Early marker of CLN2: Is neurophysiology helpful? A. Kaminska, Department of Neurophysiology, Hôpital Necker Enfants Malades, Paris, France
|
|
|
- Owen Golden
- 5 years ago
- Views:
Transcription
1 Early marker of CLN2: Is neurophysiology helpful? A. Kaminska, Department of Neurophysiology, Hôpital Necker Enfants Malades, Paris, France U/CLN2/0142 October 2017
2 Lectures: Biomarin Disclosures
3 14 patients Patients Age at disease onset: 3y (2-3.8) First symptom: Epilepsy (50%), delayed speech: (79%), regression: (14%); balance disorder (14%) Age at diagnosis 4.2 y ( )
4 Intermittent Photic stimulation (IPS) White Light flashes Patient at 30 cm from photic stimulator, look at the lamp Trains of 5 s at (1,2,6,8,10,12, Hz) separated with 7 s of pause Eye closure, eyes closed, eyes open EEG without IPS with eyes open and closed
5 Photoparoxysmal response (PPR) First EEG: 4 years ( ) First IPS : 4 months (0-23) after first EEG. PPR on the first EEG with IPS: 43% (6/14) Delay from first IPS to first PPR=0.1 y ( y PPR during follow-up: 93% (13/14)
6
7 Visual Evoked Potential At 4.5 y ( ) Abnormal 12/14 (86%) Prolonged P100 (14%) Absent waveforms (14%) Giant VEP (57%) present in all patients with early PPR
8 Photoparoxysmal response (PPR) PPR at <3 Hz, time locked (flash-per-flash): 69% (9/13) PPR at medium frequencies (5-20 Hz) IPS: 54 % PPR only at medium IPS frequencies: 23% PPR only at high IPS frequencies (>20 Hz): 15%
9 Time-locked «flash per flash» PPR at 1 Hz IPS in 3 y old boy with CLN2, Necker Hospital, Paris
10 PPR = entrainment to IPS IPS in healthy 4 y old boy Necker Hospital, Paris
11 PPR = entrainment to IPS IPS in healthy 6 y old girl Necker Hospital, Paris
12 PPR = entrainment to IPS IPS in healthy 6 y old girl Necker Hospital, Paris
13 Other EEG features Background activity at first examination: Normal:46% Slow and/or poorly organized, asymmetric (54%), rare spindles Epileptiform abnormalities: Focal (77%),Temporal bilateral (54%) Diffuse (15%) Worsening with age: Slower, low voltage, poorly organized, monomorphic Slow focal abnormalities (71%), epileptiform discharges (29%) Diffuse abnormalities (64%) Electroclinical response at IPS with myoclonic jerks (57%)
14 Cheyenne Développement normal jusqu à l âge de 3 ans Début à 3 ans une crise hémi corporelle 3-4 minutes, récidive un mois plus tard, myoclonies responsables de chutes. Ralentissement psychomoteur, persistance de crises Examen à 4 ans : Myoclonies erratiques et massives, myoclonies d intention. Ataxie, syndrome pyramidal. Stagnation psychomotrice. 1 er EEG à Necker à L âge de 4 ans Diagnostic de CLN2 à 5 ans
15 Cheyenne.. 3 ans 10 mois 150µV 1s
16 Cheyenne 3 ans 10 mois
17 Cheyenne 3 ans 10 mois
18 Cheyenne 3 ans 10 mois
19 Cheyenne 3 ans 10 mois
20 Background anomalies in sleep, multifocal spikes and myoclonus The present video is owned by the speaker and is presented under her responsibility. Prior permission was received from the patient or family. The video is not shared with any ther party. Taking pictures is prohibited 4 y old girl with CLN2, Necker Hospital, Paris
21 Cheyenne 4 ans 8 mois
22 Time-locked «flash per flash» PPR at 1 Hz IPS in 4 y old girl with CLN2, Necker Hospital, Paris
23 Myoclonic status epilepticus EEG in awakening in 4 y old girl with CLN2, Necker Hospital, Paris
24 Myoclonic status epilepticus The present video is owned by the speaker and is presented under her responsibility. Prior permission was received from the patient or family. The video is not shared with any ther party. Taking pictures is prohibited 4 y old girl with CLN2, Necker Hospital, Paris
