Understanding Seizures and Epilepsy
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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. -
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. -
What to Expect After Having a Subarachnoid Hemorrhage (SAH) Information for Patients and Families Table of Contents
What to expect after having a subarachnoid hemorrhage (SAH) Information for patients and families Table of contents What is a subarachnoid hemorrhage (SAH)? .......................................... 3 What are the signs that I may have had an SAH? .................................. 4 How did I get this aneurysm? ..................................................................... 4 Why do aneurysms need to be treated?.................................................... 4 What is an angiogram? .................................................................................. 5 How are aneurysms repaired? ..................................................................... 6 What are common complications after having an SAH? ..................... 8 What is vasospasm? ...................................................................................... 8 What is hydrocephalus? ............................................................................... 10 What is hyponatremia? ................................................................................ 12 What happens as I begin to get better? .................................................... 13 What can I expect after I leave the hospital? .......................................... 13 How will the SAH change my health? ........................................................ 14 Will the SAH cause any long-term effects? ............................................. 14 How will my emotions be affected? .......................................................... 15 When should -
Prognostic Utility of Hypsarrhythmia Scoring in Children with West
Clinical Neurology and Neurosurgery 184 (2019) 105402 Contents lists available at ScienceDirect Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro Prognostic utility of hypsarrhythmia scoring in children with West syndrome T after ketogenic diet ⁎ Yunjian Zhang1, Lifei Yu1, Yuanfeng Zhou, Linmei Zhang, Yi Wang, Shuizhen Zhou Department of Pediatric Neurology, Children’s Hospital of Fudan University, China ARTICLE INFO ABSTRACT Keywords: Objective: The aim of this study was to evaluate the clinical efficacy and electroencephalographic (EEG) changes West syndrome of West syndrome after ketogenic diet (KD) therapy and to explore the correlation of EEG features and clinical Ketogenic diet efficacy. EEG Patients and methods: We retrospectively studied 39 patients with West syndrome who accepted KD therapy from Hypsarrhythmia May 2011 to October 2017. Outcomes including clinical efficacy and EEG features with hypsarrhythmia severity scores were analyzed. Results: After 3 months of treatment, 20 patients (51.3%) had ≥50% seizure reduction, including 4 patients (10.3%) who became seizure-free. After 6 months of treatment, 4 patients remained seizure free, 12 (30.8%) had 90–99% seizure reduction, 8 (20.5%) had a reduction of 50–89%, and 15 (38.5%) had < 50% reduction. Hypsarrhythmia scores were significantly decreased at 3 months of KD. They were associated with seizure outcomes at 6 months independent of gender, the course of disease and etiologies. Patients with a hypsar- rhythmia score ≥8 at 3 months of therapy may not be benefited from KD. Conclusion: Our findings suggest a potential benefit of KD for patients with drug-resistant West syndrome. Early change of EEG after KD may be a predictor of a patient’s response to the therapy. -
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. -
What%Is%Epilepsy?%
What%is%Epilepsy?% Epilepsy(is(a(brain(disorder(in(which(a(person(has(repeated(seizures((convulsions)(over(time.(Seizures(are( episodes(of(disturbed(brain(activity(that(cause(changes(in(attention(or(behavior.( Causes( Epilepsy(occurs(when(permanent(changes(in(brain(tissue(cause(the(brain(to(be(too(excitable(or(jumpy.( The(brain(sends(out(abnormal(signals.(This(results(in(repeated,(unpredictable(seizures.((A(single(seizure( that(does(not(happen(again(is(not(epilepsy.)( Epilepsy(may(be(due(to(a(medical(condition(or(injury(that(affects(the(brain,(or(the(cause(may(be( unknown((idiopathic).( Common(causes(of(epilepsy(include:( •Stroke(or(transient(ischemic(attack((TIA)( •Dementia,(such(as(Alzheimer's(disease( •Traumatic(brain(injury( •Infections,(including(brain(abscess,(meningitis,(encephalitis,(and(AIDS( •Brain(problems(that(are(present(at(birth((congenital(brain(defect)( •Brain(injury(that(occurs(during(or(near(birth( •Metabolism(disorders(present(at(birth((such(as(phenylketonuria)( •Brain(tumor( •Abnormal(blood(vessels(in(the(brain( •Other(illness(that(damage(or(destroy(brain(tissue( •Use(of(certain(medications,(including(antidepressants,(tramadol,(cocaine,(and(amphetamines( Epilepsy(seizures(usually(begin(between(ages(5(and(20,(but(they(can(happen(at(any(age.(There(may(be(a( family(history(of(seizures(or(epilepsy.( Symptoms( Symptoms(vary(from(person(to(person.(Some(people(may(have(simple(staring(spells,(while(others(have( violent(shaking(and(loss(of(alertness.(The(type(of(seizure(depends(on(the(part(of(the(brain(affected(and( cause(of(epilepsy.( -
