Types of Seizures a Person Might Experience When They’Re Ictal Elsewhere in This Pamphlet
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Status Epilepticus Clinical Pathway
JOHNS HOPKINS ALL CHILDREN’S HOSPITAL Status Epilepticus Clinical Pathway 1 Johns Hopkins All Children's Hospital Status Epilepticus Clinical Pathway Table of Contents 1. Rationale 2. Background 3. Diagnosis 4. Labs 5. Radiologic Studies 6. General Management 7. Status Epilepticus Pathway 8. Pharmacologic Management 9. Therapeutic Drug Monitoring 10. Inpatient Status Admission Criteria a. Admission Pathway 11. Outcome Measures 12. References Last updated: July 7, 2019 Owners: Danielle Hirsch, MD, Emergency Medicine; Jennifer Avallone, DO, Neurology This pathway is intended as a guide for physicians, physician assistants, nurse practitioners and other healthcare providers. It should be adapted to the care of specific patient based on the patient’s individualized circumstances and the practitioner’s professional judgment. 2 Johns Hopkins All Children's Hospital Status Epilepticus Clinical Pathway Rationale This clinical pathway was developed by a consensus group of JHACH neurologists/epileptologists, emergency physicians, advanced practice providers, hospitalists, intensivists, nurses, and pharmacists to standardize the management of children treated for status epilepticus. The following clinical issues are addressed: ● When to evaluate for status epilepticus ● When to consider admission for further evaluation and treatment of status epilepticus ● When to consult Neurology, Hospitalists, or Critical Care Team for further management of status epilepticus ● When to obtain further neuroimaging for status epilepticus ● What ongoing therapy patients should receive for status epilepticus Background: Status epilepticus (SE) is the most common neurological emergency in children1 and has the potential to cause substantial morbidity and mortality. Incidence among children ranges from 17 to 23 per 100,000 annually.2 Prevalence is highest in pediatric patients from zero to four years of age.3 Ng3 acknowledges the most current definition of SE as a continuous seizure lasting more than five minutes or two or more distinct seizures without regaining awareness in between. -
Non-Epileptic Seizures a Short Guide for Patients and Families
Non-epileptic seizures a short guide for patients and families Department of Neurology Information for patients Royal Hallamshire Hospital What are non-epileptic seizures? In a seizure people lose control of their body, often causing shaking or other movements of arms and legs, blacking out, or both. Seizures can happen for different reasons. During epileptic seizures, the brain produces electrical impulses, which stop it from working normally. Non-epileptic seizures look a little like epileptic seizures, but are not caused by abnormal electrical activity in the brain. Non-epileptic seizures happen because of problems with handling thoughts, memories, emotions or sensations in the brain. Such problems are sometimes related to stress. However, they can also occur in people who seem calm and relaxed. Often people do not understand why they have developed non-epileptic seizures. Are non-epileptic seizures rare? For every 100,000 people, between 15 and 30 have non- epileptic seizures. Nearly half of all people brought in to hospital with suspected serious epilepsy turn out to have non- epileptic seizures instead. One of the reasons why you may not have heard of non- epileptic seizures is that there are several other names for the same problem. Non-epileptic seizures are also known as pseudoseizures, psychogenic, dissociative or functional seizures. Sometimes people who have non-epileptic seizures are told that they suffer from non-epileptic attack disorder (NEAD). How can I be sure that this is the right diagnosis? Non-epileptic seizures often look like epileptic seizures to friends, family members and even doctors. Like epilepsy, non- epileptic seizures can cause injuries and loss of control over bladder function. -
Epilepsy Foundation Awards $300K in Grants for New Treatments
