DOCSLIB.ORG

Epilepsy Syndromes in Infancy

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Epilepsy Syndromes in Infancy Review Article Epilepsy Syndromes in Infancy Christian M. Korff, MD and Douglas R. Nordli, Jr., MD An increasing number of infantile epilepsy syndromes ognized as such by the majority, thanks to the recent have been recognized. However, a significant number developments in diagnostic techniques, such as video- of infants (children aged 1-24 months) do not fit in any electroencephalography, brain imaging, and cytogenetics. of the currently used subcategories. This article re- However, despite this remarkable progress, up to a quarter views the clinical presentation, electroencephalo- of infants with epilepsy do not fit in any of the proposed graphic findings, evolution, and management of the syndromes, or are classified into broad and unspecific following entities: early infantile epileptic encephalop- categories [8]. It is likely that additional entities are athy, early myoclonic epilepsy, infantile spasms/West waiting to be defined in this age group [1]. This article syndrome, severe myoclonic epilepsy of infancy, myo- reviews the clinical characteristics, electroencephalo- clonic-astatic epilepsy, generalized epilepsy with febrile graphic findings, evolution, and management of the infan- seizures plus, malignant migrating partial seizures of tile epileptic syndromes identified in infancy so far. Their infancy, hemiconvulsions-hemiplegia-epilepsy, benign main features are summarized in Table 1, and specific myoclonic epilepsy, and benign familial/nonfamilial treatment options for some of them are presented in Table infantile seizures. Issues related to their classification 2. Issues related to their classification are addressed at the are addressed. © 2006 by Elsevier Inc. All rights end of the review, and suggestions for further improve- reserved. ments in the classification of infantile epilepsies are proposed. Korff CM, Nordli Jr DR. Epilepsy syndromes in infancy. Pediatr Neurol 2006;34:253-263. Early Infantile Epileptic Encephalopathy With Suppression-Burst (Ohtahara Syndrome) Introduction Early infantile epileptic encephalopathy, or Ohtahara syndrome, is a severe neurologic condition first described An accurate diagnosis is considered the first and most in 1976 [9]. It is a rare entity, with a relative prevalence to critical step in the management of pediatric epilepsy [1,2]. West syndrome estimated at 1/40 or less [9]. It is charac- Delineating epilepsy syndromes serves that purpose. In terized by epileptic tonic spasms and a suppression-burst addition to improving communication between caregivers electroencephalogram (Fig 1). The suppression-burst pat- and establishing clear criteria for research studies, epilepsy tern is present in both waking and sleeping states, and syndromes can suggest underlying disease pathophysiol- appears in the first 3 months of life [9]. Partial tonic ogy, provide prognostic information to the families, and seizures can be observed, but myoclonic seizures are rare aid in the selection of appropriate diagnostic tests. Epi- [9]. Etiology is heterogeneous, and includes brain malfor- lepsy syndromes are the rational basis for selection of mations, migration disorders, mitochondrial diseases, and, appropriate antiepileptic drugs, surgical procedures, or rarely, inborn errors of metabolism, such as Leigh’s alternative treatment options [1,3,4]. disease or cytochrome oxidase deficiency [9,10]. The The epilepsy syndrome classification system adopted by seizures are usually intractable to antiepileptic medica- the International League Against Epilepsy in 1989 [5] has tions, and the developmental prognosis is uniformly poor been demonstrated to be reliable and applicable to children [9]. Surgery can be considered in those patients with [6,7]. Moreover, an increasing number of infantile (1-24 cortical malformations, such as cortical dysplasia or hemi- months) epileptic syndromes are being isolated and rec- megalencephaly. Ohtahara syndrome frequently evolves From the Epilepsy Center, Children’s Memorial Hospital, Chicago, Communications should be addressed to: Illinois. Dr. Nordli; Director, Epilepsy Center; Children’s Memorial Hospital; Box #29; 2300 Children’s Plaza; Chicago, IL 60614-3394. E-mail: [email protected] Received May 5, 2005; accepted July 27, 2005. © 2006 by Elsevier Inc. All rights reserved. Korff and Nordli: Epilepsy Syndromes in Infancy 253 doi:10.1016/j.pediatrneurol.2005.08.005 ● 0887-8994/06/$—see front matter Table 1. Main characteristics of the epileptic syndromes that present in infancy Syndrome Main Characteristics Early infantile epileptic encephalopathy First months; tonic seizures, spasms; suppression-burst; severe outcome Early myoclonic epilepsy First months; myoclonias, spasms; suppression-burst (sleep); severe outcome Infantile spasms