25 Electroclinical response at IPS at 1 Hz The present video is owned by the speaker and is presented under her responsibility. Prior permission was received from the patient or family. The video is not shared with any ther party. Taking pictures is prohibited IPS in 8 old boy with CLN2, Necker Hospital, Paris
26 15 patients First EEG: 4 years (2-15) Total : 35 EEG, (8 without IPS) Photoparoxysmal response (PPR) On the first EEG = 50% (7/14) At low frequencies: 4/7 Time locked: 4/7 At > 4 Hz: 3/7 Bursts of GSW: 5/7
27 Photoparoxysmal response (PPR) PPR on at last one EEG during F-U=60% (9/15), (n=35 EEG) In 9/10 patients at 3-5 years In 7/9 on the initial EEG bi-occipital (63%) > GSW at slow frequencies at onset of IPS (88%) at 1-3 Hz (76%) «Sentinel» discharge (30%)
28 Time-locked PPR at <3 Hz
29 Sentinel discharge
30
31 CAE=18% JME=50-90% JAE=8% Jeavons Sd=>90% BME=10% Dravet syndrome=30-50% MAE=11%
32 PPR in «genetic» Generalized Epilepsy IPS in 10 old girl with JME, Necker Hospital, Paris
33 PPR in Childhood absence epilepsy IPS in 10y old boy with CAE, Necker Hospital, Paris
34 Electroclinical response at IPS in JME The present video is owned by the speaker and is presented under her responsibility. Prior permission was received from the patient or family. The video is not shared with any ther party. Taking pictures is prohibited IPS in 18 y old girl with JME, Necker Hospital, Paris
35 Conclusion (1) PPR on the first EEG in CLN2 : about 50 % Increase up to 90 % in the following year Peculiar features: Time-locked, at <3 Hz, (non specific of CLN2 ; Merrf, Lafora disease, Gaucher..) PPR>5 Hz exclusive is possible Other EEG features are helpful (background activity)
36 Time-locked «flash per flash» PPR at 1 Hz IPS in 5 y girl with MERRF disease, Necker Hospital, Paris
37 Conclusion (2) use EEG recommendations EEG should systematically include IPS with low frequencies (starting at Hz). Standardized and correctly conduced technique of IPS Obtain sleep during EEG recordings in <5 y child.
38 Conclusion (3) IPS at slow frequencies should be performed at the first EEG in the context of beginning myoclonic epilepsy +/- Speech delay +/- balance disorders/ cognitive regression +/- MRI abnormalities Increase the duration of IPS at slow frequencies to 1-2 minutes
39 Conclusion (4) If clinical context of CLN2 VEP, SSEP VEP: increased latency, decreased amplitude, giant SSEP ERG Latency, amplitude
40 DISCUSSION
41 Differential diagnosis :Myoclonic epilepsy in infancy 3 y old girl with MEI, Necker Hospital, Paris
42 Differential diagnosis: epilepsy with myoclonic atonic seizures, Myoclonic Status epilepticus EEG in awakening in 7 y old girl with MAE, Necker Hospital, Paris
43 Differential diagnosis: epilepsy with myoclonic atonic seizures Status epilepticus in 4 year old boy, Necker Hospital, Paris
Epilepsy and EEG in Clinical Practice
Mayo School of Professional Development Epilepsy and EEG in Clinical Practice November 10-12, 2016 Hard Rock Hotel at Universal Orlando Orlando, FL Course Directors Jeffrey Britton, MD and William Tatum,
EEG in Epileptic Syndrome
EEG in Epileptic Syndrome Surachai Likasitwattanakul, M.D. Division of Neurology, Department of Pediatrics Faculty of Medicine, Siriraj Hospital Mahidol University Epileptic syndrome Electroclinical syndrome
ROLE OF EEG IN EPILEPTIC SYNDROMES ASSOCIATED WITH MYOCLONUS
Version 18 A Monthly Publication presented by Professor Yasser Metwally February 2010 ROLE OF EEG IN EPILEPTIC SYNDROMES ASSOCIATED WITH MYOCLONUS EEG is an essential component in the evaluation of epilepsy.