Epilepsy Foundation Awards $300K in Grants for New Treatments dravetsyndromenews.com/2019/02/05/epilepsy-foundation-awards-300k-in-grants-for-new- treatments/ Mary Chapman February 5, 2019 With the goal of advancing development of new treatments for patients living with poorly controlled seizures, the Epilepsy Foundation has awarded $300,000 in grants to two leading researchers. The grants will go to Matthew Gentry, PhD, a professor at the University of Kentucky, and Greg Worrell, MD, PhD, professor of neurology and chair of clinical neurophysiology at the Mayo Clinic, through the foundation’s New Therapy Commercialization Grants Program and Epilepsy Innovation Seal of Excellence Award. Each recipient will receive matching funding from commercial partners. Gentry was awarded $150,000 to support pre-clinical testing of a compound (VAL-1221) that has promise to treat Lafora disease, a progressive epilepsy caused by genetic abnormalities in the brain’s ability to process a sugar molecule called glycogen. Gentry has joined with Valerion Therapeutics to develop VAL-1221, now in clinical trials for Pompe disease, a rare genetic disorder characterized by the abnormal buildup of glycogen inside cells. Early evidence suggests the compound can break down aberrant glycogen in cells of Lafora patients. 1/3 Worrell will receive $150,000 to support research Cadence Neuroscience, an early-stage company developing medical device therapies for epilepsy treatment and management. The company’s core technology is management of uncontrolled epilepsy when a patient is undergoing Phase 2 evaluation for surgery. Early evidence suggests that this procedure, which tests a variety of electrical stimulation parameters on intractable (hard-to-manage) epilepsy patients during evaluation, can be used to customize brain therapy and enhance seizure control. -
The Art of Epilepsy Management
FOCAL EPILEPSY AND SEIZURE SEMIOLOGY Piradee Suwanpakdee, MD. Division of Neurology Department of Pediatrics Phramongkutklao Hospital OUTLINE • Focal onset seizure classification • The definition of semiology • How can we get the elements of semiology? Focal seizures • Originate within networks limited to one hemisphere • May be discretely localized or more widely distributed.… www.ilae.org What is the seizure semiology? • Seizure semiology is an expression of activation and disinhibition of cerebral areas • It thus provides some information what cerebral areas are “involved” during a seizure Symptomatogenic areas Left hemisphere lateral aspect Mesial aspect Symptomatogenic areas Left Insula How can we get the elements of seizure semiology? • The information on semiology comes from patient’s and witness’ history • Video EEG provides objective data on seizure semiology • Seizure classification aims to intellectually organise and summarise information about seizure semiology Notes • Atonic seizures and epileptic spasms would not have level of awareness specified • Pedalling grouped in hyperkinetic rather than automatisms (arbitrary) • Cognitive seizures • impaired language • other cognitive domains • positive features eg déjà vu, hallucinations, perceptual distortions • Emotional seizures: anxiety, fear, joy, etc Focal onset aware seizure • This term replaces simple partial seizure • A seizure that starts in one area of the brain and the person remains alert and able to interact is called a focal onset aware seizure. • These seizures are brief, lasting seconds to less than 2 minutes. Focal clonic seizure • Indicate involvement of contralateral primary motor cortex • Reliability is good Epilepsia partialis continua focal motor status involving a small portion of the sensorimotor cortex Focal Onset Impaired Awareness Seizures • A seizure that starts in one area of the brain and the person is not aware of their surroundings • Focal impaired awareness seizures typically last 1 to 2 minutes. -
REN Newsletter Nov 2019