First year; spasms; hypsarrhythmia; developmental delay Dravet syndrome First year; partial and generalized seizures, myoclonias; normal EEG at onset; later: generalized spike-waves and multifocal spikes; severe outcome Myoclonic astatic epilepsy First year; generalized myoclonic and astatic seizures; interictal parietal theta activity and bilateral spike-waves; variable outcome Malignant migrating partial seizures of infancy First year; continuous electrographic seizures, multiple areas of onset; severe outcome Generalized epilepsy with febrile seizures (ϩ) Variable seizure and developmental phenotype in addition to febrile seizures and afebrile generalized convulsions Hemiconvulsion-hemiplegia-epilepsy Prolonged unilateral febrile seizures; subsequent hemiparesis and partial epilepsy Benign myoclonic epilepsy First 3 years; myoclonic seizures; normal interictal EEG; normal development Benign familial/nonfamilial seizures First year; partial seizures; normal development into West syndrome, and for some, later, into Lennox- helped two patients with cryptogenic etiology, but was Gastaut syndrome [4,11,12]. Controversies exist as to ineffective in all others. Three patients had their seizure whether Ohtahara syndrome, West syndrome, and Len- activity controlled on zonisamide. This publication was nox-Gastaut syndrome are part of the same spectrum of particularly important because of the prognostic findings. the age-dependent epileptic encephalopathies, or whether On evolution, two groups with different prognoses were they each represent isolated syndromes with specific identified: one of them included seven patients, who characteristics [12,13]. evolved from Ohtahara syndrome to West syndrome and A recent report on 16 patients diagnosed with early later to Lennox-Gastaut syndrome, and all of these patients infantile epileptic encephalopathy indicated that the age at died; the second included patients with spike foci on first seizure ranged from 1 day to 3 months. There was no electroencephalogram: only one patient died, and seven sex predominance. All of the patients had tonic spasms, were seizure-free. Nevertheless, all patients in the second either in clusters or isolated. Hemiconvulsions, erratic group were physically and mentally severely handicapped focal motor seizures, and asymmetric tonic seizures were [14]. observed in one third to one half of the patients. General- ized clonic seizures were observed in one patient. The interictal electroencephalogram was characterized by 1- to Early Myoclonic Epilepsy/Encephalopathy 3-second bursts of high-voltage (150-350 ␮V) slow waves This rare entity was termed “neonatal myoclonic en- and multifocal spikes, separated by suppression phases of cephalopathy” in its initial description [15]. It was further 2-5 seconds duration. This pattern was independent of the delineated and renamed “early myoclonic epileptic en- circadian rhythm. The spasms were accompanied by a cephalopathy” by Dalla Bernardina et al. in 1983 [16], and high-voltage slow wave followed by attenuation, some- “early myoclonic epilepsy” by Aicardi in 1985 [17]. Like times with low-voltage fast activity. The interictal sup- early infantile epileptic encephalopathy, it is characterized pression-burst pattern often disappeared during a cluster of by intractable seizures with onset during the first 3 months spasms. Partial seizures could precede or follow a cluster of life, suppression-burst interictal electroencephalogram, of spasms, but were also observed independently. Etiology and poor developmental prognosis [9]. Early myoclonic or associated conditions included two suspected cases of epilepsy is, however, distinguished from early infantile Aicardi syndrome, one case of hemimegalencephaly, one epileptic encephalopathy by its predominant clinical man- case of porencephaly, one case of hydrocephalus, one case ifestations, erratic myoclonias, and by the presence of a of lissencephaly, and four cases of nonspecific cerebral suppression-burst pattern mainly during sleep. Partial atrophy or dysgenesis. Adrenocorticotrophic hormone seizures are also observed, and include tonic spasms, isolated or in clusters, versive seizures, migrating clonic Table 2. Specific treatment options seizures, and asymmetric tonic seizures with occasional secondary generalization [9]. Patients with early myo- Epileptic Syndrome Treatment Options clonic epilepsy share most electroencephalographic char- Infantile spasms (West) Adrenocorticotrophic hormone, vigabatrin, acteristics with patients with early infantile epileptic en- ketogenic diet cephalopathy, although the duration of bursts and Dravet Stiripentol–valproic acid–clobazam, suppression epochs, and the length of burst-burst intervals topiramate Myoclonic astatic epilepsy Valproic acid,
Load more

Recommended publications
  • 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.