True Epileptiform Patterns (and some others)
True Epileptiform Patterns (and some others) a) What is epileptiform b) Some possible surprises c) Classification of generalized epileptiform patterns An epileptiform pattern Interpretative term based
The secrets of conventional EEG
The secrets of conventional EEG The spike/sharp wave activity o Electro-clinical characteristics of Spike/Sharp wave The polymorphic delta activity o Electro-clinical characteristics of Polymorphic delta
Idiopathic epilepsy syndromes
Idiopathic epilepsy syndromes Kamornwan Katanyuwong MD. Chiangmai University Hospital EST, July 2009 Diagram Sylvie Nyugen The Tich, Yann Pereon Childhood absence epilepsy (CAE) Age : onset between 4-10
EEG workshop. Epileptiform abnormalities. Definitions. Dr. Suthida Yenjun
EEG workshop Epileptiform abnormalities Paroxysmal EEG activities ( focal or generalized) are often termed epileptiform activities EEG hallmark of epilepsy Dr. Suthida Yenjun Epileptiform abnormalities
Idiopathic epilepsy syndromes
1 Idiopathic epilepsy syndromes PANISRA SUDACHAN, M.D. Pe diatric Neuro lo gis t Pediatric Neurology Department Pras at Neuro lo gic al Institute Epilepsy course 20 August 2016 Classification 2 1964 1970
Idiopathic Epileptic Syndromes
Idiopathic Epileptic Syndromes Greek words idios = self, own and personal pathic = suffer Kamornwan Katanuwong MD Chiangmai University Hospital 1 st Epilepsy Camp, Hua Hin 20 th August 2010 Is a syndrome
Classification of Seizures. Generalized Epilepsies. Classification of Seizures. Classification of Seizures. Bassel F. Shneker
Classification of Seizures Generalized Epilepsies Bassel F. Shneker Traditionally divided into grand mal and petit mal seizures ILAE classification of epileptic seizures in 1981 based on clinical observation
Electrophysiological characterisation of myoclonicatonic seizures in symptomatic continuous spike-waves during slow sleep syndrome
Clinical commentary with video sequences Epileptic Disord 2009; 11 (1): 90-4 Electrophysiological characterisation of myoclonicatonic seizures in symptomatic continuous spike-waves during slow sleep syndrome
This electronic thesis or dissertation has been downloaded from the King s Research Portal at
This electronic thesis or dissertation has been downloaded from the King s Research Portal at https://kclpure.kcl.ac.uk/portal/ Propagation of generalised discharges in idiopathic generalised epilepsy
Neurophysiology & EEG
Neurophysiology & EEG PG4 Core Curriculum Ian A. Cook, M.D. Associate Director, Laboratory of Brain, Behavior, & Pharmacology UCLA Department of Psychiatry & Biobehavioral Sciences Semel Institute for
Electroclinical Syndromes Epilepsy Syndromes. Angel W. Hernandez, MD Division Chief, Neurosciences Helen DeVos Children s Hospital Grand Rapids, MI
Electroclinical Syndromes Epilepsy Syndromes Angel W. Hernandez, MD Division Chief, Neurosciences Helen DeVos Children s Hospital Grand Rapids, MI Disclosures Research Grants: NIH (NINDS) Lundbeck GW Pharma
Generalised Epileptiform Patterns
Generalised Epileptiform Patterns Manori Wijayath Westmead Hospital, Sydney, Australia With slides from Elizabeth Walker and Andrew Bleasel Generalised Epilep-form Discharges: Outline 1. Generalised epilep.form
Idiopathic epilepsy syndromes
Idiopathic epilepsy syndromes PANISRA SUDACHAN, M.D. Pediatric Neurologist Pediatric Neurology Department Prasat Neurological Institue Epilepsy course 8 September 2018 Outline of topic Definition Idiopathic
The EEG in focal epilepsy. Bassel Abou-Khalil, M.D. Vanderbilt University Medical Center
The EEG in focal epilepsy Bassel Abou-Khalil, M.D. Vanderbilt University Medical Center I have no financial relationships to disclose that are relative to the content of my presentation Learning Objectives
EEG in the Evaluation of Epilepsy. Douglas R. Nordli, Jr., MD
EEG in the Evaluation of Epilepsy Douglas R. Nordli, Jr., MD Contents Epidemiology First seizure Positive predictive value Risk of recurrence Identifying epilepsy Type of epilepsy (background and IEDs)
Normal EEG of wakeful resting adults of years of age. Alpha rhythm. Alpha rhythm. Alpha rhythm. Normal EEG of the wakeful adult at rest
Normal EEG of wakeful resting adults of 20-60 years of age Suthida Yenjun, M.D. Normal EEG of the wakeful adult at rest Alpha rhythm Beta rhythm Mu rhythm Vertex sharp transients Intermittent posterior
Myoclonic status epilepticus in hypoxic ischemic encephalopathy which recurred after somatosensory evoked potential testing
ANNALS OF CLINICAL NEUROPHYSIOLOGY CASE REPORT Ann Clin Neurophysiol 2017;19(2):136-140 Myoclonic status epilepticus in hypoxic ischemic encephalopathy which recurred after somatosensory evoked potential
Idiopathic epilepsy syndromes
Idiopathic epilepsy syndromes PANISRA SUDACHAN, M.D. Pediatric Neurologist Pediatric Neurology Department Prasat Neurological Institue Epilepsy course 26 August 2017 Classification 1964 1970 1981 1989
Scope. EEG patterns in Encephalopathy. Diffuse encephalopathy. EEG in adult patients with. EEG in diffuse encephalopathy
Scope EEG patterns in Encephalopathy Dr.Pasiri Sithinamsuwan Division of Neurology Department of Medicine Phramongkutklao Hospital Diffuse encephalopathy EEG in specific encephalopathies Encephalitides
Epilepsy Syndromes: Where does Dravet Syndrome fit in?
