ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions
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Status Epilepticus Clinical Pathway
JOHNS HOPKINS ALL CHILDREN’S HOSPITAL Status Epilepticus Clinical Pathway 1 Johns Hopkins All Children's Hospital Status Epilepticus Clinical Pathway Table of Contents 1. Rationale 2. Background 3. Diagnosis 4. Labs 5. Radiologic Studies 6. General Management 7. Status Epilepticus Pathway 8. Pharmacologic Management 9. Therapeutic Drug Monitoring 10. Inpatient Status Admission Criteria a. Admission Pathway 11. Outcome Measures 12. References Last updated: July 7, 2019 Owners: Danielle Hirsch, MD, Emergency Medicine; Jennifer Avallone, DO, Neurology This pathway is intended as a guide for physicians, physician assistants, nurse practitioners and other healthcare providers. It should be adapted to the care of specific patient based on the patient’s individualized circumstances and the practitioner’s professional judgment. 2 Johns Hopkins All Children's Hospital Status Epilepticus Clinical Pathway Rationale This clinical pathway was developed by a consensus group of JHACH neurologists/epileptologists, emergency physicians, advanced practice providers, hospitalists, intensivists, nurses, and pharmacists to standardize the management of children treated for status epilepticus. The following clinical issues are addressed: ● When to evaluate for status epilepticus ● When to consider admission for further evaluation and treatment of status epilepticus ● When to consult Neurology, Hospitalists, or Critical Care Team for further management of status epilepticus ● When to obtain further neuroimaging for status epilepticus ● What ongoing therapy patients should receive for status epilepticus Background: Status epilepticus (SE) is the most common neurological emergency in children1 and has the potential to cause substantial morbidity and mortality. Incidence among children ranges from 17 to 23 per 100,000 annually.2 Prevalence is highest in pediatric patients from zero to four years of age.3 Ng3 acknowledges the most current definition of SE as a continuous seizure lasting more than five minutes or two or more distinct seizures without regaining awareness in between. -
1 ILAE Classification & Definition of Epilepsy Syndromes in the Neonate
ILAE Classification & Definition of Epilepsy Syndromes in the Neonate and Infant: Position Statement by the ILAE Task Force on Nosology and Definitions Authors: Sameer M Zuberi1, Elaine Wirrell2, Elissa Yozawitz3, Jo M Wilmshurst4, Nicola Specchio5, Kate Riney6, Ronit Pressler7, Stephane Auvin8, Pauline Samia9, Edouard Hirsch10, O Carter Snead11, Samuel Wiebe12, J Helen Cross13, Paolo Tinuper14,15, Ingrid E Scheffer16, Rima Nabbout17 1. Paediatric Neurosciences Research Group, Royal Hospital for Children & Institute of Health & Wellbeing, University of Glasgow, Member of European Reference Network EpiCARE, Glasgow, UK. 2. Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester MN, USA. 3. Isabelle Rapin Division of Child Neurology of the Saul R Korey Department of Neurology, Montefiore Medical Center, Bronx, NY USA. 4. Department of Paediatric Neurology, Red Cross War Memorial Children’s Hospital, Neuroscience Institute, University of Cape Town, South Africa. 5. Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesu’ Children’s Hospital, IRCCS, Member of European Reference Network EpiCARE, Rome, Italy 6. Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia. Faculty of Medicine, University of Queensland, Queensland, Australia. 7. Clinical Neuroscience, UCL- Great Ormond Street Institute of Child Health, London, UK. Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children NHS Foundation Trust, Member of European Reference Network EpiCARE London, UK 8. Université de Paris, AP-HP, Hôpital Robert-Debré, INSERM NeuroDiderot, DMU Innov-RDB, Neurologie Pédiatrique, Member of European Reference Network EpiCARE, Paris, France. 9. Department of Paediatrics and Child Health, Aga Khan University, East Africa. 1 10. Neurology Epilepsy Unit “Francis Rohmer”, INSERM 1258, FMTS, Strasbourg University, France. -
Pediatric Epilepsy Clips by Pediatric Elizabeth Medaugh, MSN, CPNP-PC , Pediatric Nurse Practitioner, Neurology