    [Show full text]
  • Epilepsy and Seizure Disorders: a Resource Guide for Parents
    $5HVRXUFH *XLGH IRU3DUHQWV www.chadkids.org/epilepsyonline Epilepsy and Seizure Disorder: A Parent Resource Guide ¡ ¡ ¨ © £ ¤ ¢ ¥ ¦ § ¦ ! " $ # ¡ ¨ ' ( £ ¤ ¢ ¥ ¦ %& ¥ - . , ! # ! ) * + * ' - / ! !! ! # ! + ) ' - 00 ! , ! # ! + ) ¡ ¡¡ ¨ ( ( £ ¤ ## ¢ ¥ ¦ % 12 - - 03 ! # # ) * 0 !! ) + 0 , # ! ! ) * + " ' 4 0$ ! ! , # ! + ) 5 8 9 £7 7 162 6 ¦ ¥ 2: ( - 0; ! < - 0> = ? @ 3A ! = ! # 'C B D 3 0 ! ! C D 33 ? ! ! D 3 B ! < 3; = ! # - 3> @ ! ! EF ¤ ¤ 7 ¢ G % H ? 3/ ! ! = C - A I 0 J www.chadkids.org/epilepsyonline Epilepsy and Seizure Disorder: A Parent Resource Guide 2 WWhathat is epilepsy/seizure disorder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
    [Show full text]
  • Diagnosis and Management of Epilepsies in Children and Young People 81
    81 ���� ������������������������������������������� Diagnosis and management of epilepsies 81 in children and young people A national clinical guideline 1 Introduction 1 2 Diagnosis 3 3 Investigative procedures 6 4 Management 11 5 Antiepileptic drug treatment 15 6 Management of prolonged or serial seizures and convulsive status epilepticus 21 7 Behaviour and learning 23 8 Models of care 25 9 Development of the guideline 27 10 Implementation and audit 31 Annexes 34 Abbreviations 47 References 48 March 2005 COPIES OF ALL SIGN GUIDELINES ARE AVAILABLE ONLINE AT WWW.SIGN.AC.UK KEY TO EVIDENCE STATEMENTS AND GRADES OF RECOMMENDATIONS LEVELS OF EVIDENCE 1++ High quality meta-analyses, systematic reviews of randomised controlled trials (RCTs), or RCTs with a very low risk of bias 1+ Well conducted meta-analyses, systematic reviews of RCTs, or RCTs with a low risk of bias 1 - Meta-analyses, systematic reviews of RCTs, or RCTs with a high risk of bias 2++ High quality systematic reviews of case control or cohort studies High quality case control or cohort studies with a very low risk of confounding or bias and a high probability that the relationship is causal 2+ Well conducted case control or cohort studies with a low risk of confounding or bias and a moderate probability that the relationship is causal 2 - Case control or cohort studies with a high risk of confounding or bias and a significant risk that the relationship is not causal 3 Non-analytic studies, eg case reports, case series 4 Expert opinion GRADES OF RECOMMENDATION Note: The grade of recommendation relates to the strength of the evidence on which the recommendation is based.
