Status epilepticus (SE) is an acute, one of the most common, life threatening emergency conditions in children and adolescence. According to the ILAE commission, SE is defined as a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally, prolonged seizures. Regarding duration, ILAE defines convulsive SE as bilateral tonic–clonic lasting longer that 5 minutes, and absence and focal status epilepticus as exceeding 10 minutes. Children, compared to adults, are more prone to epileptic seizures and, as a consequence, to SE, mainly due to age related maturational imbalance between excitatory and inhibitory synaptic mechanisms. The classification into generalised or focal, convulsive and nonconvulsive differs widely in aetiology, management and outcome. Early prehospital intervention with appropriate protective measures and emergency medication of seizure disorder, may prevent the perpetuation of seizure recurrence. If first line treatment fails and/or is an event, emergency hospital admission should be provided for more aggressive intravenous therapy and assessment-support.

Periodic electroencephalographic (EEG) patterns are frequently recorded during ICU EEG monitoring in patients with altered mental status; these EEG features represent electrical discharges, ictal in appearance, occuring at regular intervals. They are known as lateralized periodic discharges (LPDs), bilateral independent periodic discharges (BIPDS), generalized periodic discharges (GPDs), continuous 2/s GPDs with triphasic morphology or triphasic waves (TWs) and Stimulus Induced Evolving Lateralized Rhytmic delta activity or Si-Evolving LRDA (previously SIRPIDS); other periodic, rhythmic patterns are Occasional frontally predominant brief 2/s GRDA (FIRDA previously), Lateralized rhythmic delta activity (LRDA) and Brief potentially ictal rhythmic discharges or B (I)RDs. The role of most (not all) of these EEG patterns is controversial; there is no consensus on which patterns are associated with ongoing seizure injury, which patterns need to be treated, and how aggressively they should be treated. Many authors consider these patterns as an unstable state on an ictal-interictal EEG continuum; the aim of the present chapter is to gain knowledge of these EEG features, show their association with known neurologic pathologies/syndromes and finally how to manage them.

OBJECTIVE:To test the hypothesis that absence seizures can evolve to generalized tonic-clonic seizures, we documented electroclinical features of this novel seizure type.METHODS:In 4 large video-EEG databases, we identified recordings of seizures starting with impaired awareness that, without returning to baseline interictal state, evolved to generalized tonic-clonic seizures. We extracted the detailed semiologic and electrographic characteristics of these seizures, and we documented the clinical background, diagnoses, and therapeutic responses in these patients.RESULTS:We identified 12 seizures from 12 patients. All seizures started with a period of impaired awareness and bursts of generalized spike or polyspike and slow-wave discharges, the hallmark of absence seizures. Without returning to baseline, the nonmotor (absence) phase was followed by tonic-clonic convulsions. We called this novel generalized seizure type absence-to-bilateral-tonic-clonic seizure. Most patients had idiopathic generalized epilepsies, although with a high incidence of unusual features and poor therapeutic response.CONCLUSIONS:Absence-to-bilateral-tonic-clonic seizures are a novel generalized seizure type. Clinicians should be aware of this seizure for correctly diagnosing patients. This novel seizure type may further elucidate generalized ictogenesis.

Objective To test the hypothesis that absence seizures can evolve to generalized tonic-clonic seizures, we documented electroclinical features of this novel seizure type. Methods In 4 large video-EEG databases, we identified recordings of seizures starting with impaired awareness that, without returning to baseline interictal state, evolved to generalized tonic-clonic seizures. We extracted the detailed semiologic and electrographic characteristics of these seizures, and we documented the clinical background, diagnoses, and therapeutic responses in these patients. Results We identified 12 seizures from 12 patients. All seizures started with a period of impaired awareness and bursts of generalized spike or polyspike and slow-wave discharges, the hallmark of absence seizures. Without returning to baseline, the nonmotor (absence) phase was followed by tonic-clonic convulsions. We called this novel generalized seizure type absence-to-bilateral-tonic-clonic seizure. Most patients had idiopathic generalized epilepsies, although with a high incidence of unusual features and poor therapeutic response. Conclusions Absence-to-bilateral-tonic-clonic seizures are a novel generalized seizure type. Clinicians should be aware of this seizure for correctly diagnosing patients. This novel seizure type may further elucidate generalized ictogenesis.