Nursing May 2016 Pediatric Clips Pediatric Nursing Pediatric Epilepsy Clips by Pediatric Elizabeth Medaugh, MSN, CPNP-PC , Pediatric Nurse Practitioner, Neurology Advanced Practice Sophia had been exceeding down to sleep, mother heard a Nurses at Dayton Case Study the expectations of school. thump and went to check on her. Her teachers report her being Sophia’s eyes were wide open Children’s provides Sophia is a 7 year old Caucasian focused the first two quarters, and deviated left, left arm was quick reviews of female who presented to her but have found her attention to stiffened with rhythmic shaking, be somewhat off over the last Sophia’s mouth was described common pediatric primary care physician with concerns of incontinence few weeks. Teachers have asked as “sort of twisted” and Sophia conditions. in sleep. Sophia is an parents if Sophia has been was drooling. The event lasted otherwise healthy child, with getting good rest as she has 90 seconds. Following, Sophia uncomplicated birth history, been falling asleep in class. was disoriented and very sleepy. Father reports his sister Dayton Children’s appropriate developmental Sophia’s mother and father had similar events as a child. progression with regards note that she has had is the region’s Sophia’s neurological exam to early milestones, and several episodes of urinary was normal, therefore her pediatric referral previously diurnal/nocturnally incontinence each week over pediatrician made a referral toilet trained. No report of the last 3-4 weeks. Parents center for a to pediatric neurology. Given recent illness, infection or ill have limited fluids prior to bed Sophia’s presentation, what 20-county area. -
Benign Rolandic Epilepsy
Benign Rolandic Epilepsy What is Benign Rolandic Epilepsy (BRE)? Benign Rolandic Epilepsy is a common, mild form of childhood epilepsy characterized by brief simple partial seizures involving the face and mouth, usually occuring at night or during the early morning hours. Seizures are the result of brief disruptions in the brain’s normal neuronal activity. Who gets this form of epilepsy? Children between the ages of three and 13 years, who have no neurological or intellectual deficit.The peak age of onset is from 9-10 years of age. BRE is more common in boys than girls. What kind of seizures occur? Typically, the child experiences a sensation then a twitching at the corner of their mouth. The jerking spreads to in- volve the tongue, cheeks and face on one side, resulting in speech arrest, problems with pronunciation and drooling. Or one side of the child’s face may feel paralyzed. Consciousness is retained. On occasion, the seizure may spread and cause the arms and legs on that side to stiffen and or jerk, or it may become a generalized tonic-clonic seizure that involves the whole body and a loss of consciousness. When do the seizures occur? Seizures tend to happen when the child is waking up or during certain stages of sleep. Seizures may be infrequent, or can happen many times a day. How is Benign Rolandic Epilepsy diagnosed? A child having such seizures is given an EEG test to confirm the diagnosis, a test that graphs the pattern of electrical activity in the brain. BRE has a typical EEG spike pattern—repetitive spike activity firing predominantly from the mid-temporal or parietal areas of the brain near the rolandic (motor) strip. -
A Small Number of People Have What Is Known As Reflex Epilepsy, In
A small number of people have what is known What are the features of reflex epilepsy? as reflex epilepsy, in which seizures are set off There are many different types of reflex by specific stimuli. These can include flashing epilepsy, depending on the area of the brain that lights, a flickering computer “monitor”, sudden is affected. Seizure stimuli may be very specific, noises, a particular piece of music, or the phone or they may be broad categories. They can ringing. Some people even have seizures when include: they think about a particular subject or see their own hand! flashing or flickering lights, including computer monitors or video games. This is What are other terms for reflex epilepsy? called photosensitive epilepsy. It is the most Other terms for reflex epilepsy that you may common childhood form of reflex epilepsy, come across include: and the child may eventually outgrow it. the sight of a particular pattern; this is called epilepsy with reflex seizures visual pattern reflex epilepsy sensory precipitation epilepsy thinking about certain things stimulus sensitive epilepsy performing a certain task, such as typing; this is called praxis-induced epilepsy There are also many terms for specific types of reading reflex epilepsy. language-related stimuli such as writing, listening to speech, singing, or reciting What causes reflex epilepsy? certain passages of music; this is called The seizure stimulus is not the underlying cause musicogenic epilepsy of the epilepsy. Rather, it sends a particular certain sounds; this is called audiogenic message to a sensitive, seizure-prone area of the epilepsy brain, excites the neurons there, and causes a surprises or startles seizure. -