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
Introduction to EEG del Campo. Introduction to EEG. J.C. Martin del Campo, MD, FRCP University Health Network Toronto, Canada
Introduction to EEG J.C. Martin, MD, FRCP University Health Network Toronto, Canada What is EEG? A graphic representation of the difference in voltage between two different cerebral locations plotted over
Dravet syndrome : Clinical presentation, genetic investigation and anti-seizure medication. Bradley Osterman MD, FRCPC, CSCN
Dravet syndrome : Clinical presentation, genetic investigation and anti-seizure medication Bradley Osterman MD, FRCPC, CSCN Objectives Learn about the typical early clinical presentation of Dravet syndrome
La sindrome di Angelman: il problema delle crisi
La sindrome di Angelman: il problema delle crisi Crisi o non crisi? Questo è il problema. Pedagogia dell anamnesi: ovvero come raccontare una crisi al medico, sperando che non vada fuori strada L inizio
EEG in Medical Practice
EEG in Medical Practice Dr. Md. Mahmudur Rahman Siddiqui MBBS, FCPS, FACP, FCCP Associate Professor, Dept. of Medicine Anwer Khan Modern Medical College What is the EEG? The brain normally produces tiny
Fixation-off sensitivity in epilepsies other than the idiopathic epilepsies of childhood with occipital paroxysms: a 12-year clinical-video EEG study
Original article Epileptic Disord 2009; 11 (1): 20-36 Fixation-off sensitivity in epilepsies other than the idiopathic epilepsies of childhood with occipital paroxysms: a 12-year clinical-video EEG study
Childhood Epilepsy Syndromes. Epileptic Encephalopathies. Today s Discussion. Catastrophic Epilepsies of Childhood
CATASTROPHIC EPILEPSIES OF CHILDHOOD EPILEPTIC ENCEPHALOPATHIES Dean Sarco, MD Department of Neurology Kaiser Permanente Los Angeles Medical Center Childhood Epilepsy Syndromes Epilepsy Syndrome Grouping
Ketogenic Diet therapy in Myoclonic-Atonic Epilepsy (MAE)
KD therapy in epilepsy syndromes Ketogenic Diet therapy in Myoclonic-Atonic Epilepsy (MAE) Hirokazu Oguni, MD Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan Epilepsy Center, TMG
ICD-9 to ICD-10 Conversion of Epilepsy
ICD-9-CM 345.00 Generalized nonconvulsive epilepsy, without mention of ICD-10-CM G40.A01 Absence epileptic syndrome, not intractable, with status G40.A09 Absence epileptic syndrome, not intractable, without
Subhairline EEG Part II - Encephalopathy
Subhairline EEG Part II - Encephalopathy Teneille Gofton September 2013 Objectives To review the subhairline EEG changes seen with encephalopathy To discuss specific EEG findings in encephalopathy To outline
Disclosure Age Hauser, Epilepsia 33:1992
Pediatric Epilepsy Syndromes Gregory Neal Barnes MD/PhD Assistant Professor of Neurology and Pediatrics Director, Pediatric Epilepsy Monitoring Unit Vanderbilt University Medical Center Disclosure Investigator:
EEG in the ICU. Quiz. March Teneille E. Gofton
EEG in the ICU Quiz March 2012 Teneille E. Gofton Quiz The next several slides will show 15 subhairline EEGs. Choose the best possible answer in each scenario. Your score and solutions will be provided
EEG in Benign and Malignant Epileptic Syndromes of Childhood
Epilepsia, 43(Suppl. 3):17 26, 2002 Blackwell Publishing, Inc. International League Against Epilepsy EEG in Benign and Malignant Epileptic Syndromes of Childhood Ivo Drury Department of Neurology, Henry
Beyond the Basics in EEG Interpretation: Throughout the Life Stages
Beyond the Basics in EEG Interpretation: Throughout the Life Stages Steve S. Chung, MD, FAAN Chairman, Neuroscience Institute Director, Epilepsy Program Banner University Medical Center University of Arizona
Non epileptiform abnormality J U LY 2 7,
Non epileptiform abnormality S U D A J I R A S A K U L D E J, M D. C H U L A L O N G KO R N C O M P R E H E N S I V E E P I L E P S Y C E N T E R J U LY 2 7, 2 0 1 6 Outline Slow pattern Focal slowing
Asian Epilepsy Academy (ASEPA) & ASEAN Neurological Association (ASNA) EEG Certification Examination
Asian Epilepsy Academy (ASEPA) & ASEAN Neurological Association (ASNA) EEG Certification Examination EEG Certification Examination Aims To set and improve the standard of practice of Electroencephalography
Effects of Sleep and Circadian Rhythms on Epilepsy