Abnormalities of muscle tone, movement, and motor skills are the hallmark of cerebral palsy (CP) which results from injury to the developing brain.Clinically, the syndrome evolves over time and may only be apparent after 3-5 years of age, although suggestive signs and symptoms may be present at an earlier age.Epilepsy is common in CP and occurs in about 30% of patients.Generally, the onset is within the first 2 years of life.Epilepsy is commonly observed in children with spastic hemiplegia, followed by quadriplegia and diplegia.Significant risk factors for the development of epilepsy in patients with CP are family history, neonatal seizure, structural abnormalities, low Apgar scores, and mental retardation.Focal to bilateral tonic-clonic seizures are the most prominent seizure types, followed by focal aware or impaired awareness seizures, while infantile spasms and myoclonic jerks are seen in 25% of cases.Mental retardation is a predisposing factor for early onset of seizures and more severe epilepsy.The overall outcome of seizures in children with CP is generally poor, requiring prolonged course of antiepileptic medication, usually polytherapy with higher incidence of refractory seizures, side effects, comorbidities, and hospital admissions for drug-resistant seizures or status epilepticus.

Summary The International Bureau for Epilepsy (IBE) Executive Committee for the term 2013–2017 began in June 2013 during the 30 th 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. 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 nd Monday in February each year at the European Parliament with the participation of many stakeholders, PWE and advocates for epilepsy MEPs. 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 th , 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.