EEG in the Diagnosis, Classification, and Management of Patients With
EEG IN THE DIAGNOSIS, J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2005.069245 on 16 June 2005. Downloaded from CLASSIFICATION, AND MANAGEMENT ii2 OF PATIENTS WITH EPILEPSY SJMSmith J Neurol Neurosurg Psychiatry 2005;76(Suppl II):ii2–ii7. doi: 10.1136/jnnp.2005.069245 he human electroencephalogram (EEG) was discovered by the German psychiatrist, Hans Berger, in 1929. Its potential applications in epilepsy rapidly became clear, when Gibbs and Tcolleagues in Boston demonstrated 3 per second spike wave discharge in what was then termed petit mal epilepsy. EEG continues to play a central role in diagnosis and management of patients with seizure disorders—in conjunction with the now remarkable variety of other diagnostic techniques developed over the last 30 or so years—because it is a convenient and relatively inexpensive way to demonstrate the physiological manifestations of abnormal cortical excitability that underlie epilepsy. However, the EEG has a number of limitations. Electrical activity recorded by electrodes placed on the scalp or surface of the brain mostly reflects summation of excitatory and inhibitory postsynaptic potentials in apical dendrites of pyramidal neurons in the more superficial layers of the cortex. Quite large areas of cortex—in the order of a few square centimetres—have to be activated synchronously to generate enough potential for changes to be registered at electrodes placed on the scalp. Propagation of electrical activity along physiological pathways or through volume conduction in extracellular spaces may give a misleading impression as to location of the source of the electrical activity. Cortical generators of the many normal and abnormal cortical activities recorded in the EEG are still largely unknown. -
The Migraine-Epilepsy Syndrome
medigraphic Artemisaen línea Arch Neurocien (Mex) Vol 11, No. 4: 282-287, 2006 The Migraine- Epilepsy Syndrome Arch Neurocien (Mex) Vol. 11, No. 4: 282-287, 2006 Artículo de revisión ©INNN, 2006 de caso The migraine-epilepsy syndrome Enrique Otero Siliceo†, Fernando Zermeño EL SINDROME MIGRAÑA-EPILEPSIA represent a neural exitation. Since that the glutamate has in important rol in both patologys depending of the part of the brain more affected the symptoms might RESUMEN vary from visual to abdominal phemomena. La migraña y la epilepsia tienen varios puntos en común Key words: migraine epilepsy, EEG abnormalities, sintomática clínica y genéticamente lo que ha sido glutamate, diagnosis. postulado por más de cien años. El fenómeno referido como migraña-epilepsia sugiere que exista una he first steps of a practical, approach by patofisiología común. El síndrome de migraña o physicians in recognizing and treating neuro- epilepsia tiene fenómenos comunes de dolor adominal T logic diseases are to recognithat there are jaqueca anormalidades del EE y respuesta a droga various overlaps between migraine and epilepsy. antiepilépticas. En ocasiones el paciente puede tener Epileptic seizures and classic migraine episodes may un ataque migrañoso o una convulsión o en otras occur in the same patient. Migraine and epilepsy share ambas. La comorbilidad puede explicarse por estados several genetic, clinical, evolutive and neurophysio- de hiperrexcitabilidad neural. Alteraciones electroen- logic features. A relationship between epilepsy and cefalográficas son comunes en estos estados. En migraine has been postulated for over a hundred years apariencia el glutamato tiene un papel importante tanto and the syndrome of Migraine-Epilepsy illustrates this en la migraña como en la epilepsia. -
Photosensitive Epilepsy
