DOCSLIB.ORG

A Proposal for Case Definitions and Outcome Measures in Studies of Infantile Spasms and West Syndrome: Consensus Statement of the West Delphi Group

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
A Proposal for Case Definitions and Outcome Measures in Studies of Infantile Spasms and West Syndrome: Consensus Statement of the West Delphi Group Epilepsia, 45(11):1416–1428, 2004 Blackwell Publishing, Inc. C 2004 International League Against Epilepsy A Proposal for Case Definitions and Outcome Measures in Studies of Infantile Spasms and West Syndrome: Consensus Statement of the West Delphi Group Andrew L. Lux and John P. Osborne Bath Unit for Research in Paediatrics, Royal United Hospital, Bath, and School for Health, University of Bath, Bath, United Kingdom for The West Delphi Group Summary: Purpose: To reach a broad consensus on case def- Primary electroclinical outcome denotes cessation of spasms initions, outcomes, and outcome measures that will ease future with resolution of hypsarrhythmia. West syndrome should be a study design and facilitate comparison of data from different defined subset of the syndrome of infantile spasms. An infan- studies of infantile spasms and West syndrome. tile spasms single-spasm variant should be recognized. Ways Methods: Persons who had recently presented or published are suggested of handling subtle spasms in the context of clin- first-author original research in this field were invited to partici- ical studies. It proposes a standard for reporting modifying and pate in an e-mail Delphi process and to invite other investigators atypical features of hypsarrhythmia, a minimal set of baseline or clinicians who they thought might participate. characteristics and outcomes that should be reported in trials of Results: The process consisted of six rounds, anonymous ex- infantile spasms, and suggests a standard definition of relapse. cept to the facilitator. In total, responses were received from 46 Consensus was not reached on a definition of hypsarrhythmia. participants. The final statement was approved by 31 participants Conclusions: We reached a clear consensus on many aspects from 15 countries. It concluded that the primary clinical out- of study design for the investigation of infantile spasms, although come, cessation of spasms, should denote absence of witnessed incomplete consensus was found on how to define EEG criteria. spasms from within 14 days of commencement of treatment, Key Words: Epilepsy—Infantile spasms—West syndrome— and for ≥28 consecutive days from the last witnessed spasm. Consensus—Clinical trials. Infantile spasms (West syndrome) is a form of epilepsy that is associated with many underlying conditions and Accepted June 20, 2004. often a poor developmental outcome. Its main clinical Address correspondence and reprint requests to Dr. J.P. Osborne at features are spasms that usually occur in clusters and Bath Unit for Research in Paediatrics, Royal United Hospital, Combe onset that almost always occurs during the first 2 years Park, Bath, BA1 3NG, U.K. E-mail: [email protected] of life. It is characteristically associated with an EEG The West Delphi Group pattern called hypsarrhythmia, although hypsarrhythmia Andrew L. Lux and John P. Osborne, Bath Unit for Research in Paediatrics, Bath, England, and School for Health, University of may not be present throughout the course of the spasms Bath, Bath, U.K.; Giuliano Avanzini and Tiziana Granata, Istituto and is not regarded as essential for the diagnosis of in- Neurologico “C. Besta,” Milan, Italy; Tallie Z. Baram, University of fantile spasms. Hypsarrhythmia is an interictal pattern California at Irvine, Irvine, CA, U.S.A.; Roberto Caraballo, Hospital Nacional de Pediatria “Juan P. Garrahan,” Buenos Aires, Ar- that usually changes during clinical attacks to lower- gentina; Kevin Farrell, University of British Columbia, Vancouver, amplitude slow waves or to a sudden flattening that is British Columbia, Canada; Ellie Hancock, Great Ormond Street Hospi- tal, London, U.K.; Masatoshi Ito, Shiga Medical Center for Children, Moriyama, Japan; Jun Kohyama, Tokyo Medical and Dental Univer- William Soler Children’s Hospital, Havana, Cuba; Dietz Rating, sity, Tokyo, Japan; Roshan Koul, Sultan Qaboosh Hospital, Alkhad, University of Heidelberg, Heidelberg, Germany; Bernhard Schmitt, Oman; Wei-Ling Lee, National Neuroscience Institute, Singapore; University Children’s Hospital, Zurich, Switzerland; Malinee A. Jianxiang Liao, Shenzhen Children’s Hospital, Shenzhen, China; Thambyayah, Hospital Selayang, Kuala Lumpur, Malaysia; Federico