Seizures, Epilepsy and Neuropsychiatry
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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. -
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. -
Mesial Frontal Epilepsy
Epikpsia, 39(Suppl. 4):S49-S61. 1998 Lippincon-Raven Publishers, Philadelphia 0 International League Against Epilepsy Mesial Frontal Epilepsy Norman K. So Oregon Comprehensive Epilepsy Program, Legacy Portland Hospitals, Portland, Oregon, U.S.A. Summary: The mesiofrontal cortex comprises a number of occur. The task of localization of the epileptogenic zone can be distinct anatomic and functional areas. Structural lesions and challenging, whether EEG or imaging methods are used. Suc- cortical dysgenesis are recognized causes of mesial frontal epi- cessful localization can lead to a rewarding outcome after epi- lepsy, but a specific gene defect may also be important, as seen lepsy surgery, particularly in those with an imaged lesion. in some forms of familial frontal lobe epilepsy. The predomi- Key Words: Mesial frontal epilepsy-cingulate gyrus- nant seizure manifestations, which are not necessarily strictly Supplementary motor area-Absence seizure-Hypermotor correlated with a specific ictal onset zone, are absence, hyper- seizure-Postural tonic seizure-Epilepsy surgery. motor, and postural tonic seizures. Other seizure types also FUNCTIONAL ANATOMY convolution. Traditional cytoarchitectonics have further subdivided this anterior frontal region. The frontal pole The frontal lobe is the largest lobe in the brain, ac- refers to the anterior most portion of the frontal lobe, but counting for one-third to one-half of total brain volume there is little consensus on how far back this extends. and weight. On the medial surface, the most important One or more curved.sulci are seen anterior and inferior to landmark is the cingulate sulcus (Fig. 1). This runs as an the cingulate sulcus, called the superior and inferior ros- inverted “C” following the contour of the corpus callo- tral sulci. -
The Double Generalization Phenomenon in Juvenile Absence Epilepsy
Epilepsy & Behavior 21 (2011) 318–320 Contents lists available at ScienceDirect Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh Case Report The double generalization phenomenon in juvenile absence epilepsy Daniel San-Juan a,b,⁎, Adriana Patricia M. Mayorga a, David J. Anschel c, Alvaro Moreno Avellán a, Maricarmen F. González-Aragón a, Andrew J. Cole d a Clinical Neurophysiology Department, National Institute of Neurology, Mexico City, Mexico b Centro Neurológico, Hospital ABC Santa Fe, Mexico City, Mexico c Clinical Neurophysiology Department, Saint Charles Hospital, Port Jefferson, NY, USA d Epilepsy Service, Massachusetts General Hospital, Boston, MA, USA article info abstract Article history: The characterization of a seizure as generalized or focal onset depends on a basic knowledge of the underlying Received 12 March 2011 pathophysiology. Recently, an uncommon phenomenon in generalized epilepsy—evolution of seizures Revised 7 April 2011 from generalized to focal followed by secondary generalization—was reported for the first time. We describe a Accepted 8 April 2011 15-year-old boy, initially classified as having partial epilepsy, who had a typical absence seizure that became Available online 14 May 2011 focal with second secondary generalization (double generalization). On the basis of these findings his epilepsy fi Keywords: was classi ed as juvenile absence epilepsy and his treatment was changed, resulting in seizure freedom. This fi Juvenile absence epilepsy is the rst report of this unusual electroclinical evolution in a patient with juvenile absence epilepsy. The Generalized epilepsy recognition of this particular pattern allows correct classification and impacts both treatment and prognosis. Focal findings © 2011 Elsevier Inc. -
“Seizures (Epilepsy)” a Seizure: Partial – Start in a Specific Part of the Brain, Not in the Whole Is a Symptom of an Electrical Disturbance in the Brain Brain