    [Show full text]
  • Epilepsy in Children – Brain Growth and Behavior
    logy & N ro eu u r e o N p h f y o s l i a o l n o r Kanemura and Aihara, J Neurol Neurophysiol 2013, S2 g u y o J Journal of Neurology & Neurophysiology ISSN: 2155-9562 DOI: 10.4172/2155-9562.S2-006 ReviewResearch Article Article OpenOpen Access Access Epilepsy in Children – Brain Growth and Behavior Hideaki Kanemura1* and Masao Aihara2 1Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Japan 2Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Japan Abstract The degree of behavioral problems associated with epilepsy in children is greater than would be expected on the basis of the existence of a chronic illness alone. Involvement of the frontal lobe such as atypical evolution of benign childhood epilepsy with centrotemporal spikes (BCECTS), epilepsy with continuous spike-waves during slow sleep (CSWS), and frontal lobe epilepsy (FLE) are characterized by impairment of neuropsychological abilities, and are frequently associated with behavioral disorders. In volumetric studies using three-dimensional (3D) magnetic resonance imaging (MRI), frontal and prefrontal lobe volumes revealed growth disturbance in patients with atypical evolution of BCECTS, CSWS, and FLE compared with those of normal subjects. These studies also revealed that in patients with shorter seizure durations and abnormal EEG periods, developmental abnormalities of the frontal lobe were soon restored to a more normal growth ratio. Conversely, growth disturbances of the prefrontal lobes were persistent in the patients with longer seizure durations and abnormal EEG periods. These findings suggest that seizure and the duration of paroxysmal anomalies may be associated with prefrontal lobe growth abnormalities, which are associated with neuropsychological problems.
    [Show full text]
  • Provincial Guidelines for the Management of Epilepsy in Adults
    PROVINCIAL GUIDELINES FOR THE MANAGEMENT OF EPILEPSY IN ADULTS AND CHILDREN Epilepsy Implementation Task Force Version 1.0 | Critical Care Services Ontario | January 2015 These Guidelines are a Product of Critical Care Services Ontario (CCSO) Provincial Guidelines for the Management of Epilepsy in Adults and Children is the result of a collaborative effort between CCSO, the Epilepsy Implementation Task Force (EITF), and Provincial Neurosurgery Ontario (PNO). The EITF was established in June 2013 to develop and implement a provincial framework to maximize value from the system of epilepsy care in Ontario. To support the flow of patients towards appropriate treatment for epilepsy, this document contains a set of guidelines to help with the diagnosis, treatment and referral practices from the moment of a patient’s first seizure. The EITF works in collaboration with PNO to support equitable and timely access to neurosurgical care, including epilepsy surgery, and to help maintain the province’s neurosurgical capacity. How to Use This Document The Guidelines included in this document have been developed by a sub-group of the Epilepsy Implementation Task Force for any health care provider engaged in the care of patients with epilepsy before referral to surgery. The guidelines are based on current processes and represent expectations for the highest standards of epilepsy care. This document provides recommendations only. For information about these Guidelines, please contact: Critical Care Services Ontario Phone: 416-340-4800 x 5577 Email: [email protected] Website: www.criticalcareontario.ca CCSO is funded by the Government of Ontario. Version Control Name of document Provincial Guidelines for the Management of Epilepsy in Adults and Children Version 1.0 Created January 2015 Recommended next review November 2016 Approved By The Epilepsy Implementation Task Force (EITF) and Provincial Neurosurgery Ontario (PNO) Disclaimer: The contents of these Guidelines may change over time.
