Epileptology Epileptology the Modern State of Science Epileptology the Modern State of Science
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Pediatric Epilepsy Clips by Pediatric Elizabeth Medaugh, MSN, CPNP-PC , Pediatric Nurse Practitioner, Neurology
Nursing May 2016 Pediatric Clips Pediatric Nursing Pediatric Epilepsy Clips by Pediatric Elizabeth Medaugh, MSN, CPNP-PC , Pediatric Nurse Practitioner, Neurology Advanced Practice Sophia had been exceeding down to sleep, mother heard a Nurses at Dayton Case Study the expectations of school. thump and went to check on her. Her teachers report her being Sophia’s eyes were wide open Children’s provides Sophia is a 7 year old Caucasian focused the first two quarters, and deviated left, left arm was quick reviews of female who presented to her but have found her attention to stiffened with rhythmic shaking, be somewhat off over the last Sophia’s mouth was described common pediatric primary care physician with concerns of incontinence few weeks. Teachers have asked as “sort of twisted” and Sophia conditions. in sleep. Sophia is an parents if Sophia has been was drooling. The event lasted otherwise healthy child, with getting good rest as she has 90 seconds. Following, Sophia uncomplicated birth history, been falling asleep in class. was disoriented and very sleepy. Father reports his sister Dayton Children’s appropriate developmental Sophia’s mother and father had similar events as a child. progression with regards note that she has had is the region’s Sophia’s neurological exam to early milestones, and several episodes of urinary was normal, therefore her pediatric referral previously diurnal/nocturnally incontinence each week over pediatrician made a referral toilet trained. No report of the last 3-4 weeks. Parents center for a to pediatric neurology. Given recent illness, infection or ill have limited fluids prior to bed Sophia’s presentation, what 20-county area. -
Childhood Epilepsy: an Update on Diagnosis and Management
American Journal of Neuroscience Review Childhood Epilepsy: An Update on Diagnosis and Management Khaled Saad Department of Pediatrics, Faculty of Medicine, University of Assiut, Assiut 71516, Egypt Article history Abstract: Within the past few years there is a rapid expansion in our Received: 23-10-2014 understanding of epilepsy. The development of new anti-epileptic drugs and Revised: 25-11-2014 refinements to epilepsy surgery are widening the therapeutic options for Accepted: 12-01-2015 epilepsy. In addition, the classification of the epilepsies continues to evolve based on an increased understanding of the molecular genetics of the condition and this includes the recognition of possible novel epilepsy syndromes. This review considers some of these exciting developments, as well as addressing the essential features of the diagnosis, investigations, management and impact of epilepsy in childhood. Keywords: Children, Epilepsy, Seizure, Anti-Epileptic Drugs Introduction guidelines for optimal practices, rational therapy and counseling (Aylwad, 2008; Tamber and Mountz, 2012; Epilepsy is a common heterogeneous neurological Sharma, 2013). problem in children. It exerts a significant physical, psychological, economic and social toll on children and Definitions their caregivers. Fifty million people have epilepsies A seizure is defined as an excessive burst of abnormal globally; more than half of them are children. In the synchronized neuronal activity affecting small or large USA; between 25,000 and 40,000 children will have a neuronal networks that results in clinical manifestations first non-febrile seizure each year. The problem is that are sudden, transient and usually brief. Epilepsy is further compounded in developing countries as they add defined as a disorder of the brain characterized by any of about 75-80% of new cases of epilepsy (Guerrini, 2006; the following conditions: (1) At least two unprovoked (or Tamber and Mountz, 2012; Sharma, 2013). -
Benign Rolandic Epilepsy