photosensitive epilepsy factsheet If you have epilepsy you may be able to identify triggers – situations that set off your seizures. Common triggers include stress or tiredness. If seizures are triggered by flashing lights 1 or certain patterns, this is called photosensitive epilepsy. how common is photosensitive epilepsy? how is photosensitive epilepsy treated? Around 1 in 100 people has epilepsy, and of these Photosensitive epilepsy usually responds well to people, around 3% have photosensitive epilepsy. anti-epileptic drugs (AEDs) that treat generalised Photosensitive epilepsy is more common in children seizures (seizures that affect both sides of the brain and young people (up to 5%) and is less commonly at once). diagnosed after the age of 20. See our booklet and chart medication for epilepsy for more information. how can I tell if I am photosensitive? Many people know this if they have a seizure Triggers are individual, but the following sources when they are exposed to flashing lights or patterns in themselves are not generally likely to trigger as a photosensitive trigger will usually cause photosensitive seizures. See over the page for a seizure straightaway. possible triggers and what increases the risk. An electroencephalogram (EEG) may include testing • UK TV programme content. Ofcom regulates for photosensitive epilepsy. This involves looking at a material shown on TV in the UK. The regulations light which will flash at different speeds. If this causes restrict the flash rate to three hertz or less, and any changes in brain activity the technician can stop they also restrict the area of screen allowed for the flashing light before a seizure develops. -
Title in All Caps
Epilepsy Syndromes: Where does Dravet Syndrome fit in? Scott Demarest MD Assistant Professor, Departments of Pediatrics and Neurology University of Colorado School of Medicine Children's Hospital Colorado Disclosures Scott Demarest has consulted for Upsher-Smith on an unrelated subject matter. No conflicts of interest Objectives • What is an Epilepsy Syndrome? • How do we define epilepsy syndromes? • Genetic vs Phenotype (Features) • So what? Why do we care about Epilepsy Syndromes? • How do we organize and categorize Epilepsy Syndromes? • What epilepsy syndromes are similar to Dravet Syndrome and what is different about them? Good Resource International League Against Epilepsy Epilepsydiagnosis.org https://www.epilepsydiagnosis.org/syndrome/epilepsy- syndrome-groupoverview.html What is an Epilepsy Syndrome? A syndrome is a collection of common clinical traits. For Epilepsy this is usually about: • What type of seizures occur? • Age seizure start? Electroclinical • Development? Features or • What does the EEG look like? Phenotype • Other Co-morbidities… Course of an Epilepsy Syndrome Developmental Trajectories - Theoretical Model Normal Previously Normal with Epileptic Encephalopathy Development Never Normal Gray represents Epilepsy Onset the intensity of Age Epilepsy How distinct are Epilepsy Syndromes? A B C Many features might overlap, but the hope is that the cluster of symptoms are “specific” to that epilepsy syndrome…this is often better in theory than practice. How does the individual patient fit? A B C Is this patient at type A,B or C? What about Syndromes Defined by Genes? Is SCN1A the same as Dravet Syndrome? …I don’t have a perfect answer for this… many diseases are being defined by the gene (CDKL5, SCN8A, CHD2). -
Towards an International Classification of Vestibular Disorders
Journal of Vestibular Research 19 (2009) 1–13 1 DOI 10.3233/VES-2009-0343 IOS Press Classification of vestibular symptoms: Towards an international classification of vestibular disorders First consensus document of the Committee for the Classification of Vestibular Disorders of the B ar´ any´ Society Alexandre Bisdorffa,∗, Michael Von Brevernb, Thomas Lempertc and David E. Newman-Tokerd aDepartment of Neurology, Centre Hospitalier Emile Mayrisch, L-4005 Esch-sur-Alzette, Luxembourg bVestibular Research Group Berlin, Department of Neurology, Park-Klinik Weissensee, Berlin, Germany cVestibular Research Group Berlin, Department of Neurology, Schlosspark-Klinik, Berlin, Germany dDepartment of Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA On behalf of the Committee for the Classification of Vestibular Disorders of the Bar´ any´ Society: Pierre Bertholon, Alexandre Bisdorff, Adolfo Bronstein, Herman Kingma, Thomas