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, Eugen Trinka Department of Neurology, Medical University of Innsbruck, Austria Received April 9, 2009; Accepted November 18, 2009 ABSTRACT – Background. Myoclonic status epilepticus (MSE) is rarely found in juvenile myoclonic epilepsy (JME) and its clinical features are not well described. We aimed to analyze MSE incidence, precipitating factors and clini- cal course by studying patients with JME from a large outpatient epilepsy clinic. Methods. We retrospectively screened all patients with JME treated at the Department of Neurology, Medical University of Innsbruck, Austria between 1970 and 2007 for a history of MSE. We analyzed age, sex, age at seizure onset, seizure types, EEG, MRI/CT findings and response to antiepileptic drugs. Results. Seven patients (five women, two men; median age at time of MSE 31 years; range 17-73) with MSE out of a total of 247 patients with JME were identi- fied. The median follow-up time was seven years (range 0-35), the incidence was 3.2/1,000 patient years. Median duration of epilepsy before MSE was 26 years (range 10-58). We identified three subtypes: 1) MSE with myoclonic seizures only in two patients, 2) MSE with generalized tonic clonic seizures in three, and 3) generalized tonic clonic seizures with myoclonic absence status in two patients. All patients responded promptly to benzodiazepines. One patient had repeated episodes of MSE. Precipitating events were identified in all but one patient. Drug withdrawal was identified in four patients, one of whom had additional sleep deprivation and alcohol intake. -
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. -
Brain Injury and Opioid Overdose
Brain Injury and Opioid Overdose: Acquired Brain Injury is damage to the brain 2.8 million brain injury related occurring after birth and is not related to congenital or degenerative disease. This includes anoxia and hospital stays/deaths in 2013 hypoxia, impairment (lack of oxygen), a condition consistent with drug overdose. 70-80% of hospitalized patients are discharged with an opioid Rx Opioid Use Disorder, as defined in DSM 5, is a problematic pattern of opioid use leading to clinically significant impairment, manifested by meaningful risk 63,000+ drug overdose-related factors occurring within a 12-month period. deaths in 2016 Overdose is injury to the body (poisoning) that happens when a drug is taken in excessive amounts “As the number of drug overdoses continues to rise, and can be fatal. Opioid overdose induces respiratory doctors are struggling to cope with the increasing number depression that can lead to anoxic or hypoxic brain of patients facing irreversible brain damage and other long injury. term health issues.” Substance Use and Misuse is: The frontal lobe is • Often a contributing factor to brain injury. History of highly susceptible abuse/misuse is common among individuals who to brain oxygen have sustained a brain injury. loss, and damage • Likely to increase for individuals who have misused leads to potential substances prior to and post-injury. loss of executive Acute or chronic pain is a common result after brain function. injury due to: • Headaches, back or neck pain and other musculo- Sources: Stojanovic et al 2016; Melton, C. Nov. 15,2017; Devi E. skeletal conditions commonly reported by veterans Nampiaparampil, M.D., 2008; Seal K.H., Bertenthal D., Barnes D.E., et al 2017; with a history of brain injury. -
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. -
Traumatic Brain Injury and Domestic Violence
TRAUMATIC BRAIN INJURY AND DOMESTIC VIOLENCE Women who are abused often suffer injury to their head, neck, and face. The high potential for women who are abused to have mild to severe Traumatic Brain Injury (TBI) is a growing concern, since the effects can cause irreversible psychological and physical harm. Women who are abused are more likely to have repeated injuries to the head. As injuries accumulate, likelihood of recovery dramatically decreases. In addition, sustaining another head trauma prior to the complete healing of the initial injury may be fatal. Severe, obvious trauma does not have to occur for brain injury to exist. A woman can sustain a blow to the head without any loss of consciousness or apparent reason to seek medical assistance, yet display symptoms of TBI. (NOTE: While loss of consciousness can be significant in helping to determine the extent of the injury, people with minor TBI often do not lose consciousness, yet still have difficulties as a result of their injury). Many women suffer from a TBI unknowingly and misdiagnosis is common since symptoms may not be immediately apparent and may mirror those of mental health diagnoses. In addition, subtle injuries that are not identifiable through MRIs or CT scans may still lead to cognitive symptoms. What is Traumatic Brain Injury? Traumatic brain injury (TBI) is defined as an injury to the brain that is caused by external physical force and is not present at birth or degenerative. TBI can be caused by: • A blow to the head, o e.g., being hit on the head forcefully with object or fist, having one’s head smashed against object/wall, falling and hitting head, gunshot to head.