Benign Rolandic Epilepsy What is Benign Rolandic Epilepsy (BRE)? Benign Rolandic Epilepsy is a common, mild form of childhood epilepsy characterized by brief simple partial seizures involving the face and mouth, usually occuring at night or during the early morning hours. Seizures are the result of brief disruptions in the brain’s normal neuronal activity. Who gets this form of epilepsy? Children between the ages of three and 13 years, who have no neurological or intellectual deficit.The peak age of onset is from 9-10 years of age. BRE is more common in boys than girls. What kind of seizures occur? Typically, the child experiences a sensation then a twitching at the corner of their mouth. The jerking spreads to in- volve the tongue, cheeks and face on one side, resulting in speech arrest, problems with pronunciation and drooling. Or one side of the child’s face may feel paralyzed. Consciousness is retained. On occasion, the seizure may spread and cause the arms and legs on that side to stiffen and or jerk, or it may become a generalized tonic-clonic seizure that involves the whole body and a loss of consciousness. When do the seizures occur? Seizures tend to happen when the child is waking up or during certain stages of sleep. Seizures may be infrequent, or can happen many times a day. How is Benign Rolandic Epilepsy diagnosed? A child having such seizures is given an EEG test to confirm the diagnosis, a test that graphs the pattern of electrical activity in the brain. BRE has a typical EEG spike pattern—repetitive spike activity firing predominantly from the mid-temporal or parietal areas of the brain near the rolandic (motor) strip. -
Causes of Mortality in Early Infantile Epileptic Encephalopathy: a Systematic Review
Epilepsy & Behavior 85 (2018) 32–36 Contents lists available at ScienceDirect Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh Review Causes of mortality in early infantile epileptic encephalopathy: A systematic review Graciane Radaelli a,b, Francisco de Souza Santos b, Wyllians Vendramini Borelli b,LeonardoPisanib, Magda Lahorgue Nunes b,d, Fulvio Alexandre Scorza c,d, Jaderson Costa da Costa b,d,⁎ a Federal University of São Paulo (UNIFESP)/Paulista School of Medicine, São Paulo, Brazil b Brain Institute of Rio Grande do Sul (BraIns), Pontifical Catholic University of Rio Grande do Sul, Porto Alegre, RS, Brazil c Laboratory of Neuroscience, Department of Neurology and Neurosurgery, Federal University of São Paulo, São Paulo, SP, Brazil d CNPq, Brazil article info abstract Article history: Introduction: Early infantile epileptic encephalopathy syndrome (EIEE), also known as Ohtahara syndrome, is an Received 6 March 2018 age-dependent epileptic encephalopathy syndrome defined by clinical features and electroencephalographic Revised 25 April 2018 findings. Epileptic disorders with refractory seizures beginning in the neonatal period and/or early infancy Accepted 5 May 2018 have a potential risk of premature mortality, including sudden death. We aimed to identify the causes of death Available online xxxx in EIEE and conducted a literature survey of fatal outcomes. Methods: We performed a literature search in MEDLINE, EMBASE, and Web of Science for data from inception Keywords: “ ”“ ”“ ”“ ” “ ” Ohtahara syndrome until September 2017. The terms death sudden, unexplained death, SUDEP, lethal, and fatal and the “ ”“ ”“ ”“ Early infantile epileptic syndrome medical subject heading terms epileptic encephalopathy, mortality, death, sudden infant death syn- Suppression burst drome,” and “human” were used in the search strategy. -
Antiepileptic Drug Treatment of Rolandic Epilepsy and Panayiotopoulos
ADC Online First, published on September 8, 2014 as 10.1136/archdischild-2013-304211 Arch Dis Child: first published as 10.1136/archdischild-2013-304211 on 8 September 2014. Downloaded from Review Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility Louise C Mellish,1 Colin Dunkley,2 Colin D Ferrie,3 Deb K Pal1 ▸ Additional material is ABSTRACT published online only. To view Background The evidence base for management of What is already known on this topic? please visit the journal online (http://dx.doi.org/10.1136/ childhood epilepsy is poor, especially for the most common specific syndromes such as rolandic epilepsy (RE) archdischild-2013-304211). ▸ UK management of rolandic epilepsy and and Panayiotopoulos syndrome (PS). Considerable 1King’s College London, Panayiotopoulos syndrome are not well known international variation in management and controversy London, UK and there is limited scientific basis for drug 2 about non-treatment indicate the need for high quality Sherwood Forest Hospitals, treatment or non-treatment. Notts, UK randomised controlled trials (RCT). The aim of this study 3 ▸ Paediatric opinion towards clinical trial