Marissa B. Lukban and Aida R. Mendoza-Salonga, University of Vigevano, Ospedale Pediatrico Bambinu Gesu, Rome, Italy; Anannit the Philippines College of Medicine, Manila, Philippines; Anthony Visudtibhan, Mahidol University, Bangkok, Thailand; William White- G. Marson, University of Liverpool, Liverpool, U.K.; Brian Neville, house, University of Nottingham, Nottingham, U.K.; Virginia Wong, Institute of Child Health, London, U.K.; Harry O. Nomayo, Klinikum The University of Hong Kong, Hong Kong SAR, China; Hideo Ya- Weiden Kinderklinik, Weiden, Germany; Hirokazu Oguni. Tokyo manouchi Dokkyo University School of Medicine, Tochigi, Japan; Women’s Medical University, Tokyo, Japan; Shunsuke Ohtahara, Chainllie Young, Washington University School of Medicine, St. Louis, Okayama University Medical School, Okayama, Japan; Hian-Tat Ong, Mo, U.S.A.; and Zhongshou Zhou, China-Japan Friendship Hospital, National University Hospital, Singapore; Desiderio Pozo Lauzan, Beijing, China. 1416 WEST DELPHI GROUP CONSENSUS STATEMENT 1417 known as an electrodecremental period. Various EEG fea- plored some controversial areas. Those agreeing to par- tures have traditionally been labeled modified or atypical ticipate were asked to formulate further questions as a hypsarrhythmia (1–4). unique contribution to the study. We made provision for Several International League Against Epilepsy (ILAE) participants to enter after the first round because not all in- commissions and workshops have proposed definitions terested persons would necessarily have been contacted in and classifications of infantile spasms, and some elements time to respond to Round 1. We suggested that, to be con- of these are not mutually consistent. For example, propos- sidered members of the West Delphi Group, participants als from the ILAE Commissions on Classification and would need to make contributions to other rounds and to Terminology in 1985 and 1989 suggested that cases of agree the final draft of the proposal. We also suggested infantile spasms should always have onset of symptoms that minority and dissenting views would be clearly rep- before age 12 months, whereas a 1991 workshop of the resented in the final draft, and that we would not censor ILAE Commission on Pediatric Epilepsy suggested that views. infantile spasms “transcends age groups and may occur The content and form of subsequent rounds was deter- in infancy or childhood” (5–7). Variation in age at onset mined in part by the suggestions of participants. A bal- as a defining criterion alters epidemiologic characteristics ance was reached between eliciting quantitative informa- such as median age at onset of illness and affects estimates tion about the strength of agreements and eliciting qualita- of age-dependent outcomes. In addition to differences in tive and creative views on how we might approach prob- definitions and criteria proposed by the ILAE, many other lems of definition, classification, and measurement. We variations in definition, classification, and outcome crite- tried to maintain a focus on aspects of definition and out- ria are found in published studies. come and to develop proposals that would clarify and An initial proposal for standardized case definitions, simplify study design without introducing unnecessary outcomes, and outcome measures in infantile spasms constraints. The first round of West Delphi was sent in was presented at the Symposium on West Syndrome and January 2001, and the final round was sent in November other Infantile Epileptic Encephalopathies, Tokyo, Febru- 2003. ary 2001 (8). Many participants were enthusiastic about this proposal, although some thought that, in particular, it RESULTS would be difficult to achieve a consensus on EEG charac- teristics. In Round 1, 31 responses were received from partici- pants in 14 countries, and overall, 42 respondents from 16 countries. In addition, three potential participants stated a METHODS firm intention to participate but made no substantial con- West Delphi followed the format of a Delphi process, tribution to the process. One response was initially on a method for eliciting expert consensus developed by the behalf of members of the Konigsteiner¨ Kreis, a group of RAND Corporation in the early 1950s (9). A Delphi pro- pediatric neurologists that had already started to address cess essentially involves isolated experts giving their judg- the issue of case definitions and outcome measures in in- ments or opinions to a moderator or facilitator, who makes fantile spasms. These participants continued to confer and those opinions anonymous and