Effects of Sleep and Circadian Rhythms on Epilepsy Milena Pavlova, M.D. Medical Director, Faulkner Neurophysiology Laboratory Department of Neurology, Brigham and Women s Hospital Harvard Medical School
Asian Epilepsy Academy (ASEPA) EEG Certification Examination
Asian Epilepsy Academy (ASEPA) EEG Certification Examination EEG Certification Examination Aims To set and improve the standard of practice of Electroencephalography (EEG) in the Asian Oceanian region
Overlap cases of eyelid myoclonia with absences and juvenile myoclonic epilepsy
Seizure (2006) 15, 359 365 www.elsevier.com/locate/yseiz Overlap cases of eyelid myoclonia with absences and juvenile myoclonic epilepsy A. Destina Yalçın *, Hulki Forta, Elif Kılıç Neurology Clinic, Şişli
Neonatal Seizure Cases. Courtney Wusthoff, MD MS Assistant Professor, Neurology Neurology Director, LPCH Neuro NICU
Neonatal Seizure Cases Courtney Wusthoff, MD MS Assistant Professor, Neurology Neurology Director, LPCH Neuro NICU Disclosures I have no conflicts of interest I will discuss off-label use of anti-epileptic
Overview: Idiopathic Generalized Epilepsies
Epilepsia, 44(Suppl. 2):2 6, 2003 Blackwell Publishing, Inc. 2003 International League Against Epilepsy Overview: Idiopathic Generalized Epilepsies Richard H. Mattson Department of Neurology, Yale University
Neurophysiologic Assessment
Neurophysiologic Assessment Electrophysiology is very helpful to detect whether myoclonus is cortical, subcortical or spinal/segmental. Polymyography is the first step in the neurophysiologic assessment
AMERICAN BOARD OF PSYCHIATRY AND NEUROLOGY, INC. SUBSPECIALTY CERTIFICATION EXAMINATION IN EPILEPSY MEDICINE
SUBSPECIALTY CERTIFICATION EXAMINATION IN EPILEPSY MEDICINE 2014 Content Blueprint (November 26, 2012) Number of questions: 200 I. Classification 7 9% II. Routine EEG 16 20% III. Evaluation 22 26% IV.
Idiopathic Photosensitive Occipital Lobe Epilepsy
Idiopathic Photosensitive Occipital Lobe Epilepsy 2 Idiopathic photosensitive occipital lobe epilepsy (IPOE) 5, 12, 73, 75, 109, 110 manifests with focal seizures of occipital lobe origin, which are elicited
ENCEPHALOPATHY RECOGNIZING METABOLIC AND ANOXIC CHANGES
ENCEPHALOPATHY RECOGNIZING METABOLIC AND ANOXIC CHANGES ENCEPHALOPATHY Encephalopathy is a general term that means brain disease, damage, or malfunction. The major symptom of encephalopathy is an altered
Chronic Management of Idiopathic Generalized epilepsies (IGE) Hassan S.Hosny M.D. Prof of Neurology, Cairo University
Chronic Management of Idiopathic Generalized epilepsies (IGE) Hassan S.Hosny M.D. Prof of Neurology, Cairo University Sanaa 2009 Points of Discussion Prevalence compared to focal epilepsy Adult form Status
Ketogenic diet in patients with myoclonic-astatic epilepsy
Original article Epileptic Disord 2006; 8 (2): 151-5 Ketogenic diet in patients with myoclonic-astatic epilepsy Roberto Horacio Caraballo 1, Ricardo Oscar Cersósimo 1, Diego Sakr 1, Araceli Cresta 2, Nidia
Post-anoxic status epilepticus and EEG patterns
Post-anoxic status epilepticus and EEG patterns Nicolas Gaspard, MD, PhD Université Libre de Bruxelles Hôpital Erasme, Bruxelles, Belgique Yale University School of Medicine, New Haven, CT, USA DISCLOSURES
Localization a quick look
Localization a quick look Covering the basics Differential amplifiers Polarity convention 10-20 electrode system Basic montages: bipolar and referential Other aspects of displaying the EEG Localization
EEG IN FOCAL ENCEPHALOPATHIES: CEREBROVASCULAR DISEASE, NEOPLASMS, AND INFECTIONS
246 Figure 8.7: FIRDA. The patient has a history of nonspecific cognitive decline and multiple small WM changes on imaging. oligodendrocytic tumors of the cerebral hemispheres (11,12). Electroencephalogram
Photosensitivity in epileptic syndromes of childhood and adolescence
Original article Epileptic Disord 2008; 10 (2): 136-43 Photosensitivity in epileptic syndromes of childhood and adolescence Yang Lu 1,2, Stephan Waltz 1,3, Katja Stenzel 1,4, Hiltrud Muhle 1, Ulrich Stephani
Clinical Course and EEG Findings of 25 Patients Initially Diagnosed with Childhood Absence Epilepsy
Med. Bull. Fukuoka Univ.403/4105 1102013 Clinical Course and EEG Findings of 25 Patients Initially Diagnosed with Childhood Absence Epilepsy Noriko NAKAMURA, Sawa YASUMOTO, Takako FUJITA, Yuko TOMONOH,