designs Department of Paediatric is, therefore, to describe current UK practice and explore is also unknown and important to assess prior Neurology, Leeds General the feasibility of different RCT designs for RE and PS. Infirmary, Leeds, UK to further planning. Methods We conducted an online survey of 590 UK Correspondence to paediatricians who treat epilepsy. Thirty-two questions Professor Deb K Pal, covered annual caseload, investigation and management Department of Basic and practice, factors influencing treatment, antiepileptic drug Clinical Neuroscience, King’s What this study adds? College London, Institute of preferences and hypothetical trial design preferences. -
Antiepileptic Drug Treatment of Rolandic Epilepsy and Panayiotopoulos
Review Arch Dis Child: first published as 10.1136/archdischild-2013-304211 on 8 September 2014. Downloaded from Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility Louise C Mellish,1 Colin Dunkley,2 Colin D Ferrie,3 Deb K Pal1 ▸ Additional material is ABSTRACT published online only. To view Background The evidence base for management of What is already known on this topic? please visit the journal online (http://dx.doi.org/10.1136/ childhood epilepsy is poor, especially for the most common specific syndromes such as rolandic epilepsy (RE) archdischild-2013-304211). ▸ UK management of rolandic epilepsy and and Panayiotopoulos syndrome (PS). Considerable 1King’s College London, Panayiotopoulos syndrome are not well known international variation in management and controversy London, UK and there is limited scientific basis for drug 2 about non-treatment indicate the need for high quality Sherwood Forest Hospitals, treatment or non-treatment. Notts, UK randomised controlled trials (RCT). The aim of this study 3 ▸ Paediatric opinion towards clinical trial designs Department of Paediatric is, therefore, to describe current UK practice and explore is also unknown and important to assess prior Neurology, Leeds General the feasibility of different RCT designs for RE and PS. Infirmary, Leeds, UK to further planning. Methods We conducted an online survey of 590 UK Correspondence to paediatricians who treat epilepsy. Thirty-two questions Professor Deb K Pal, covered annual caseload, investigation and management Department of Basic and practice, factors influencing treatment, antiepileptic drug Clinical Neuroscience, King’s What this study adds? College London, Institute of preferences and hypothetical trial design preferences. -
Neonatal Seizures Revisited
children Review Neonatal Seizures Revisited Konrad Kaminiów 1 , Sylwia Kozak 1 and Justyna Paprocka 2,* 1 Students’ Scientific Society, Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, Poland; [email protected] (K.K.); [email protected] (S.K.) 2 Department of Pediatric Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, 40-752 Katowice, Poland * Correspondence: [email protected] Abstract: Seizures are the most common neurological disorder in newborns and are most prevalent in the neonatal period. They are mostly caused by severe disorders of the central nervous system (CNS). However, they can also be a sign of the immaturity of the infant’s brain, which is characterized by the presence of specific factors that increase excitation and reduce inhibition. The most common disorders which result in acute brain damage and can manifest as seizures in neonates include hypoxic-ischemic encephalopathy (HIE), ischemic stroke, intracranial hemorrhage, infections of the CNS as well as electrolyte and biochemical disturbances. The therapeutic management of neonates and the prognosis are different depending on the etiology of the disorders that cause seizures which can lead to death or disability. Therefore, establishing a prompt diagnosis and implementing appropriate treatment are significant, as they can limit adverse long-term effects and improve outcomes. In this review paper, we present the latest reports on the etiology, pathomechanism, clinical symptoms and guidelines for the management of neonates with acute symptomatic seizures. Keywords: neonatal seizures; pathophysiology; genetics; inborn errors of metabolism Citation: Kaminiów, K.; Kozak, S.; Paprocka, J. -
Epilepsy Syndromes E9 (1)