redistributes them to the send a single response to subsequent rounds. The 10 initial full group of experts (10). Because the experts are able questions covered the following areas: clinical features, to relate their opinions to that of the larger group, this restrictions for age at onset, assessing developmental de- facilitates an iterative process by which they can finally lay, the usefulness of etiologic subgroups, the likelihood reach broad agreement. This method has been used before of reaching consensus on a definition of hypsarrhythmia, in the fields of neurology, pediatrics, and psychiatry (11– using aggregated data, defining developmental delay, and 14). In West Delphi, responses were collated by A.L.L., the
Load more

Recommended publications
  • Prognostic Utility of Hypsarrhythmia Scoring in Children with West
    Clinical Neurology and Neurosurgery 184 (2019) 105402 Contents lists available at ScienceDirect Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro Prognostic utility of hypsarrhythmia scoring in children with West syndrome T after ketogenic diet ⁎ Yunjian Zhang1, Lifei Yu1, Yuanfeng Zhou, Linmei Zhang, Yi Wang, Shuizhen Zhou Department of Pediatric Neurology, Children’s Hospital of Fudan University, China ARTICLE INFO ABSTRACT Keywords: Objective: The aim of this study was to evaluate the clinical efficacy and electroencephalographic (EEG) changes West syndrome of West syndrome after ketogenic diet (KD) therapy and to explore the correlation of EEG features and clinical Ketogenic diet efficacy. EEG Patients and methods: We retrospectively studied 39 patients with West syndrome who accepted KD therapy from Hypsarrhythmia May 2011 to October 2017. Outcomes including clinical efficacy and EEG features with hypsarrhythmia severity scores were analyzed. Results: After 3 months of treatment, 20 patients (51.3%) had ≥50% seizure reduction, including 4 patients (10.3%) who became seizure-free. After 6 months of treatment, 4 patients remained seizure free, 12 (30.8%) had 90–99% seizure reduction, 8 (20.5%) had a reduction of 50–89%, and 15 (38.5%) had < 50% reduction. Hypsarrhythmia scores were significantly decreased at 3 months of KD. They were associated with seizure outcomes at 6 months independent of gender, the course of disease and etiologies. Patients with a hypsar- rhythmia score ≥8 at 3 months of therapy may not be benefited from KD. Conclusion: Our findings suggest a potential benefit of KD for patients with drug-resistant West syndrome. Early change of EEG after KD may be a predictor of a patient’s response to the therapy.
    [Show full text]
  • “Seizures (Epilepsy)” a Seizure: Partial – Start in a Specific Part of the Brain, Not in the Whole Is a Symptom of an Electrical Disturbance in the Brain Brain
    “Seizures (Epilepsy)” A seizure: Partial – start in a specific part of the brain, not in the whole Is a symptom of an electrical disturbance in the brain brain. Unlike generalized seizures, partial seizures can have a Is a rare event warning before they occur (aura). Auras are actually a kind of Has a typical beginning (best clue for accurate diagnosis) seizure. There are several different kinds of partial seizures: Is involuntary simple (motor, sensory or psychological), complex, partial Lasts only a short time (90% complete in 90 seconds) seizure with secondary generalization. May cause post seizure impairments. Most seizures do not involve convulsions. Accurate seizure diagnosis by the health care provider is very The most common type of seizure is one mostly involving important because the medications used to treat seizures often vary loss of awareness. depending on the type. There are 20 types of seizures in the Seizures can be very subtle and hard to notice. International Classification of Seizures (over 2,000 types reported in the literature). Examples of post-seizure impairments: A detailed description of the seizure by the person observing the Post ictal confusion (length is individual) seizure is necessary for accurate diagnosing. Having a seizure while in Initial difficulty speaking the doctor’s office is very rare. Confusion about when, where, or what was just happening Memory disturbance which can last a while (behaving Seizure observation: - 3 important ones to make (in order of usual normally but can’t retain/absorb information) importance): Headache with some kinds of seizures What happened right as the seizure was beginning? What is epilepsy? What happened after the seizure was over? What happened during the seizure? Epilepsy is a condition where a person has “recurrent, unprovoked” seizures.