REN November 2019 RARE EPILEPSY NETWORK (REN) NEWSLETTER November 2019 Issue Aaron’s Ohtahara International Rett Foundation Syndrome Foundation Aicardi Syndrome Foundation The Jack Pribaz Foundation Alternating Hemiplegia of Childhood KCNQ2 Cure Foundation Alliance Aspire for a Cure Lennox-Gastaut Syndrome Bridge the Gap Foundation SYNGAP Liv4TheCure The Brain Recovery Project NORSE Institute Carson Harris PCDH19 Alliance Foundation Inside this Issue: Phelan-McDermid CFC International Syndrome Foundation Chelsea’s Hope Pitt-Hopkins I. Upcoming events & News………..….….. p 2-6 The Cute Syndrome Research Foundation Foundation II. Active clinical trials and studies.….……. p 7 CSWS & ESES RASopathies Foundation Network III. About REN / Contact us ..….…………..… p 8 Doose Syndrome Ring 14 USA Epilepsy Alliance Outreach Dravet Syndrome Ring 20 Foundation Chromosome Alliance Dup15q Alliance SLC6A1 Connect Hope for Hypothalamic Hamartomas TESS Foundation Infantile Spasms Tuberous Sclerosis Community Alliance International Wishes for Elliott Foundation for CDKL5 Research !1 REN November 2019 SAVE THE DATES - KIm Rice The 4th International Lafora Workshop was held in San Diego September 6 – 8, 2018, attended by nearly 100 scientists and clinicians from eight countries as well as 25 family members of Lafora patients. Lafora research has progressed rapidly since the first Lafora Workshop in June, 2014, organized and funded by Chelsea’s Hope Lafora Research Fund. As a result of bringing together a handful of researchers working on REN workshop at the American this extremely rare orphan disease, the Lafora Epilepsy Epilepsy Society Meeting (AES) Cure Initiative (LECI) was formed and researchers are Sunday, December 8, 2019 now working collaboratively under a $9 million NIH grant. 12-2pm There are now two drug development companies (Ionis Hilton Baltimore, Baltimore, MD Pharmaceuticals and Valerion Therapeutics) More details to come soon. -
Myths and Truths About Pediatric Psychogenic Nonepileptic Seizures
Clin Exp Pediatr Vol. 64, No. 6, 251–259, 2021 Review article CEP https://doi.org/10.3345/cep.2020.00892 Myths and truths about pediatric psychogenic nonepileptic seizures Jung Sook Yeom, MD, PhD1,2,3, Heather Bernard, LCSW4, Sookyong Koh, MD, PhD3,4 1Department of Pediatrics, Gyeongsang National University Hospital, 2Gyeongsang Institute of Health Science, Gyeongsang National University College of Medicine, Jinju, Korea; 3Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA; 4Department of Pediatrics, Children's Healthcare of Atlanta, Atlanta, GA, USA Psychogenic nonepileptic seizures (PNES) is a neuropsychiatric • PNES are a manifestation of psychological and emotional condition that causes a transient alteration of consciousness and distress. loss of self-control. PNES, which occur in vulnerable individuals • Treatment for PNES does not begin with the psychological who often have experienced trauma and are precipitated intervention but starts with the diagnosis and how the dia- gnosis is delivered. by overwhelming circumstances, are a body’s expression of • A multifactorial biopsychosocial process and a neurobiological a distressed mind, a cry for help. PNES are misunderstood, review are both essential components when treating PNES mistreated, under-recognized, and underdiagnosed. The mind- body dichotomy, an artificial divide between physical and mental health and brain disorders into neurology and psychi- atry, contributes to undue delays in the diagnosis and treat ment Introduction of PNES. One of the major barriers in the effective dia gnosis and treatment of PNES is the dissonance caused by different illness Psychogenic nonepileptic seizures (PNES) are paroxysmal perceptions between patients and providers. While patients attacks that may resemble epileptic seizures but are not caused are bewildered by their experiences of disabling attacks beyond by abnormal brain electrical discharges. -
Clinicians Using the Classification Will Identify a Seizure As Focal Or Generalized Onset If There Is About an 80% Confidence Level About the Type of Onset