    [Show full text]
  • Investigation of Seizures in Infants
    Chapter 23 Investigation of seizures in infants RICHARD E. APPLETON1 and AILSA McLELLAN2 1The Roald Dahl EEG Unit, Paediatric Neurosciences Foundation, Alder Hey Children’s NHS Foundation Trust, Liverpool, and 2Department of Paediatric Neurosciences, Royal Hospital for Sick Children, Edinburgh The investigation of seizures in infancy (i.e. within the first year of life) begins with establishing whether the seizures are epileptic or non-epileptic in origin. The ‘broad’ differential diagnosis of possible seizures and ‘epilepsy’ is multiple and is particularly difficult under the age of 12 months and includes: Gastro-oesophageal reflux (Sandifer’s syndrome) Pallid syncopal attacks (reflex anoxic seizures) Cyanotic breath-holding attacks Cardiac arrhythmias Münchausen syndrome by proxy (passive or active both representing a form of child abuse) Shuddering spells and jitteriness Hyperekplexia Benign neonatal sleep myoclonus Benign myoclonus of infancy Tonic reflex activity and involuntary movements (seen in children with neurological impairment including cerebral palsy or hydrocephalus). Once a non-epileptic disorder has been excluded or the episodes are considered to be obviously epileptic, then the following conditions/investigations should be considered on a chronological basis. Perinatal (first week of life) and neonatal (first month of life) seizures The newborn period is the time of life with the highest risk of seizures1-3. This is because of the relative lack, and immature development of inhibitory neurotransmitters and their pathways. The immature and developing brain is susceptible to a large number of both cerebral and systemic insults including: Asphyxia (hypoxic-ischaemic encephalopathy) the most common and also most serious cause of neonatal seizures – particularly in term infants Intra- and periventricular haemorrhage – particularly in pre-term infants Metabolic dysfunction (e.g.
    [Show full text]
  • Fostering Epilepsy Care in Europe All Rights Reserved
    EPILEPSY IN THE WHO EUROPEAN REGION: Fostering Epilepsy Care in Europe All rights reserved. No part of this publication may be reproduced, stored in a database or retrieval system, or published, in any form or any way, electronically, mechanically, by print, photoprint, microfilm or any other means without prior written permission from the publisher. Address requests about publications of the ILAE/IBE/WHO Global Campaign Against Epilepsy: Global Campaign Secretariat SEIN P.O. Box 540 2130 AM Hoofddorp The Netherlands e-mail: [email protected] ISBN NR. 978-90-810076-3-4 Layout/ Printing: Paswerk Bedrijven, Cruquius, Netherlands Table of contents Foreword ................................................................................................................................................. 4 Preface ................................................................................................................................................. 5 Acknowledgements ........................................................................................................................................ 6 Tribute ................................................................................................................................................. 7 Abbreviations ................................................................................................................................................. 8 Background information on the European Region .........................................................................................
    [Show full text]
  • Diagnosing Epilepsy in Children and Adolescents
    2019 Annual Epilepsy Pediatric Patient Care Conference Diagnosing Epilepsy in Children and Adolescents Korwyn Williams, MD, PhD Staff Epileptologist, BNI at PCH Clinical Assistant Professor, Department Child Health – College of Medicine, University of AZ, Phoenix February 9, 2019 Disclosures None relevant to this talk. Aims & Goals • Discuss the difference • Better understand the between seizure & existence of the epilepsy varieties of seizures • Discuss the diagnosis • Be able to make the process distinction between • Discuss potential seizure and epilepsy mimics of seizures in • Become aware of the children limitations of the diagnostic process Overview • Definitions • Why People Have Epilepsy • The Process of Diagnosing