A study of 72 children with eyelid myoclonia precipitated by eye closure in Yogyakarta
Neurol J Southeast Asia 2003; 8 : 15 23 A study of 72 children with eyelid myoclonia precipitated by eye closure in Yogyakarta Harsono MD Department of Neurology, Faculty of Medicine, Gadjah Mada University,
Epileptic Seizures, Syndromes, and Classifications. Heidi Currier, MD Minnesota Epilepsy Group, PA St. Paul, MN
Epileptic Seizures, Syndromes, and Classifications Heidi Currier, MD Minnesota Epilepsy Group, PA St. Paul, MN Definitions Diagnosis of Seizures A seizure is a sudden surge of electrical activity in the
Dysfunctional FINDINGS IN EEG. About 18 Cases were Seen in Consultation in our Hospital
Cronicon OPEN ACCESS NEUROLOGY Clinical Images Angel Molina Leon* Dysfunctional FINDINGS IN EEG. About 18 Cases were Seen in Consultation in our Hospital Clinical Neurophysiology Service. Centro medico
Fixation-Off and Eyes Closed Catamenial Generalized Nonconvulsive Status Epilepticus with Eyelid Myoclonic Jerks
~ ~ ~~ ~ ~~~~ Epilepsia, 39(6):664468. 1998 Lippincott-Raven Publishers, Philadelphia 0 International League Against Epilepsy Fixation-Off and Eyes Closed Catamenial Generalized Nonconvulsive Status Epilepticus
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
Introduction. Clinical manifestations. Historical note and terminology
Epilepsy with myoclonic absences Douglas R Nordli Jr MD ( Dr. Nordli of University of Southern California, Keck School of Medicine has no relevant financial relationships to disclose. ) Jerome Engel Jr
Benign infantile focal epilepsy with midline spikes and waves during sleep: a new epileptic syndrome or a variant of benign focal epilepsy?
riginal article Epileptic Disord 2010; 12 (3): 205-11 Benign infantile focal epilepsy with midline spikes and waves during sleep: a new epileptic syndrome or a variant of benign focal epilepsy? Santiago
Electroencephalography. Role of EEG in NCSE. Continuous EEG in ICU 25/05/59. EEG pattern in status epilepticus
EEG: ICU monitoring & 2 interesting cases Electroencephalography Techniques Paper EEG digital video electroencephalography Dr. Pasiri Sithinamsuwan PMK Hospital Routine EEG long term monitoring Continuous
Multiple Choice Questions for Part I
Multiple Choice Questions for Part I 1. Neurons in the cerebral cortex are organized in: A. Three horizontal layers B. Four horizontal layers C. Six horizontal layers with layer IV receiving inputs from
Common EEG pattern in critical care
Common EEG pattern in critical care พ.ญ.ส ธ ดา เย นจ นทร Causes Direct neuronal injury Cerebral dysfunction : encephalopathy Psychic problems EEG in critical care 1 October 2009, Pramongkutklao Hospital
Photic Stimulation. Literature Review. Dr RM Sherratt Consultant Neurophysiologist Luton & Dunstable University Hospital
Photic Stimulation Literature Review Dr RM Sherratt Consultant Neurophysiologist Luton & Dunstable University Hospital Technical and Methodological Considerations in Photic Stimulation for EEG RM Sherratt
Classification of Epilepsy: What s new? A/Professor Annie Bye
Classification of Epilepsy: What s new? A/Professor Annie Bye The following material on the new epilepsy classification is based on the following 3 papers: Scheffer et al. ILAE classification of the epilepsies:
Photosensitivity in Idiopathic Generalized Epilepsies
Epilepsia, 46(Suppl. 9):67 72, 2005 Blackwell Publishing, Inc. C International League Against Epilepsy Photosensitivity in Idiopathic Generalized Epilepsies Athanasios Covanis Neurology Department, The
Epilepsy. Annual Incidence. Adult Epilepsy Update
Adult Epilepsy Update Annual Incidence J. Layne Moore, MD, MPH Associate Professor Department of Neurology and Pharmacy Director, Division of Epilepsy The Ohio State University Used by permission Health
No relevant disclosures
No relevant disclosures - Epileptic Encephalopathy (EE): Epileptic activity itself contributes to cognitive and behavioural impairments - Developmental and Epileptic Encephalopathy (DEE): Impairments occur
Classification of Status Epilepticus: A New Proposal Dan Lowenstein, M.D. University of California, San Francisco