When Hanneke de Boer died peacefully in her own home after a long illness faced with great dignity a light was extinguished on a life devoted to sufferers with epilepsy. Hanneke was one of the longest serving and greatest advocates of people with epilepsy of all ages, not only in her much loved country, The Netherlands, but across the world. Hanneke was born in 1946 and at the age of 19 she first joined SEIN (Stichting Epilepsie Instellingen Nederland) as a vocational consultant, where she remained until shortly before her death. She was committed to empowering people with epilepsy to find employment by interacting personally with them and with potential employers. She was also engaged in the lives of her patients and often freely provided support to those struggling with their education courses. Hanneke first became involved with IBE when she was appointed Chair of the Employment Commission in 1983. She became Editor of IE News in 1985. She served as IBE Secretary General from 1989 to 1993, as President from 1993 to 1997 and as Past President from 1997 to 2001. She never fully retired from her work and until recently was still a member of the Joint ILAE-IBE Global Outreach Task Force and Chair of the IBE Legislation Task Force. She received an impressive series of awards, including the Ambassador for Epilepsy Award (1987), the IBE-ILAE Social Accomplishment Award (2005) and the Lifetime Achievement Award (2009), the most prestigious of all ILAE and IBE recognitions. In her own country, she was inducted as an Officer of the Orange Order of Nassau by the Royal Dutch House, one of the highest (and rarely given) awards presented in the Netherlands. Each of us had the privilege to collaborate with Hanneke, one of us for 25 years from 1989 to 2014; ER and Hanneke worked particularly closely as Presidents respectively of ILAE and IBE between 1993 and 1997 and then in promoting the ILAE/IBE/WHO Global Campaign, to which Hanneke was especially committed. It is fair to say that all the Officers of ILAE and IBE that we have met over many years were committed to the objectives of the League and the Bureau on behalf of people with epilepsy, some more so than others, but none more so than Hanneke. Hanneke's dedication to promoting the needs and advancing the hopes and aspirations of people with epilepsy and their families by personal and collective action is testified not only by her track record, but also by our own personal observations and reflections. At the professional level she had an extraordinary capacity for detailed attention to agendas, priorities, plans of action, and minutes. But implementing such actions and goals required considerable diplomatic and social skills, which she combined with a steely determination. In large joint initiatives there are invariably some dissenting voices, but during the many collaborative endeavours in which we worked together, relations between the League and the Bureau were excellent, in large measure due to Hanneke's patient and diplomatic attention to everyone's point of view and her persuasive powers of keeping everyone on board. Hanneke was not a revolutionary, but she was cautiously open to new ideas. She was initially sceptical when the Global Campaign was first proposed, fearing it would undermine the finances of IBE which were always less healthy than those of ILAE. But once persuaded of the merits of the concept and the necessary safeguards, she became the most persistent and vocal advocate of the Campaign, which benefitted enormously from her detailed planning skills and determined diplomacy. We had great fun together, assisted by many colleagues, developing and launching the various “Declarations” on Epilepsy in five different continents between 1998 and 2000, culminating in the launch of the second phase of the Campaign in Geneva in 2001 supported by the new Director General of WHO, Dr. Gro Harlem Brundtland, whose speech on that occasion was a milestone in the social history of epilepsy. Hanneke had a particular interest in empowering people with epilepsy in Africa, but she also travelled to China on many occasions and played a major role in SEIN's application to become a WHO Collaborating Centre, working closely with the China Association Against Epilepsy. Latin America was another region in which Hanneke took an interest, especially in Chile and Colombia. She spoke in the Colombian Parliament to support efforts for introducing employment legislation to protect people with epilepsy. We remember the many planning visits that we had with Hanneke to WHO headquarters in Geneva, where just mentioning her name elicited great respect, admiration and affection. It is sad that, because of her illness, she could not participate in the considerable efforts leading to the 2015 World Health Assembly Resolution on Epilepsy, although we are sure she was deeply supportive and proud of the outcome, a natural extension of the Global Campaign. We never saw Hanneke display anger, however frustrated by obstinate colleagues; and she could occasionally be obstinate herself. In the face of unresolvable divergence of views one of her techniques was to withdraw from communication. But she always soon emerged as her usual friendly self, often with some new approach to the problem. However, when she faced her final challenge she again withdrew from her numerous concerned colleagues and friends, sadly not to re-emerge, but leaving us full of admiration for her life, career and legacy, almost wholly devoted to the care of people with epilepsy at the local, national and international level. Notwithstanding some early family adversity, Hanneke had a kind, friendly and generous nature. Wherever she travelled in the world she arrived bearing Dutch gifts, mostly from the workshops of SEIN. Every spring our gardens blossom with flowers of different shapes and colours which sprout from the Dutch bulbs that Hanneke gave us, and will remain a lasting memory of her kindness. When the first granddaughter of one of us was born 7 years ago, Hanneke promptly sent a pair of baby clogs, which was again typical of her generosity. Many of us felt we were part of her wider family. The door to her home in Haarlem, where she lived with her beloved cat, was always open. Countless people whom she will have met during her work, from all corners of the world, were made welcome. She loved to cook and she enjoyed a glass of red wine. Most of all, she loved to share these with her guests. This is more than an obituary for Hanneke. It is also an appreciation, a dedication, indeed an attempt at a celebration of a remarkable individual who dedicated her life to improve the quality of life of people with epilepsy. She will be deeply missed, but her memory will always continue to inspire our epilepsy community.

Summary Introduction. Eyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy. Aim and method. 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). Review and differential diagnosis. Definition : Jeavons 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. Demographical data : 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. Etiology : JS, as is the case for all idiopathic generalized epilepsies, is genetic and the familial preponderance and concordance is high. Pathophysiology : 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. Clinical forms of JS : we have identified four forms of JS; early onset (< 4 years), mild form, classical form and an ELMA-JME form. Diagnosis : the diagnosis of JS is based on the history, clinical observation and provocation and the confirmation with an EEG. Differential diagnosis : is easily made from tics, other idiopathic generalized or focal cryptogenic/symptomatic epilepsies. Conclusion. JS is characterized by unique electro-clinical features evoked by voluntary or on command eye closure in the light and photosensitivity.

