHA) (May 26<sup>th</sup>, 2015) the Resolution on the Burden of epilepsy, and calls on United Nation Member States to implement the WHA68.20 actions and for WHO to report back in 2018. The creation by IBE/ILAE in 2016 the legal entity Epilepsy Alliance Europe has given the opportunity for both organizations to play a partnership role in many research projects aiming to reduce the epilepsy burden in Europe and worldwide.</p></abstract>

Actions related to International Bureau for Epilepsy during my term as President 2013–2017

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2017-0006_s_001"><h3>Background</h3><p>Adenosine is regarded as an endogenous anticonvulsant and its agonists have been proved to affect the anticonvulsant activity of a number of antiepileptic drugs (AEDs) in animal models of seizures.</p></sec><sec id="j_joepi-2017-0006_s_002"><h3>Aim</h3><p>To evaluate effects of adenosine agonists on carbamazepine (CBZ) and valproate (VPA) in mouse model of generalized tonic-clonic convulsions.</p></sec><sec id="j_joepi-2017-0006_s_003"><h3>Methods</h3><p>The following adenosine receptor agonists were used: A<sub>1</sub> – cyclohexyladenosine, A<sub>2A</sub> – CGS 21 680, A<sub>3</sub> – N<sup>6</sup>-benzyl-NECA and A<sub>1</sub> (preferentially) and A<sub>2</sub> – 2-chloroadenosine. Their possible anticonvulsant effects were studied in a threshold electroconvulsive test for maximal electroconvulsions. The protective activity of AEDs alone or in combinations with adenosine agonists was evaluated in the form of their respective ED<sub>50</sub> values necessary to protect 50% of mice against tonic extension of the hind limbs, following maximal electroshock, delivered through ear electrodes. The specificity of interactions between AEDs and adenosine agonists was challenged with an adenosine receptor A<sub>1</sub> and A<sub>2</sub> antagonist, aminophylline (5 mg/kg). The effects of AEDs alone or with adenosine agonists were tested for the occurrence of adverse effects (AE) (impairment of motor coordination) in a chimney test. All combinations with an enhancement the protective activity of CBZ or VPA were verified with the free plasma or brain concentration of these AED.</p></sec><sec id="j_joepi-2017-0006_s_004"><h3>Results</h3><p>Adenosine receptor agonists (cycloheksyladenosine up to 4 mg/kg; CGS 21 680 – 8 mg/kg; N<sup>6</sup>-benzyl-NECA – 1 mg/kg; 2-chloroadenosine – 2 mg/kg) did not significantly affect the threshold for maximal electroconvulsions. Cycloheksyladenosine (1 mg/kg), N<sup>6</sup>-benzyl-NECA (0.5 and 1 mg/kg) and 2-chloroadenosine (1 mg/kg) potentiated the anticonvulsant activity of CBZ. Valproate’s protective action was enhanced by one adenosine agonist – cycloheksyladenosine (1 mg/kg). Only the combination of CBZ + N<sup>6</sup>-benzyl-NECA (1 mg/kg) was resistant to aminophylline (5 mg/kg). Pharmacokinetic interactions were evident in case of the combination of CBZ + N<sup>6</sup>-benzyl-NECA (1 mg/kg) and resulted in an increased free plasma concentration of this CBZ. Interestingly, total brain concentration of CBZ confirmed the pharmacokinetic interaction as regards CBZ + N<sup>6</sup>-benzyl-NECA (1 mg/kg).</p></sec><sec id="j_joepi-2017-0006_s_005"><h3>Conclusion</h3><p>The best profile was shown by the combination of CBZ + 2-chloroadenosine which involved no AE or a pharmacokinetic interaction. The remaining positive combinations in terms of anticonvulsant activity were associated with general profound AE and pharmacokinetic interactions in some of them.</p></sec></abstract>

Adenosine receptor agonists differentially affect the anticonvulsant action of carbamazepine and valproate against maximal electroshock test-induced seizures in mice

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. 