Classification of Status Epilepticus: A New Proposal Dan Lowenstein, M.D. University of California, San Francisco for the ILAE Taskforce for Classification of Status Epilepticus: Eugen Trinka, Hannah Cock,
Childhood absence epilepsy. EEG Course 2010: Generalized Epileptiforms. Juvenile absence epilepsy. Childhood Absence Epilepsy - video
Childhood absence epilepsy EEG Course 2010: Generalized Epileptiforms Pongkiat Kankirawatana, MD. Division of Pediatric Neurology University of Alabama at Birmingham 10% prevalence, incidence 7/100,000/yr
Myoclonic encephalopathy in the CDKL5 gene mutation
Clinical Neurophysiology 117 (2006) 223 227 www.elsevier.com/locate/clinph Myoclonic encephalopathy in the CDKL5 gene mutation Sabrina Buoni a, *, Raffaella Zannolli a, **, Vito Colamaria b, Francesca
Generalized seizures, generalized spike-waves and other things. Charles Deacon MD FRCPC Centre Hospitalier Universitaire de Sherbrooke
Generalized seizures, generalized spike-waves and other things Charles Deacon MD FRCPC Centre Hospitalier Universitaire de Sherbrooke Objectives Give an overview of generalized EEG discharges and seizures
The 2017 ILAE Classification of Seizures
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,
Epilepsy: diagnosis and treatment. Sergiusz Jóźwiak Klinika Neurologii Dziecięcej WUM
Epilepsy: diagnosis and treatment Sergiusz Jóźwiak Klinika Neurologii Dziecięcej WUM Definition: the clinical manifestation of an excessive excitation of a population of cortical neurons Neurotransmitters:
Epileptic syndrome in Neonates and Infants. Piradee Suwanpakdee, MD. Division of Neurology Department of Pediatrics Phramongkutklao Hospital
Epileptic syndrome in Neonates and Infants Piradee Suwanpakdee, MD. Division of Neurology Department of Pediatrics Phramongkutklao Hospital AGE SPECIFIC INCIDENCE OF EPILEPSY Hauser WA, et al. Epilepsia.
SUBSPECIALTY CERTIFICATION EXAMINATION IN EPILEPSY MEDICINE Content Blueprint (December 21, 2015)
SUBSPECIALTY CERTIFICATION EXAMINATION IN EPILEPSY MEDICINE 2016 Content Blueprint (December 21, 2015) Number of questions: 200 1. Classification 8-12% 2. Routine EEG 16-20% 3. Evaluation 23-27% 4. Management
Periodic and Rhythmic Patterns. Suzette M LaRoche, MD Mission Health Epilepsy Center Asheville, North Carolina
Periodic and Rhythmic Patterns Suzette M LaRoche, MD Mission Health Epilepsy Center Asheville, North Carolina Continuum of EEG Activity Neuronal Injury LRDA GPDs SIRPIDs LPDs + NCS Burst-Suppression LPDs
Practical 3 Nervous System Physiology 2 nd year English Module. Dept. of Physiology, Carol Davila University of Medicine and Pharmacy
Electroencephalography l h (EEG) Practical 3 Nervous System Physiology 2 nd year English Module Dept. of Physiology, Carol Davila University of Medicine and Pharmacy What is EEG EEG noninvasively records
Focal epilepsy recruiting a generalised network of juvenile myoclonic epilepsy: a case report
Clinical commentary Epileptic Disord 2014; 16 (3): 370-4 Focal epilepsy recruiting a generalised network of juvenile myoclonic epilepsy: a case report Myo Khaing 1,2, Kheng-Seang Lim 1, Chong-Tin Tan 1
Epileptic Seizures, Syndromes and Classifications
Epileptic Seizures, Syndromes and Classifications Randa Jarrar, MD Child Neurologist Phoenix Children's Hospital Clinical Assistant Professor, Department of Pediatrics University of Arizona Assistant Professor,
DIFFERENTIATION OF TYPICAL ABSENCE SEIZURES IN EPILEPTIC SYNDROMES
Brain (1989), 112, 1039-1056 DIERENTIATION O TYPICAL ABSENCE SEIZURES IN EPILEPTIC SYNDROMES A VIDEO EEG STUDY O 224 SEIZURES IN 20 PATIENTS by C. P. PANAYIOTOPOULOS, T. OBEID and G. WAHEED (rom the Division
Seizure Semiology and Neuroimaging Findings in Patients with Midline Spikes
Epilepsia, 42(12):1563 1568, 2001 Blackwell Science, Inc. International League Against Epilepsy Seizure Semiology and Neuroimaging Findings in Patients with Midline Spikes *Ekrem Kutluay, *Erasmo A. Passaro,
*Pathophysiology of. Epilepsy
*Pathophysiology of Epilepsy *Objectives * At the end of this lecture the students should be able to:- 1.Define Epilepsy 2.Etio-pathology of Epilepsy 3.Types of Epilepsy 4.Role of Genetic in Epilepsy 5.Clinical
3 rd video - EEG course. in paediatric epilepsies: from seizures to syndromes PRO GRA MME. 1 st - 3 rd November 2018 MADRID.