    [Show full text]
  • Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission
    ILAE POSITION PAPER Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology *Robert S. Fisher, †J. Helen Cross, ‡Jacqueline A. French, §Norimichi Higurashi, ¶Edouard Hirsch, #Floor E. Jansen, **Lieven Lagae, ††Solomon L. Moshe, ‡‡Jukka Peltola, §§Eliane Roulet Perez, ¶¶Ingrid E. Scheffer, and ##***Sameer M. Zuberi Epilepsia, 58(4):522–530, 2017 doi: 10.1111/epi.13670 SUMMARY The International League Against Epilepsy (ILAE) presents a revised operational clas- sification of seizure types. The purpose of such a revision is to recognize that some sei- zure types can have either a focal or generalized onset, to allow classification when the onset is unobserved, to include some missing seizure types, and to adopt more trans- parent names. Because current knowledge is insufficient to form a scientifically based classification, the 2017 Classification is operational (practical) and based on the 1981 Classification, extended in 2010. Changes include the following: (1) “partial” becomes “focal”; (2) awareness is used as a classifier of focal seizures; (3) the terms dyscognitive, simple partial, complex partial, psychic, and secondarily generalized are eliminated; Dr. Robert S. Fisher, (4) new focal seizure types include automatisms, behavior arrest, hyperkinetic, auto- past president of nomic, cognitive, and emotional; (5) atonic, clonic, epileptic spasms, myoclonic, and American Epilepsy tonic seizures can be of either focal or generalized onset; (6) focal to bilateral tonic– Society and editor of clonic seizure replaces secondarily generalized seizure; (7) new generalized seizure Epilepsia and types are absence with eyelid myoclonia, myoclonic absence, myoclonic–atonic, epilepsy.com, led the myoclonic–tonic–clonic; and (8) seizures of unknown onset may have features that can Seizure Classification still be classified.
    [Show full text]
  • Understanding Seizures and Epilepsy
    Understanding Sei zures & Epilepsy Selim R. Benbadis, MD Leanne Heriaud, RN Comprehensive Epilepsy Program Table of Contents * What is a seizure and what is epilepsy?....................................... 3 * Who is affected by epilepsy? ......................................................... 3 * Types of seizures ............................................................................. 3 * Types of epilepsy ............................................................................. 6 * How is epilepsy diagnosed? .......................................................... 9 * How is epilepsy treated? .............................................................. 10 Drug therapy ......................................................................... 10 How medication is prescribed ............................................ 12 Will treatment work?............................................................ 12 How long will treatment last?............................................. 12 Other treatment options....................................................... 13 * First aid for a person having a seizure ....................................... 13 * Safety and epilepsy ....................................................................... 14 * Epilepsy and driving..................................................................... 15 * Epilepsy and pregnancy ............................................................... 15 * More Information .......................................................................... 16 Comprehensive
    [Show full text]
  • Occipital and Parietal Lobe Epilepsies
    Chapter 15 Occipital and parietal lobe epilepsies JOHN S. DUNCAN UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London Epileptic seizures of parietal and occipital origin are heterogeneous and mainly characterised by the presenting auras, although the most dramatic clinical manifestations may reflect spread, and overshadow the focal origin. The two lobes serve mainly sensory functions, and the characteristic seizure phenomena are therefore subjective sensations. The incidence of these seizures is not well known, but they are generally considered rare. Occipital seizures have been reported to constitute 8% and parietal seizures 1.4% of total seizures in the prevalent population with epilepsy1,2. The pattern of seizures is most commonly focal seizures without impairment of awareness, with occasional secondary generalisation. Focal seizures with impairment of awareness are rare and usually indicate spread of the seizure into the temporal lobe. Seizures with somatosensory symptomatology1-3 Somatosensory seizures may arise from any of the three sensory areas of the parietal lobe, but the post-central gyrus is most commonly involved. Seizures present with contralateral, or rarely ipsilateral, or bilateral sensations. All sensory modalities may be represented, most commonly tingling and numbness, alone or together. There may be prickling, tickling or crawling sensations, or a feeling of electric shock in the affected body part. The arms and the face are the most common sites, but any segment or region may be affected. The paraesthesia may spread in a Jacksonian manner, and when this occurs motor activity in the affected body member follows the sensations in about 50% of cases.