GENERALIZED ONSET SEIZURES Generalized onset seizures are not characterized by level of awareness, because awareness is almost always impaired. Generalized tonic-clonic: Immediate loss of Generalized epileptic spasms: Brief seizures with awareness, with stiffening of all limbs (tonic phase), flexion at the trunk and flexion or extension of the followed by sustained rhythmic jerking of limbs and limbs. Video-EEG recording may be required to face (clonic phase). Duration is typically 1 to 3 minutes. determine focal versus generalized onset. The seizure may produce a cry at the start, falling, tongue biting, and incontinence. Generalized typical absence: Sudden onset when activity stops with a brief pause and staring, Generalized clonic: Rhythmical sustained jerking of sometimes with eye fluttering and head nodding or limbs and/or head with no tonic stiffening phase. other automatic behaviors. If it lasts for more than These seizures most often occur in young children. several seconds, awareness and memory are impaired. Recovery is immediate. The EEG during these seizures Generalized tonic: Stiffening of all limbs, without always shows generalized spike-waves. clonic jerking. Generalized atypical absence: Like typical absence Generalized myoclonic: Irregular, unsustained jerking seizures, but may have slower onset and recovery and of limbs, face, eyes, or eyelids. The jerking of more pronounced changes in tone. Atypical absence generalized myoclonus may not always be left-right seizures can be difficult to distinguish from focal synchronous, but it occurs on both sides. impaired awareness seizures, but absence seizures usually recover more quickly and the EEG patterns are Generalized myoclonic-tonic-clonic: This seizure is like different. -
Model Section 504 Plan for a Student with Epilepsy
8301 Professional Place, Landover, MD 20785 MODEL SECTION 504 PLAN FOR A STUDENT WITH EPILEPSY [NOTE: This Model Section 504 Plan lists a broad range of services and accommodations that might be needed by a student with epilepsy in the school setting and on school-related trips. The plan must be individualized to meet the specific needs of the particular child for whom the plan is being developed and should include only those items that are relevant to the child. Some students may need additional services and accommodations that have not been included in this Model Plan, and those services and accommodations should be included by those who develop the plan. The plan should be a comprehensive and complete document that includes all of the services and accommodations needed by the student.] Section 504 Plan for _____________________________ (Name of Student) Student I.D. Number__________________ School___________________________________ School Year_______________ _________________ ________________ Epilepsy____ Birth Date Grade Disability Homeroom Teacher_____________________ Bus Number________ OBJECTIVES/GOALS OF THIS PLAN: Epilepsy, also referred to as a seizure disorder, is generally defined by a tendency for recurrent seizures, unprovoked by any known cause such as hypoglycemia. A seizure is an event in the brain which is characterized by excessive electrical discharges. Seizures may cause a myriad of clinical changes. A few of the possibilities may include unusual mental disturbances such as hallucinations, abnormal movements, such as rhythmic jerking of limbs or the body, or loss of consciousness. In addition to abnormalities during the seizure itself, individuals may have abnormal mental experiences immediately before or after the seizure, or even in between seizures. -
Seizure Disorders
Seizure Disorders Author Anthony Murro, M.D. Medical College of Georgia Epilepsy Program Department of Neurology Medical College of Georgia Augusta, GA 30912 Contents · Seizure Definitions · Types of Seizures · Partial Seizures · Generalized Seizures · Automatisms · Post-ictal state · Evolution of seizure type · Provoked Seizures · Causes of provoked seizures · Causes of unprovoked seizures · Electroencephalogram · Neuroimaging · Epilepsy syndromes o Temporal lobe epilepsy: o Frontal lobe epilepsy: o Occipital lobe epilepsy: o West Syndrome o Lennox Gastaut Syndrome o Benign partial epilepsy syndromes o Childhood absence epilepsy o Juvenile myoclonic epilepsy (JME) o Simple Febrile Seizure o Complex Febrile Seizure o Neonatal seizures · Anticonvulsant therapy o When to start and stop anticonvulsant therapy o Principles of anticonvulsant therapy o Anticonvulsant side effects o Phenytoin o Carbamazepine o Phenobarbital o Valproate o Lamotrigine o ACTH/Corticosteroid o Oxcarbazepine o Zonisamide · Status Epilepticus · Drug Therapy for status epilepticus · Epilepsy Surgery · Counseling and patient education · Self assessment examination · Examination answers · References · Disclaimer · Permitted