Lempert, Jose Antonio Lopez Escamez, Måns Magnusson, Lloyd B. Minor, David E. Newman-Toker, Nicolas´ Perez,´ Philippe Perrin, Mamoru Suzuki, Michael von Brevern, John Waterston and Toshiaki Yagi Received 4 February 2009 Accepted 1 September 2009 1. Introduction such as psychiatry and headache, where often there is no histopathologic, radiographic, physiologic, or other The Committee for Classification of Vestibular Dis- independent diagnostic standard available. However, orders of the Bar´ any´ Society was inaugurated at the diagnostic standards and classification are also crucial meeting of the Bar´ any´ Society in Uppsala 2006. Its in areas of medicine such as epilepsy and rheumatolo- charge is to promote development of an implementable gy, where, although confirmatory tests do exist, there classification of vestibular disorders. is substantial overlap in clinical features or biomarkers Symptom and disease definitions are a fundamen- across syndromes. -
Myoclonic Status Epilepticus in Juvenile Myoclonic Epilepsy
Original article Epileptic Disord 2009; 11 (4): 309-14 Myoclonic status epilepticus in juvenile myoclonic epilepsy Julia Larch, Iris Unterberger, Gerhard Bauer, Johannes Reichsoellner, Giorgi Kuchukhidze, Eugen Trinka Department of Neurology, Medical University of Innsbruck, Austria Received April 9, 2009; Accepted November 18, 2009 ABSTRACT – Background. Myoclonic status epilepticus (MSE) is rarely found in juvenile myoclonic epilepsy (JME) and its clinical features are not well described. We aimed to analyze MSE incidence, precipitating factors and clini- cal course by studying patients with JME from a large outpatient epilepsy clinic. Methods. We retrospectively screened all patients with JME treated at the Department of Neurology, Medical University of Innsbruck, Austria between 1970 and 2007 for a history of MSE. We analyzed age, sex, age at seizure onset, seizure types, EEG, MRI/CT findings and response to antiepileptic drugs. Results. Seven patients (five women, two men; median age at time of MSE 31 years; range 17-73) with MSE out of a total of 247 patients with JME were identi- fied. The median follow-up time was seven years (range 0-35), the incidence was 3.2/1,000 patient years. Median duration of epilepsy before MSE was 26 years (range 10-58). We identified three subtypes: 1) MSE with myoclonic seizures only in two patients, 2) MSE with generalized tonic clonic seizures in three, and 3) generalized tonic clonic seizures with myoclonic absence status in two patients. All patients responded promptly to benzodiazepines. One patient had repeated episodes of MSE. Precipitating events were identified in all but one patient. Drug withdrawal was identified in four patients, one of whom had additional sleep deprivation and alcohol intake. -
Epilepsy Syndromes E9 (1)
EPILEPSY SYNDROMES E9 (1) Epilepsy Syndromes Last updated: September 9, 2021 CLASSIFICATION .......................................................................................................................................... 2 LOCALIZATION-RELATED (FOCAL) EPILEPSY SYNDROMES ........................................................................ 3 TEMPORAL LOBE EPILEPSY (TLE) ............................................................................................................... 3 Epidemiology ......................................................................................................................................... 3 Etiology, Pathology ................................................................................................................................ 3 Clinical Features ..................................................................................................................................... 7 Diagnosis ................................................................................................................................................ 8 Treatment ............................................................................................................................................. 15 EXTRATEMPORAL NEOCORTICAL EPILEPSY ............................................................................................... 16 Etiology ................................................................................................................................................ 16