    [Show full text]
  • Infantile Spasms: an Update on Pre-Clinical Models and EEG Mechanisms
    children Review Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms Remi Janicot, Li-Rong Shao and Carl E. Stafstrom * Division of Pediatric Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA; [email protected] (R.J.); [email protected] (L.-R.S.) * Correspondence: [email protected]; Tel.: +1-(410)-955-4259; Fax: +1-(410)-614-2297 Received: 19 November 2019; Accepted: 23 December 2019; Published: 6 January 2020 Abstract: Infantile spasms (IS) is an epileptic encephalopathy with unique clinical and electrographic features, which affects children in the middle of the first year of life. The pathophysiology of IS remains incompletely understood, despite the heterogeneity of IS etiologies, more than 200 of which are known. In particular, the neurobiological basis of why multiple etiologies converge to a relatively similar clinical presentation has defied explanation. Treatment options for this form of epilepsy, which has been described as “catastrophic” because of the poor cognitive, developmental, and epileptic prognosis, are limited and not fully effective. Until the pathophysiology of IS is better clarified, novel treatments will not be forthcoming, and preclinical (animal) models are essential for advancing this knowledge. Here, we review preclinical IS models, update information regarding already existing models, describe some novel models, and discuss exciting new data that promises to advance understanding of the cellular mechanisms underlying the specific EEG changes seen in IS—interictal hypsarrhythmia and ictal electrodecrement. Keywords: infantile spasms; West syndrome; epilepsy; childhood; epileptic encephalopathy; electroencephalogram (EEG); hypsarrhythmia; electrodecrement; animal model 1. Introduction Epileptic encephalopathies (EEs) are a spectrum of disorders that mostly begin during infancy and have poor neurological and behavioral outcomes.
    [Show full text]
  • A Novel Deletion Mutation in EPM2A Underlies Progressive Myoclonic Epilepsy (Lafora Body Disease) in a Pakistani Family
    Neurology Asia 2021; 26(2) : 427 – 433 A novel deletion mutation in EPM2A underlies progressive myoclonic epilepsy (Lafora body disease) in a Pakistani family 1Fizza Orooj MRCP, 2Umm-e-Kalsoom PhD, 3XiaoChu Zhao, 1Arsalan Ahmad MD, 4Imran Nazir Ahmed MD, 5Muhammad Faheem PhD, 5Muhammad Jawad Hassan PhD, 3,6Berge A. Minasian MD 1Division of Neurology, Shifa International Hospital, Shifa Tameer-e-Millat University, Islamabad, Pakistan; 2Department of Biochemistry, Hazara University, Mansehra, KPK, Pakistan; 3Program in Genetics and Genome Biology, The Hospital for Sick Children, Toronto, Canada; 4Department of Pathology, Shifa International Hospital, Shifa Tameer-e-Millat University, Islamabad, Pakistan; 5Department of Biological Sciences, National University of Medical Sciences, Rawalpindi, Pakistan; 6Department of Pediatrics, University of Texas Southwestern, Dallas, Teas, USA Abstract Lafora body disease (MIM-254780), a glycogen storage disease, characterized by Lafora bodies (deformed glycogen molecules) accumulating in multiple organs, is a rare form of myoclonic epilepsy. It manifests in early adolescent years, initially with seizures and myoclonus, followed by dementia and progressive cognitive decline, ultimately culminating in death within 10 years. In Pakistan so far 5 cases have been reported. Here, we report a new case of Lafora body disease belonging to a consanguineous family from Pakistan. Histopathological analysis confirmed presence of lafora bodies in the patient`s skin. Sanger sequencing revealed novel homozygous 5bp deletion mutation (NM_005670.4; c.359_363delGTGTG) in exon 2 of the EPM2A gene, which was truly segregated in the family. These results will increase our understanding regarding the aetiology of this disorder and will further add to the mutation spectrum of EPM2A gene.