“Seizures (Epilepsy)” A seizure: Partial – start in a specific part of the brain, not in the whole Is a symptom of an electrical disturbance in the brain brain. Unlike generalized seizures, partial seizures can have a Is a rare event warning before they occur (aura). Auras are actually a kind of Has a typical beginning (best clue for accurate diagnosis) seizure. There are several different kinds of partial seizures: Is involuntary simple (motor, sensory or psychological), complex, partial Lasts only a short time (90% complete in 90 seconds) seizure with secondary generalization. May cause post seizure impairments. Most seizures do not involve convulsions. Accurate seizure diagnosis by the health care provider is very The most common type of seizure is one mostly involving important because the medications used to treat seizures often vary loss of awareness. depending on the type. There are 20 types of seizures in the Seizures can be very subtle and hard to notice. International Classification of Seizures (over 2,000 types reported in the literature). Examples of post-seizure impairments: A detailed description of the seizure by the person observing the Post ictal confusion (length is individual) seizure is necessary for accurate diagnosing. Having a seizure while in Initial difficulty speaking the doctor’s office is very rare. Confusion about when, where, or what was just happening Memory disturbance which can last a while (behaving Seizure observation: - 3 important ones to make (in order of usual normally but can’t retain/absorb information) importance): Headache with some kinds of seizures What happened right as the seizure was beginning? What is epilepsy? What happened after the seizure was over? What happened during the seizure? Epilepsy is a condition where a person has “recurrent, unprovoked” seizures. -
Managing Children with Epilepsy School Nurse Guide
MANAGING CHILDREN WITH EPILEPSY SCHOOL NURSE GUIDE ACKNOWLEDGEMENTS TO THOSE WHO HAVE CONTRIBUTED TO THE NOTEBOOK Children’s Hospital of Orange County Melodie Balsbaugh, RN Sue Nagel, RN Giana Nguyen, CHOC Institutes Fullerton School District Jane Bockhacker, RN Orange Unified School District Andrea Bautista, RN Martha Boughen, RN Karen Hanson, RN TABLE OF CONTENTS I. EPILEPSY What is epilepsy? Facts about epilepsy Basic neuroanatomy overview Classification of epileptic seizures Diagnostic Tests II. TREATMENT Medications Vagus Nerve Stimulation Ketogenic Diet Surgery III. SAFETY First Aid IV. SPECIAL CONCERNS MedicAlert Helmets Driving Employment and the law V. EPILEPSY AT SCHOOL School epilepsy assessment tool Seizure record Teaching children about epilepsy lesson plan Creating your own individualized health care plan VI. RESOURCES/SUPPORT GROUPS VII. ACCESS TO HEALTHCARE CHOC Epilepsy Center After-Hours Care After Hours Health Care Advice Healthy Families California Kids MediCal CHOC Clinics Healthy Tomorrows VIII. REFERENCES EPILEPSY WHAT IS EPILEPSY? Epilepsy is a neurological disorder. The brain contains millions of nerve cells called neurons that send electrical charges to each other. A seizure occurs when there is a sudden and brief excess surge of electrical activity in the brain between nerve cells. This results in an alteration in sensation, behavior, and consciousness. Seizures may be caused by developmental problems before birth, trauma at birth, head injury, tumor, structural problems, vascular problems (i.e. stroke, abnormal blood vessels), metabolic conditions (i.e. low blood sugar, low calcium), infections (i.e. meningitis, encephalitis) and idiopathic causes. Children who have idiopathic seizures are most likely to respond to medications and outgrow seizures. -
Operational Classification of Seizure Types by the International League Against Epilepsy: Position Paper of the ILAE Commission
ILAE POSITION PAPER Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology *Robert S. Fisher, †J. Helen Cross, ‡Jacqueline A. French, §Norimichi Higurashi, ¶Edouard Hirsch, #Floor E. Jansen, **Lieven Lagae, ††Solomon L. Moshe, ‡‡Jukka Peltola, §§Eliane Roulet Perez, ¶¶Ingrid E. Scheffer, and ##***Sameer M. Zuberi Epilepsia, 58(4):522–530, 2017 doi: 10.1111/epi.13670 SUMMARY The International League Against Epilepsy (ILAE) presents a revised operational clas- sification of seizure types. The purpose of such a revision is to recognize that some sei- zure types can have either a focal or generalized onset, to allow classification when the onset is unobserved, to include some missing seizure types, and to adopt more trans- parent names. Because current knowledge is insufficient to form a scientifically based classification, the 2017 Classification is operational (practical) and based on the 1981 Classification, extended in 2010. Changes include the following: (1) “partial” becomes “focal”; (2) awareness is used as a classifier of focal seizures; (3) the terms dyscognitive, simple partial, complex partial, psychic, and secondarily generalized are eliminated; Dr. Robert S. Fisher, (4) new focal seizure types include automatisms, behavior arrest, hyperkinetic, auto- past president of nomic, cognitive, and emotional; (5) atonic, clonic, epileptic spasms, myoclonic, and American Epilepsy tonic seizures can be of either focal or generalized onset; (6) focal to bilateral tonic– Society and editor of clonic seizure replaces secondarily generalized seizure; (7) new generalized seizure Epilepsia and types are absence with eyelid myoclonia, myoclonic absence, myoclonic–atonic, epilepsy.com, led the myoclonic–tonic–clonic; and (8) seizures of unknown onset may have features that can Seizure Classification still be classified. -