Photosensitivity in epilepsy is common and has high heritability, but its genetic basis remains uncertain. Galizia et al. reveal an overrepresentation of unique variants of CHD2 — which encodes the transcriptional regulator ‘chromodomain helicase DNA-binding protein 2’ — in photosensitive epilepsies, and show that chd2 knockdown in zebrafish causes photosensitivity.

PurposeTo determine clinical phenotypes, evolution and genetic background of a large family with a combination of two unusual forms of reflex epilepsies.MethodPhenotyping was performed in eighteen family members (10 F, 8 M) including standardized EEG recordings with intermittent photic stimulation (IPS). Genetic analyses (linkage scans, Whole Exome Sequencing (WES) and Functional studies) were performed using photoparoxysmal EEG responses (PPRs) as affection status.ResultsThe proband suffered from speaking induced jaw-jerks and increasing limb jerks evoked by flickering sunlight since about 50 years of age. Three of her family members had the same phenotype. Generalized PPRs were found in seven members (six above 50 years of age) with myoclonus during the PPR.Evolution was typical: Sensitivity to lights with migraine-like complaints around adolescence, followed by jerks evoked by lights and spontaneously with dropping of objects, and strong increase of light sensitivity and onset of talking induced jaw jerks around 50 years.Linkage analysis showed suggestive evidence for linkage to four genomic regions. All photosensitive family members shared a heterozygous R129C mutation in the SCNM1 gene that regulates splicing of voltage gated ion channels. Mutation screening of 134 unrelated PPR patients and 95 healthy controls, did not replicate these findings.ConclusionThis family presents a combination of two rare reflex epilepsies. Genetic analysis favors four genomic regions and points to a shared SCNM1 mutation that was not replicated in a general cohort of photosensitive subjects. Further genetic studies in families with similar combination of features are warranted.

Dear Editor-in-Chief I would like to thank Striano for giving me the opportunity to write a few words on Radovici or Jeavons. There is no doubt that Radovici (1932, 1996) first described a case that could be classified as eyelid myoclonia and absences (seizure type) and or as self-induced epilepsy (Jeavons and Harding, 1975), in the same way we diagnose and classify them today. Furthermore, in those days when the EEG was at early stages of development, clinical observations were impossible to be matched with EEG findings. Even in recent years eminent epileptologists misdiagnosed Jeavons syndrome and eyeclosure sensitivity is seen in some EEG figure of Janz and Christian’s original paper in JME (Stefan and Theodore, 2012). In the book of Wallace and Farrel in 2004 it is stated that the first case of eyelid myoclonia was recognized by Radovici but subsequently Jeavons described ELMA as a syndrome. Therefore, there is no doubt that Jeavons first put together the cluster of clinical and EEG characteristics and first stated that constitute a separate type of photosensitive epilepsy. In my recent paper (Covanis, 2015) my aim was to review eyelid myoclonia and absences as a syndrome, named Jeavons syndrome and add to numerous reports since 1977 in order for Jeavons syndrome to be reconized as such by the International Community. A recent report gives us hope (Fisher et al., 2015).

The authors regret that there was an error in the authorship of this paper. The author name containing the error has now been corrected to Stan F. van de Graaf. The authors would like to apologise for any inconvenience caused. Clinical and genetic analysis of a family with two rare reflex epilepsiesSeizure - European Journal of EpilepsyVol. 29PreviewEpileptic jaw jerks (orofacial myoclonus) provoked by reading is a rare, but known syndrome [1]. Isolated speaking-induced facial myoclonic jerks without provocation by reading and writing is even more special [1,2]. We describe a family with members who have both speaking induced jerks and reflex photosensitivity still active above 50 years of age; a phenotype that to our knowledge has never been reported. Full-Text PDF Open Archive