    [Show full text]
  • All Seizures
    ALL about SEIZURES What is Epilepsy? Epilepsy is a neurological disorder – a physical condition – which causes sudden bursts of hyperactivity in the brain. This hyperactivity produces “seizures” which vary from one person to another in frequency and form. A seizure may appear as • a brief stare • a change of awareness • a convulsion A seizure may last a few seconds or a few minutes. Epilepsy • is not a disease • is not a psychological disorder • is not contagious Causes In approximately 60-75% of all cases, there is no known cause. Of the remaining cases, there are a number of fre- quently identified causes. Identifiable Causes • brain injury to the fetus during pregnancy • birth trauma (lack of oxygen) • aftermath of infection (meningitis) • head trauma (car accident, sports injury, shaken baby syndrome) • substance abuse • alteration in blood sugar (hypoglycemia) • other metabolic illness (hypocalcemia) • brain tumor • stroke Is There a Cure? Although treatments are available to reduce the frequency and severity of seizures, there is no known cure for epilepsy. Complex partial seizure: a person loses awareness as the Seizures seizure begins and appears dazed and confused. The person There are many different types of seizures. will exhibit meaningless behaviours such as random walk- ing, mumbling, head turning, or pulling at clothing. These Most are classified within 2 main categories: partial seizures behaviours cannot be recalled by the person after the seizure. and generalized seizures. Generalized Seizures Incidence of Seizure Types Generalized seizures occur when the excessive neural activi- ty in the brain encompasses the entire brain. The 2 most common forms are generalized absence seizures and tonic- clonic seizures.
    [Show full text]
  • Pediatric Epilepsy
    PEDIATRIC EPILEPSY Ø Epilepsy is one of the most common chronic neurological disorders. It is characterized by recurrent unprovoked seizures or an enduring predisposition to generate epileptic seizures. If epilepsy begins in childhood, it is often outgrown. Seizures are common in childhood and adolescence. Approximately 3% of children will experience a seizure. Ø A seizure occurs when there is a sudden change in behavior or sensation caused by abnormal and excessive electrical hypersynchronization of neuronal networks in the cerebral cortex. Normal inhibition is overcome by excessive excitatory stimuli. Ø If the cause of the seizures is known (for example: genetic, inborn errors of metabolism, metabolic (eg: low glucose, electrolyte abnormalities), structural (eg: malformations, tumours, bleeds, stroke, traumatic brain injury), infectious, inflammatory, or toxins) it is classified as symptomatic. If the cause is unknown, it is classified as idiopathic. 1. WHERE DID THE SEIZURE START? / WHAT KIND OF SEIZURE IS IT? 2. IS AWARENESS YES FOCAL ONSET GENERALIZED UNKNOWN IMPAIRED? NO Seizure that originates ONSET ONSET in a focal cortical area Seizure that involves When it is unclear YES with associated clinical both sides of the where the seizure 3. PROGRESSION TO BILATERAL? features. brain from the onset. starts. NO SEIZURE SEMIOLOGY (The terminology for seizure types is designed to be useful for communicating the key characteristics of seizures) CLONIC: sustained rhythmical TONIC: muscles stiffen or ATONIC: sudden loss of muscle tone, MYOCLONUS: sudden lighting- jerking movements. tense. lasting seconds. like jerk, may cluster. EPILEPTIC SPASM: sudden AUTONOMIC: eg: AUTOMATISMS: ABSENCE: brief (≤ 10s), OTHERS: change flexion, extension, or flexion- rising epigastric stereotyped, purposeless frequent (up to 100’s) in cognition, extension of proximal and sensations, waves of movements.