Understanding Seizures and Epilepsy
Understanding Sei zures & Epilepsy Selim R. Benbadis, MD Leanne Heriaud, RN Comprehensive Epilepsy Program Table of Contents * What is a seizure and what is epilepsy?....................................... 3 * Who is affected by epilepsy? ......................................................... 3 * Types of seizures ............................................................................. 3 * Types of epilepsy ............................................................................. 6 * How is epilepsy diagnosed? .......................................................... 9 * How is epilepsy treated? .............................................................. 10 Drug therapy ......................................................................... 10 How medication is prescribed ............................................ 12 Will treatment work?............................................................ 12 How long will treatment last?............................................. 12 Other treatment options....................................................... 13 * First aid for a person having a seizure ....................................... 13 * Safety and epilepsy ....................................................................... 14 * Epilepsy and driving..................................................................... 15 * Epilepsy and pregnancy ............................................................... 15 * More Information .......................................................................... 16 Comprehensive -
Occipital and Parietal Lobe Epilepsies
Chapter 15 Occipital and parietal lobe epilepsies JOHN S. DUNCAN UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London Epileptic seizures of parietal and occipital origin are heterogeneous and mainly characterised by the presenting auras, although the most dramatic clinical manifestations may reflect spread, and overshadow the focal origin. The two lobes serve mainly sensory functions, and the characteristic seizure phenomena are therefore subjective sensations. The incidence of these seizures is not well known, but they are generally considered rare. Occipital seizures have been reported to constitute 8% and parietal seizures 1.4% of total seizures in the prevalent population with epilepsy1,2. The pattern of seizures is most commonly focal seizures without impairment of awareness, with occasional secondary generalisation. Focal seizures with impairment of awareness are rare and usually indicate spread of the seizure into the temporal lobe. Seizures with somatosensory symptomatology1-3 Somatosensory seizures may arise from any of the three sensory areas of the parietal lobe, but the post-central gyrus is most commonly involved. Seizures present with contralateral, or rarely ipsilateral, or bilateral sensations. All sensory modalities may be represented, most commonly tingling and numbness, alone or together. There may be prickling, tickling or crawling sensations, or a feeling of electric shock in the affected body part. The arms and the face are the most common sites, but any segment or region may be affected. The paraesthesia may spread in a Jacksonian manner, and when this occurs motor activity in the affected body member follows the sensations in about 50% of cases. -
Infantile Spasms: an Update on Pre-Clinical Models and EEG Mechanisms
children Review Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms Remi Janicot, Li-Rong Shao and Carl E. Stafstrom * Division of Pediatric Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA; [email protected] (R.J.); [email protected] (L.-R.S.) * Correspondence: [email protected]; Tel.: +1-(410)-955-4259; Fax: +1-(410)-614-2297 Received: 19 November 2019; Accepted: 23 December 2019; Published: 6 January 2020 Abstract: Infantile spasms (IS) is an epileptic encephalopathy with unique clinical and electrographic features, which affects children in the middle of the first year of life. The pathophysiology of IS remains incompletely understood, despite the heterogeneity of IS etiologies, more than 200 of which are known. In particular, the neurobiological basis of why multiple etiologies converge to a relatively similar clinical presentation has defied explanation. Treatment options for this form of epilepsy, which has been described as “catastrophic” because of the poor cognitive, developmental, and