Epilepsy • Mimics of Seizures • Testing • Challenges • Conclusions • Resources Definitions Seizure vs Epilepsy Seizure Epilepsy • An abnormal, excessive or • At least two unprovoked (or synchronous neuronal reflex) seizures occurring greater activity in the brain. than 24 hours apart, OR • One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years, OR • Diagnosis of an epilepsy Fisher et al., Epilepsia, 55(4):475– syndrome 482, 2014 EPILEPSY SEIZURES POST-TRAUMATIC UNPROVOKED FEBRILE focal without alteration In awareness POST-STROKE bilateral tonic-clonic focal with alteration In awareness OVERDOSES tonic absence ELECTROLYTE clonic ABNORMALITIES epileptic spasms atonic myoclonic HYPOGLYCEMIC
    [Show full text]
  • Care Recommendations for Children
    Recommendations for Care of Children with Epilepsy Seeking the best treatment from the right doctor at the right time! Contents Children withWhat of Doctors Type Treat Epilepsy? Children with New Onset Seizures Epilepsy Medication Children Taking Children with Uncontrolled Seizures (Refractory Epilepsy) Making the Office MostChild’s of Your Visit This booklet is to help parents and caregivers know when it is appropriate to seek treatment for a child experiencing seizures. For additional resources about epilepsy and tools to help manage your child’s condition, please visit www.epilepsyandmychild.org. 1 2 3 4 5 What Type of Doctors Treat Children with Children withWhat of Doctors Type Treat Epilepsy? Children with New Onset Seizures Epilepsy Medication Children Taking Children with Uncontrolled Seizures (Refractory Epilepsy) Making the Office MostChild’s of Your Visit Epilepsy? There are several types of doctors who treat epilepsy. For many children, epilepsy can be treated and success- fully managed by a primary care physician, a family physi- cian, or a pediatrician. This is often the case for those whose seizures are well controlled on one medication or who live in rural areas and must travel for hours to see an epilepsy specialist. Most children with epilepsy under the age of 16 are treated by a pediatric neurologist. A pediatric neurologist specializes in childhood diseases of the brain, spinal cord, and nervous system. A pediatric neurologist learns about treating epilepsy in children and is involved in their care during residency. A pediatric epileptologist is a neurologist who com- pletes a one or two year subspecialty fellowship in clinical neurophysiology and/or epilepsy in addition to the training in epilepsy that a pediatric neurologist receives.
    [Show full text]
  • Epileptic Syndromes in Infancy and Childhood Rima Nabbouta,B and Olivier Dulaca,B
    Epileptic syndromes in infancy and childhood Rima Nabbouta,b and Olivier Dulaca,b aDepartment of Neuropediatrics, Centre de re´fe´rence Purpose of review e´pilepsies rares, Hoˆpital Necker-Enfants malades, APHP, Necker-Enfants malades and bUniversity Paris The aim of this article is to review new epilepsy syndromes, acquire a new Descartes, Paris, France understanding of older ones and emphasize the impact of this concept on basic Correspondence to Rima Nabbout, MD, PhD, research regarding aetiology and treatment. Department of Neuropediatrics, Centre de re´fe´rence Recent findings e´pilepsies rares, Hoˆpital Necker-Enfants malades, APHP, 149 rue de Se`vres, Paris Cedex 15, F-75743, In addition to those included in the classification of the International League Against France Epilepsy, new epilepsy syndromes comprise febrile seizures plus, benign familial Tel: +33 1 42192695; fax: +33 1 42192692; e-mail: [email protected], neonatal–infantile seizures (BFNIS), benign infantile focal epilepsy with midline spikes [email protected] and waves during sleep (BFIS), malignant migrating partial seizures in infancy, devastating epilepsy in school age children and late onset cryptogenic spasms. Current Opinion in Neurology 2008, 21:161–166 Genetics played a central role in identifying some new entities (BFNIS, BFIS with choreoathetosis), to delineate older syndromes (Dravet syndrome and myoclonic astatic epilepsy) and determine their mechanisms (infantile spasms, pyridoxine dependent seizures, neonatal encephalopathy with suppression bursts). Summary A significant number of children, mainly infants, do not fit in any of the described epilepsy syndromes. Still many patients with infantile epilepsy require the identification of cause or recognition of an epilepsy syndrome.