Use Statement Seizure Definitions · An epileptic seizure is a disorder of abnormal synchronous electrical brain activity. · A clinical seizure is a epileptic seizure with symptoms. · A subclinical seizure is a epileptic seizure without symptoms. · A non-epileptic seizure (pseudoseizure) is a disorder with symptoms similar to a epileptic seizure. However, a non-epileptic seizure is not caused by abnormal synchronous electrical brain activity. · A cryptogenic seizure is a seizure that occurs from an unknown cause. Older publications use idiopathic to describe a seizure of unknown etiology but the current ILAE guidelines discourage the use of idiopathic for describing a seizure of unknown etiology. · A symptomatic seizure is a seizure that occurs from a known or suspected brain insult known to increase the risk of developing epilepsy. -
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 -
Infantile Spasms 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 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z Infantile Spasms Sign Up for Our Quarterly Newsletter ARTICLE FAMILY STORIES RESOURCES Get “Families First” plus updates on grants, family resources and more. Sign Up Now DON SHIELDS, MD Dr. Shields is Professor Emeritus of Neurology and Pediatrics, David Geffen Find Us On School of Medicine at UCLA Positions held in National and International and other medical organizations President, Child Neurology Foundation Recent Tweets Vice President, Pediatric Epilepsy Foundation Councilor, Child Neurology Society, 1994-1996 2/25/16 - Great read! @disabilityscoop In Bid To Nominating Committee, American Epilepsy Society, 1996 Understand Autism, Scientists Turn Membership Committee, Professors of Child Neurology, 1995 To Monkeys https://t.co/5t9uz6NhWu Chairman, Membership committee, Child Neurology Society, 1988-1993 https://t.co/5t9uz6NhWu Membership committee, Child Neurology Society, 1986-1988 Co-chairman Awards Committee, American Epilepsy Society, 1989-1992 2/25/16 - Informative read! @mnt Epilepsy and marijuana: could Awards for scientific accomplishment cannabidiol reduce seizures? https://t.co/E3TlMqTQpy UCLA School of Medicine Sherman Mellinkoff Faculty Award for dedication https://t.co/E3TlMqTQpy to the art of medicine and to the finest in doctor-patient -
Myoclonic Atonic Epilepsy Another Generalized Epilepsy Syndrome That Is “Not So” Generalized
EDITORIAL Myoclonic atonic epilepsy Another generalized epilepsy syndrome that is “not so” generalized John M. Zempel, MD, Myoclonic atonic/astatic epilepsy (MAE), first described have shown predominant thalamic activation PhD well by Doose1 (pronounced dough sah: http://www. and default mode network deactivation.6–8 Even Tadaaki Mano, MD, PhD youtube.com/watch?v5hNNiWXV2wF0), is a general- Lennox-Gastaut syndrome, a devastating epileptic ized electroclinical syndrome with early onset charac- encephalopathy with EEG findings of runs of slow terized by myoclonic, atonic/astatic, generalized spike and wave and paroxysmal higher frequency Correspondence to tonic-clonic, and absence seizures (but not tonic activity, has fMRI correlates that are more focal than Dr. Zempel: [email protected] seizures) in association with generalized spike-wave expected in a syndrome with widespread EEG (GSW) discharges. Thought to have a genetic com- abnormalities.9,10 Neurology® 2014;82:1486–1487 ponent that has proven to be complicated,2 MAE EEG-fMRI is maturing as a research and clinical sometimes occurs in children who have otherwise technique. Recording scalp EEG in an electrically been developing normally and has variable outcome. hostile environment is not an easy task. Substantial MAE is typically treated with antiseizure medications technical artifacts, such as changing imaging gradients that are used for generalized epilepsy syndromes, with and ballistocardiogram (ECG-linked artifact observed perhaps a best response to valproate, felbamate, or the in the scalp electrodes), contaminate the EEG signal. ketogenic diet.3,4 However, the relatively distinctive EEG discharges in In this issue of Neurology®, Moeller et al.5 report patients with epilepsy have partially circumvented on the fMRI correlates of GSW discharges as mea- this problem.