This document provides guidance on the use of valproate in girls and women of childbearing age from a joint Task Force of the Commission on European Affairs of the International League Against Epilepsy (CEA-ILAE) and the European Academy of Neurology (EAN), following strengthened warnings from the Coordination Group for Mutual Recognition and Decentralised Procedures-Human (CMDh) of the European Medicines Agency (EMA), which highlight the risk of malformations and developmental problems in infants who are exposed to valproate in the womb. To produce these recommendations, the Task Force has considered teratogenic risks associated with use of valproate and treatment alternatives, the importance of seizure control and of patient and fetal risks with seizures, and the effectiveness of valproate and treatment alternatives in the treatment of different epilepsies. The Task Force's recommendations include the following: (1) Where possible, valproate should be avoided in women of childbearing potential. (2) The choice of treatment for girls and women of childbearing potential should be based on a shared decision between clinician and patient, and where appropriate, the patient's representatives. Discussions should include a careful risk-benefit assessment of reasonable treatment options for the patient's seizure or epilepsy type. (3) For seizure (or epilepsy) types where valproate is the most effective treatment, the risks and benefits of valproate and other treatment alternatives should be discussed. (4) Valproate should not be prescribed as a first-line treatment for focal epilepsy. (5) Valproate may be offered as a first-line treatment for epilepsy syndromes where it is the most effective treatment, including idiopathic (genetic) generalized syndromes associated with tonic-clonic seizures. (6) Valproate may be offered as a first-line treatment in situations where pregnancy is highly unlikely (e.g., significant intellectual or physical disability). (7) Women and girls taking valproate require regular follow-up for ongoing consideration of the most appropriate treatment regimen.

Tuesday May 26, 2015, will be remembered as an historic day in the fight against epilepsy. On that date, the World Health Assembly approved unanimously the Resolution on the “Global Burden of Epilepsy and the Need for Coordinated Action at the Country Level to Address its Health, Social and Public Knowledge Implications,” which urges Member States to implement a coordinated action against epilepsy and its consequences. This event, which comes almost 20 years after the establishment of the Global Campaign against Epilepsy, is another landmark in the longstanding collaboration among the World Health Organization (WHO), the International League Against Epilepsy (ILAE), and the International Bureau for Epilepsy (IBE) in addressing the needs of people with epilepsy. It also acted as a catalyst for other professional societies, including the World Federation of Neurology (WFN), to join forces in promoting a common action against epilepsy. The Resolution did not happen by chance, but came at the end of a long journey that involved the hard and tireless work of many dedicated individuals around the globe.

Epileptic encephalopathies represent a group of devastating epileptic disorders that appear early in life and are characterized by pharmacoresistant generalized or focal seizures, persistent severe EEG abnormalities, and cognitive dysfunction or decline. The ictal and interictal epileptic discharges are age-specific and are either the main cause or contribute to cognitive deterioration in the idiopathic or symptomatic group respectively. Despite choosing the most appropriate anti-seizure drugs for the seizure-type and syndrome the results are often disappointing and polytherapy and/or alternative therapy becomes unavoidable. In those cases, consideration should be given to the quality of life of the child and carers. In this review we will discuss the clinical and EEG characteristics, evolution and management of age-related epileptic encephalopathies, recognized by the International League Against Epilepsy.

>间断性闪光刺激(IPS)是脑电图监测中常用的检测方法,旨在发现儿童及成人异常癫痫样放电对闪光的敏感性(即光敏感性)。但在临床实践中,不同的国家以及不同的脑电图室采用的IPS方法不同,导致结果有所差异。我们认为应该建立标准的检测方法,从而能够可重复地定性和定量检测光敏感性,更利于监测和识别癫痫和光敏感性患者。与1999年发表的方法相比较,新方法不仅增加了内容,而且对IPS检测技术以及相关的操作原理进行了详述。为了提高有效性及实用性,该方法通过6年的欧洲神经病学及癫痫专