EPILEPSY SYNDROMES E9 (1) Epilepsy Syndromes Last updated: September 9, 2021 CLASSIFICATION .......................................................................................................................................... 2 LOCALIZATION-RELATED (FOCAL) EPILEPSY SYNDROMES ........................................................................ 3 TEMPORAL LOBE EPILEPSY (TLE) ............................................................................................................... 3 Epidemiology ......................................................................................................................................... 3 Etiology, Pathology ................................................................................................................................ 3 Clinical Features ..................................................................................................................................... 7 Diagnosis ................................................................................................................................................ 8 Treatment ............................................................................................................................................. 15 EXTRATEMPORAL NEOCORTICAL EPILEPSY ............................................................................................... 16 Etiology ................................................................................................................................................ 16 -
ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions
ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions N Specchio1, EC Wirrell2*, IE Scheffer3, R Nabbout4, K Riney5, P Samia6, SM Zuberi7, JM Wilmshurst8, E Yozawitz9, R Pressler10, E Hirsch11, S Wiebe12, JH Cross13, P Tinuper14, S Auvin15 1. Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesu’ Children’s Hospital, IRCCS, Member of European Reference Network EpiCARE, Rome, Italy 2. Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester MN, USA. 3. University of Melbourne, Austin Health and Royal Children’s Hospital, Florey Institute, Murdoch Children’s Research Institute, Melbourne, Australia. 4. Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Necker–Enfants Malades Hospital, APHP, Member of European Reference Network EpiCARE, Institut Imagine, INSERM, UMR 1163, Université de Paris, Paris, France. 5. Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia. Faculty of Medicine, University of Queensland, Queensland, Australia. 6. Department of Paediatrics and Child Health, Aga Khan University, East Africa. 7. Paediatric Neurosciences Research Group, Royal Hospital for Children & Institute of Health & Wellbeing, University of Glasgow, Member of European Refence Network EpiCARE, Glasgow, UK. 8. Department of Paediatric Neurology, Red Cross War Memorial Children’s Hospital, Neuroscience Institute, University of Cape Town, South Africa. 9. Isabelle Rapin Division of Child Neurology of the Saul R Korey Department of Neurology, Montefiore Medical Center, Bronx, NY USA. 10. Programme of Developmental Neurosciences, UCL NIHR BRC Great Ormond Street Institute of Child Health, Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children, London, UK 11. -
Analysis of Serial Electroencephalographic Predictors of Seizure Recurrence in Rolandic Epilepsy
Child's Nervous System (2019) 35:1579–1583 https://doi.org/10.1007/s00381-019-04275-0 ORIGINAL ARTICLE Analysis of serial electroencephalographic predictors of seizure recurrence in Rolandic epilepsy Hongwei Tang1 & Yanping Wang1 & Ying Hua1 & Jianbiao Wang1 & Miao Jing1 & Xiaoyue Hu1 Received: 23 February 2019 /Accepted: 25 June 2019 /Published online: 2 July 2019 # Springer-Verlag GmbH Germany, part of Springer Nature 2019 Abstract Purpose We aimed to assess the relationship between electroencephalography (EEG) markers and seizure recurrence in cases with benign epilepsy with centrotemporal spikes (BECT) in a long-term follow-up study. Methods We analyzed the data of 52 children with BECT who were divided into 2 groups: the isolated group and recurrence group. The clinical profiles and initial/serial visual EEG recordings of both groups were evaluated. The entire follow-up period ranged from 12 to 65 months. Results None of the clinical characteristics differed between the 2 groups. Serial EEGs showed that the appearance of Rolandic spikes in the frontal region was more prevalent in the recurrence group. Moreover, a significant correlation was found between bilateral asynchronous discharges and