3 rd video - EEG course 1 st - 3 rd November 2018 MADRID www.video-eeg2018.com Day 1 1st of November 9:00-9:30 resentation of the course: P D. Stefano Sannino, H.E. Ambassador of Italy in Spain D. César
Objectives. Amanda Diamond, MD
Amanda Diamond, MD Objectives Recognize symptoms suggestive of seizure and what those clinical symptoms represent Understand classification of epilepsy and why this is important Identify the appropriate
Epilepsy 101. Russell P. Saneto, DO, PhD. Seattle Children s Hospital/University of Washington November 2011
Epilepsy 101 Russell P. Saneto, DO, PhD Seattle Children s Hospital/University of Washington November 2011 Specific Aims How do we define epilepsy? Do seizures equal epilepsy? What are seizures? Seizure
EEG IN THE DIAGNOSIS, CLASSIFICATION, AND MANAGEMENT OF PATIENTS WITH EPILEPSY
ii2 EEG IN THE DIAGNOSIS, CLASSIFICATION, AND MANAGEMENT OF PATIENTS WITH EPILEPSY Correspondence to: Dr Shelagh Smith, National Society for Epilepsy, Chalfont St Peter, Bucks SL9 0RJ, UK; shelaghs@epilepsynse.org.uk
Overview of the epilepsies of childhood and comorbidities
Overview of the epilepsies of childhood and comorbidities Dr Amy McTague BRC Catalyst Fellow/Honorary Consultant Paediatric Neurologist UCL Great Ormond Street Institute of Child Health Epilepsy is a common
Epilepsy in Menkes Disease: Analysis of Clinical Stages
Epilepsia, 47(2):380 386, 2006 Blackwell Publishing, Inc. C 2006 International League Against Epilepsy Epilepsy in Menkes Disease: Analysis of Clinical Stages Nadia Bahi-Buisson, Anna Kaminska, Rima Nabbout,
9 The Abnormal EEG. An EEG is considered abnormal if it has findings
C h a p t e r 9 The Abnormal EEG An EEG is considered abnormal if it has findings known to be associated with a pathologic or disease state. As discussed in Chapter 8, The Structure and Philosophy of the
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,
Video Material and Epilepsy
Epikpsiu, 35(6): 1208-12 16, 1994 Raven Press, Ltd., New York 0 International League Against Epilepsy Video Material and Epilepsy G. F. A. Harding, P. M. Jeavons, and A. S. Edson Clinical Neurophysiology
Angelman syndrome and pseudo-hypsarrhythmia: a diagnostic pitfall
Clinical commentary with video sequences Epileptic Disord 2011; 13 (3): 331-5 Angelman syndrome and pseudo-hypsarrhythmia: a diagnostic pitfall Stephane Darteyre 1, Laure Mazzola 2, Philippe Convers 2,
AMERICAN BOARD OF CLINICAL NEUROPHYSIOLOGY
AMERICAN BOARD OF CLINICAL NEUROPHYSIOLOGY Part I Content Outline I. Physiology and Instrumentation 30% A. Physiology 1. Anatomy of neural generation 2. Mechanisms of EEG and evoked potential generation
Early predictors of refractory epileptic seizures
Mohamed M.Mostafa et al. Early predictors of refractory epileptic seizures Mohamed M.Mostafa 1, Ali Akram 2, Mohamed Osman 3, Mamdooh Eissa 4 Depatments of Neurology, Ain Shams University 1, Psychiatry
Seizure 19 (2010) Contents lists available at ScienceDirect. Seizure. journal homepage:
Seizure 19 (2010) 368 372 Contents lists available at ScienceDirect Seizure journal homepage: www.elsevier.com/locate/yseiz Case report Gastaut type-idiopathic childhood occipital epilepsy and childhood
The Fitting Child. A/Prof Alex Tang
The Fitting Child A/Prof Alex Tang Objective Define relevant history taking and physical examination Classify the types of epilepsy in children Demonstrate the usefulness of investigations Define treatment
Sleep in Epilepsy. Kurupath Radhakrishnan,
Sleep in Epilepsy Kurupath Radhakrishnan, Retired Senior Professor (Emeritus), R. Madavan Nayar Center for Comprehensive Epilepsy Care, Retired Director, Sree Chitra Tirunal Institute for Medical Sciences