    [Show full text]
  • Epilepsy Terms
    Absence seizure A generalized seizure, usually lasting less than 20 seconds, characterized by a blank stare & sometimes blinking, eye rolling or chewing movements. Can occur many times a day. Often mistaken for daydreaming. Usually begins in childhood. Outgrown by approximately 75% of children. Formerly called petit mal. Antiepileptic drugs Medication used to control seizures. Also called anticonvulsants. Atonic seizure A generalized seizure characterized by sudden loss of muscle tone, causes the head or body to drop suddenly with falling & potential injury. Recovery in a few seconds to a minute. Protective helmets are helpful to protect from injury.Also called a drop attack. Aura A warning period at the beginning of a seizure. May sense a feeling of fear or doom, or strange sensations such as an odd smell or taste, nausea, or palpitations. Actually a simple partial seizure occurring seconds or minutes before a complex partial or secondarily generalized tonic-clonic seizure, or it may occur alone. Automatism Purposeless, automatic & involuntary movements during a seizure, such as chewing, lip-smacking, picking at clothing or wandering around confused; may occur during complex partial & absence seizures. Benign rolandic epilepsy Epilepsy syndrome of childhood characterized by partial seizure affecting the fact, causing drooling & inability to speak, may be followed by a convulsion. Typically occur at night and are usually outgrown by age 16. Also called benign partial epilepsy of childhood. Catamenial epilepsy In women, the tendency for seizures to occur around the time of menstruation. Clonic seizure A generalized seizure characterized by rhythmic jerking movements involving both sides of the body. Complex partial seizure A seizure that affects only part of the brain, but causes impaired consciousness or awareness.
    [Show full text]
  • The New Classification of Seizures
    J R Coll Physicians Edinb 2017; 47: 336–8 | doi: 10.4997/JRCPE.2017.406 PAPER The new classi cation of seizures: an overview for the general physician S Liyanagedera1, RP Williams2, RM Bracewell3 ClinicalThe International League Against Epilepsy Classi cation of the Epilepsies, Correspondence to: rst presented in 1981, has been widely adopted across the globe. In RM Bracewell Abstract 2017 it was revised to allow for more robust, speci c, exible and logical Walton Centre NHS classi cation of seizures. A number of new seizure types are recognised. Foundation Trust Classi cation should be timely as it plays a vital role in the diagnosis and Liverpool L9 7LJ management of patients with epilepsy. Accurate classi cation also underpins UK epilepsy research from pathophysiology to public health. Here we review the basic and extended forms of the classi cation. Semiology (symptoms and signs) is used as the foundation for Email: grouping seizures under focal, generalised or of unknown onset. Focal seizures can be further [email protected] classi ed by the presence or absence of awareness and motor signs. Generalised seizures engage bilateral networks from the onset and these can be either motor or non-motor. Seizures of unknown onset can be classi ed as motor, non-motor, tonic–clonic, epileptic spasms, or behaviour arrest. Keywords: classifi cation, epilepsy, focal, generalised, seizures Declaration of interests: RMB is the Editor-in-Chief of JRCPE Introduction Basic seizure classifi cation Epilepsy is a highly prevalent condition.1 About 10–20% of For practitioners not having an expertise in epilepsy the basic acute medical admissions in the UK are neurological;2,3 of seizure classifi cation was devised.
    [Show full text]
  • The 2017 ILAE Classification of Seizures Robert S
    The 2017 ILAE Classification of Seizures Robert S. Fisher, MD, PhD Maslah Saul MD Professor of Neurology Director, Stanford Epilepsy Center In 2017, the ILAE released a new classification of seizure types, largely based upon the existing classification formulated in 1981. Primary differences include specific listing of certain new focal seizure types that may previously only have been in the generalized category, use of awareness as a surrogate for consciousness, emphasis on classifying focal seizures by the first clinical manifestation (except for altered awareness), a few new generalized seizure types, ability to classify some seizures when onset is unknown, and renaming of certain terms to improve clarity of meaning. The attached PowerPoint slide set may be used without need to request permission for any non-commercial educational purpose meeting the usual "fair use" requirements. Permission from [email protected] is however required to use any of the slides in a publication or for commercial use. When using the slides, please attribute them to Fisher et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia doi: 10.1111/epi.13671. ILAE 2017 Classification of Seizure Types Basic Version 1 Focal Onset Generalized Onset Unknown Onset Impaired Aware Motor Motor Awareness Tonic-clonic Tonic-clonic Other motor Other motor Motor Non-Motor (Absence) Non-Motor Non-Motor Unclassified 2 focal to bilateral tonic-clonic 1 Definitions, other seizure types and descriptors are listed in the accompanying paper & glossary of terms 2 Due to inadequate information or inability to place in other categories From Fisher et al. Instruction manual for the ILAE 2017 operational classification of seizure types.
    [Show full text]