seizure recurrence. However, on initial EEG of these patients, neither of the EEG features exhibited statistical significance. Conclusion The presence of frontal focus and bilateral asynchrony appeared to be hallmarks of BECT patients with higher risk for seizure recurrence. Keywords BECT . Rolandic epilepsy . EEG . Seizure Abbreviations Introduction EEG electroencephalography BECT Benign epilepsy with centrotemporal spikes Benign childhood epilepsy with centrotemporal spikes, also MRI Magnetic resonance imaging known as benign Rolandic epilepsy, accounts for approxi- AEDs Antiepileptic drugs mately 13–25% of cases of epilepsy in school-aged children, LEV Levetiracetam and is one of the most common epileptic syndromes in child- OXC Oxcarbazepine hood [20]. -
(12) Patent Application Publication (10) Pub. No.: US 2010/0210567 A1 Bevec (43) Pub
US 2010O2.10567A1 (19) United States (12) Patent Application Publication (10) Pub. No.: US 2010/0210567 A1 Bevec (43) Pub. Date: Aug. 19, 2010 (54) USE OF ATUFTSINASATHERAPEUTIC Publication Classification AGENT (51) Int. Cl. A638/07 (2006.01) (76) Inventor: Dorian Bevec, Germering (DE) C07K 5/103 (2006.01) A6IP35/00 (2006.01) Correspondence Address: A6IPL/I6 (2006.01) WINSTEAD PC A6IP3L/20 (2006.01) i. 2O1 US (52) U.S. Cl. ........................................... 514/18: 530/330 9 (US) (57) ABSTRACT (21) Appl. No.: 12/677,311 The present invention is directed to the use of the peptide compound Thr-Lys-Pro-Arg-OH as a therapeutic agent for (22) PCT Filed: Sep. 9, 2008 the prophylaxis and/or treatment of cancer, autoimmune dis eases, fibrotic diseases, inflammatory diseases, neurodegen (86). PCT No.: PCT/EP2008/007470 erative diseases, infectious diseases, lung diseases, heart and vascular diseases and metabolic diseases. Moreover the S371 (c)(1), present invention relates to pharmaceutical compositions (2), (4) Date: Mar. 10, 2010 preferably inform of a lyophilisate or liquid buffersolution or artificial mother milk formulation or mother milk substitute (30) Foreign Application Priority Data containing the peptide Thr-Lys-Pro-Arg-OH optionally together with at least one pharmaceutically acceptable car Sep. 11, 2007 (EP) .................................. O7017754.8 rier, cryoprotectant, lyoprotectant, excipient and/or diluent. US 2010/0210567 A1 Aug. 19, 2010 USE OF ATUFTSNASATHERAPEUTIC ment of Hepatitis BVirus infection, diseases caused by Hepa AGENT titis B Virus infection, acute hepatitis, chronic hepatitis, full minant liver failure, liver cirrhosis, cancer associated with Hepatitis B Virus infection. 0001. The present invention is directed to the use of the Cancer, Tumors, Proliferative Diseases, Malignancies and peptide compound Thr-Lys-Pro-Arg-OH (Tuftsin) as a thera their Metastases peutic agent for the prophylaxis and/or treatment of cancer, 0008. -
Pediatric Disorders
Neurological Disorder Part 3 - Pediatric Disorders CDKL5 Disorder • Characteristics: • Rare x-linked genetic disorder • CDLK5 mutations cause deficiencies in the protein needed for normal brain development • More common in females; however males with the disorder are affected much more severely than females © Trusted Neurodiagnostics Academy CDKL5 Disorder • Characteristics • CDKL5D mutations can be found in children who have been diagnosed with infantile spasms, Lennox Gastaut syndrome, Rett Syndrome, West Syndrome and autism © Trusted Neurodiagnostics Academy CDKL5 Disorder • Symptoms: • Infantile spasms beginning the first 3 - 6 months of life • Neurodevelopmental impairment • Patients cannot walk, talk, or feed themselves • Repetitive hand movements (stereotypies) © Trusted Neurodiagnostics Academy CDKL5 Disorder • Seizures: • Early onset • Infantile spasms, myoclonic, tonic, tonic-clonic seizures • Status epilepticus and non convulsive status epilepticus can occur © Trusted Neurodiagnostics Academy CDKL5 Disorder • Diagnosis: • Genetic blood testing to confirm the change or mutation on the CDKL5 gene • EEG © Trusted Neurodiagnostics Academy CDKL5 Disorder • EEG Findings: • Early in the disorder • EEG may be normal or slightly abnormal • During progression of the disorder • Some background activity is slow and epileptic discharges can be seen in one or more areas • Burst Suppression • Atypical hypsarrhythmia © Trusted Neurodiagnostics Academy CDKL5 Disorder © Trusted Neurodiagnostics Academy CDKL5 Disorder • Treatment: • Seizures