epileptic prognosis, are limited and not fully effective. Until the pathophysiology of IS is better clarified, novel treatments will not be forthcoming, and preclinical (animal) models are essential for advancing this knowledge. Here, we review preclinical IS models, update information regarding already existing models, describe some novel models, and discuss exciting new data that promises to advance understanding of the cellular mechanisms underlying the specific EEG changes seen in IS—interictal hypsarrhythmia and ictal electrodecrement. Keywords: infantile spasms; West syndrome; epilepsy; childhood; epileptic encephalopathy; electroencephalogram (EEG); hypsarrhythmia; electrodecrement; animal model 1. Introduction Epileptic encephalopathies (EEs) are a spectrum of disorders that mostly begin during infancy and have poor neurological and behavioral outcomes. -
Typical Absence Seizures and Their Treatment
Arch Dis Child 1999;81:351–355 351 Arch Dis Child: first published as 10.1136/adc.81.4.351 on 1 October 1999. Downloaded from CURRENT TOPIC Typical absence seizures and their treatment C P Panayiotopoulos Typical absences (previously known as petit there are inappropriate generalisations regard- mal) are generalised seizures that are distinc- ing their use in the treatment of other tively diVerent from any other type of epileptic epilepsies. fit.1 They are pharmacologically unique2–5 and demand special attention in their treatment.6 The prevalence of typical absences among Typical absence seizures children with epilepsies is about 10%, probably Typical absence seizures are defined according with a female preponderance.6 Typical ab- to clinical and electroencephalogram (EEG) 16 sences are easy to diagnose and treat. There- ictal and interictal expression. Clinically, the fore, it is alarming that 40% of children with hallmark of the absence is abrupt and brief typical absences were inappropriately treated impairment of consciousness, with interrup- with contraindicated drugs, such as car- tion of the ongoing activity, and usually unresponsiveness. The seizure lasts for a few to Department of Clinical bamazepine and vigabatrin, according to a 7 20 seconds and ends suddenly with resumption Neurophysiology and recent report from London, UK. Epilepsies, St Thomas’ The purpose of this paper is to oVer some of the pre-absence activity, as if had not been Hospital, London guidance to paediatricians regarding diagnosis interrupted. Although some absence seizures SE1 7EH, UK and management of typical absence seizures. can manifest with impairment of consciousness C P Panayiotopoulos This is also important because of the introduc- only, this is often combined with the following: tion of new antiepileptic drugs. -
A Novel Deletion Mutation in EPM2A Underlies Progressive Myoclonic Epilepsy (Lafora Body Disease) in a Pakistani Family
Neurology Asia 2021; 26(2) : 427 – 433 A novel deletion mutation in EPM2A underlies progressive myoclonic epilepsy (Lafora body disease) in a Pakistani family 1Fizza Orooj MRCP, 2Umm-e-Kalsoom PhD, 3XiaoChu Zhao, 1Arsalan Ahmad MD, 4Imran Nazir Ahmed MD, 5Muhammad Faheem PhD, 5Muhammad Jawad Hassan PhD, 3,6Berge A. Minasian MD 1Division of Neurology, Shifa International Hospital, Shifa Tameer-e-Millat University, Islamabad, Pakistan; 2Department of Biochemistry, Hazara University, Mansehra, KPK, Pakistan; 3Program in Genetics and Genome Biology, The Hospital for Sick Children, Toronto, Canada; 4Department of Pathology, Shifa International Hospital, Shifa Tameer-e-Millat University, Islamabad, Pakistan; 5Department of Biological Sciences, National University of Medical Sciences, Rawalpindi, Pakistan; 6Department of Pediatrics, University of Texas Southwestern, Dallas, Teas, USA Abstract Lafora body disease (MIM-254780), a glycogen storage disease, characterized by Lafora bodies (deformed glycogen molecules) accumulating in multiple organs, is a rare form of myoclonic epilepsy. It manifests in early adolescent years, initially with seizures and myoclonus, followed by dementia and progressive cognitive decline, ultimately culminating in death within 10 years. In Pakistan so far 5 cases have been reported. Here, we report a new case of Lafora body disease belonging to a consanguineous family from Pakistan. Histopathological analysis confirmed presence of lafora bodies in the patient`s skin. Sanger sequencing revealed novel homozygous 5bp deletion mutation (NM_005670.4; c.359_363delGTGTG) in exon 2 of the EPM2A gene, which was truly segregated in the family. These results will increase our understanding regarding the aetiology of this disorder and will further add to the mutation spectrum of EPM2A gene.