    [Show full text]
  • Frontal-Temporal-Lobe-Epilepsy.Pdf
    Epilepsy & Behavior 99 (2019) 106487 Contents lists available at ScienceDirect Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh Are frontal and temporal lobe epilepsy dissociable in their memory functioning? Michelle Y. Kibby a,⁎, Morris J. Cohen b, Lisa Stanford c, Yong D. Park d a Southern Illinois University, Department of Psychology, LSII, Room 281, Carbondale, IL 62901-6502, USA b Pediatric Neuropsychology International, 2963 Foxhall Circle, Augusta, GA 30907, USA c NeuroDevelopmental Science Center, Akron Children's Hospital, Considine Professional Building, 215 W. Bowery St., Suite 4400, Akron, OH 44308, United States of America d Department of Neurology, Medical College of Georgia at Augusta University Children's Medical Center, 1446 Harper Street, Augusta, GA 30912, USA article info abstract Article history: There is controversy in the literature as to how dissociable frontal lobe epilepsy (FLE) and temporal lobe epilepsy Received 15 April 2019 (TLE) are in terms of memory deficits. Some researchers have demonstrated that FLE is associated with greater Revised 6 August 2019 executive dysfunction including working memory, whereas TLE is associated with greater memory impairment. Accepted 6 August 2019 Others have found the two groups to be comparable in memory functioning. Hence, we examined this question in Available online xxxx children with FLE and TLE versus typically developing controls. We found most of the expected effects when the groups with focal onset epilepsy were compared to controls. Specifically, children with left TLE performed worse Keywords: Temporal lobe epilepsy on verbal short-term memory/learning and long-term memory measures. In contrast, children with right TLE ex- Frontal lobe epilepsy hibited a more global pattern of difficulty on short-term memory/learning measures but performed worse than Working memory controls on long-term memory for faces.
    [Show full text]
  • Focal and Multifocal Seizures Sign up for Our Quarterly Newsletter
    PATIENTS OR CAREGIVERS ADVOCATES PROVIDERS OR RESEARCHERS DONORS Home Sign Up for Newsletter Who We Are What We Do Contact Donate Now Disorder Directory: Learn from the Experts EDUCATIONAL ARTICLES PLUS FAMILY STORIES AND RESOURCES INDEX ABCDEFGHIJKLMNOPQRSTUVWXYZ Focal and Multifocal Seizures Sign Up for Our Quarterly Newsletter ARTICLE FAMILY STORIES RESOURCES Get “Families First” plus updates on grants, family resources and more. Sign Up Now SAMIYA AHMAD, MD & YU-TZE NG, MD, FRACP Samiya Ahmad, MD is an Assistant Professor of Pediatrics at Baylor College of Find Us On Medicine and the Children’s Hospital of San Antonio in San Antonio, TX. She received her medical degree from Dow Medical College in Karachi, Pakistan. She began her pediatric residency at Saint Louis University in Saint Louis, MO. Dr. Ahmad went on to complete her pediatric, child neurology, and sleep Recent Tweets medicine training at Baylor College of Medicine in Houston, TX. After completion of her sleep medicine fellowship, she worked at the University of 2/25/16 - Great read! Texas Health Science Center at San Antonio as an Assistant Professor of @disabilityscoop In Bid To Pediatrics and Neurology. Her areas of expertise include sleep disorders, Understand Autism, Scientists Turn particularly those in children with neurodevelopmental disabilities. She is a To Monkeys https://t.co/5t9uz6NhWu https://t.co/5t9uz6NhWu Diplomate of the American Board of Psychiatry and Neurology with Special Qualification in Pediatric Neurology. She is married to neonatologist Kaashif Ahmad and is the proud mother of two wonderful children. 2/25/16 - Informative read! @mnt Epilepsy and marijuana: could Yu-tze Ng, MBBS, FRACP is the section chief for pediatric neurology for the cannabidiol reduce seizures? Children’s Hospital of San Antonio, Professor of Pediatrics at Baylor College of https://t.co/E3TlMqTQpy Medicine (BCM) serves as the Chair Holder for the Gary Dudley and Charlie https://t.co/E3TlMqTQpy Amato Chair in Pediatric Neurology.
    [Show full text]