Eli M. Mizrahi

Glossary of descriptive terminology for ictal semiology: report of the ILAE task force on classification and terminology.

INTRODUCTION PRINCIPLES FOR TERMS AND DEFINITIONS DATA SOURCES I GENERAL TERMS 1.0 SEMIOLOGY 2.0 EPILEPTIC SEIZURE 3.0 ICTUS 4.0 EPILEPSY 5.0 FOCAL 6.0 GENERALIZED 7.0 CONVULSION II TERMS DESCRIBING EPILEPTIC SEIZURE SEMIOLOGY 1.0 MOTOR 1.1 ELEMENTARY MOTOR 1.1.1 TONIC 1.1.1.1 EPILEPTIC SPASM 1.1.1.2 POSTURAL 1.1.1.2.1 VERSIVE 1.1.1.2.2 DYSTONIC 1.1.2 MYOCLONIC 1.1.2.1 NEGATIVE MYOCLONIC 1.1.2.2 CLONIC 1.1.2.2.1 JACKSONIAN MARCH 1.1.3 TONIC-CLONIC 1.1.3.1 GENERALIZED TONIC-CLONIC SEIZURE 1.1.4 ATONIC 1.1.5 ASTATIC 1.1.6 SYNCHRONOUS 1.2 AUTOMATISM 1.2.1 OROALIMENTARY 1.2.2 MIMETIC 1.2.3 MANUAL OR PEDAL 1.2.4 GESTURAL 1.2.5 HYPERKINETIC 1.2.6 HYPOKINETIC 1.2.7 DYSPHASIC 1.2.8 DYSPRAXIC 1.2.9 GELASTIC 1.2.10 DACRYSTIC 1.2.11 VOCAL 1.2.12 VERBAL 1.2.13 SPONTANEOUS 1.2.14 INTERACTIVE 2.0 NON-MOTOR 2.1 AURA 2.2 SENSORY 2.2.1 ELEMENTARY 2.2.1.1 SOMATOSENSORY 2.2.1.2 VISUAL 2.2.1.3 AUDITORY 2.2.1.4 OLFACTORY 2.2.1.5 GUSTATORY 2.2.1.6 EPIGASTRIC 2.2.1.7 CEPHALIC 2.2.1.8 AUTONOMIC 2.2.2 EXPERIENTIAL 2.2.2.1 AFFECTIVE 2.2.2.2 MNEMONIC 2.2.2.3 HALLUCINATORY 2.2.2.4 ILLUSORY 2.3 DYSCOGNITIVE 3.0 AUTONOMIC EVENTS 3.1 AUTONOMIC AURA 3.2 AUTONOMIC SEIZURE 4.0 SOMATOTOPIC MODIFIERS 4.1 LATERALITY 4.1.1 UNILATERAL 4.1.1.1 HEMI4.1.2 GENERALIZED (syn. “bilateral”) 4.1.2.1 ASYMMETRICAL 4.1.2.2 SYMMETRICAL 4.2 BODY PART 4.3 CENTRICITY 4.3.1 AXIAL Epilepsia, 42(9):1212–1218, 2001 Blackwell Science, Inc.

Characterization and classification of neonatal seizures

To characterize and classify neonatal seizures, we studied 349 neonates, using a portable, cribside EEG/ polygraphic/video monitoring system. We recorded 415 clinical seizures in 71 infants; 11 other infants had electrical seizure activity without clinical accompaniments. Each seizure was analyzed in terms of its clinical character and its relationship to the presence of EEG seizure activity. Focal clonic seizures, some forms of myoclonic seizures, and focal tonic seizures were consistently associated with electrical seizure activity. Most “subtle” seizures, all generalized tonic seizures, and some forms of myoclonic seizures were either not associated with EEG seizure activity or had an inconsistent relationship with such activity. Seizures that were consistently and coherently related to focal EEG seizure activity had a high correlation with focal brain lesions and a favorable short-term outcome. Seizures with no relationship or an inconsistent relationship to EEG seizure activity were correlated with diffuse processes such as hypoxic-ischemic encephalopathy and a poor short-term outcome. The clinical and background EEG features of infants whose seizures were not accompanied by EEG seizure activity suggest that these seizures may not be epileptic in character.

Long-term treatment with responsive brain stimulation in adults with refractory partial seizures.

Objective: The long-term efficacy and safety of responsive direct neurostimulation was assessed in adults with medically refractory partial onset seizures. Methods: All participants were treated with a cranially implanted responsive neurostimulator that delivers stimulation to 1 or 2 seizure foci via chronically implanted electrodes when specific electrocorticographic patterns are detected (RNS System). Participants had completed a 2-year primarily open-label safety study (n = 65) or a 2-year randomized blinded controlled safety and efficacy study (n = 191); 230 participants transitioned into an ongoing 7-year study to assess safety and efficacy. Results: The average participant was 34 (±11.4) years old with epilepsy for 19.6 (±11.4) years. The median preimplant frequency of disabling partial or generalized tonic-clonic seizures was 10.2 seizures a month. The median percent seizure reduction in the randomized blinded controlled trial was 44% at 1 year and 53% at 2 years (p < 0.0001, generalized estimating equation) and ranged from 48% to 66% over postimplant years 3 through 6 in the long-term study. Improvements in quality of life were maintained (p < 0.05). The most common serious device-related adverse events over the mean 5.4 years of follow-up were implant site infection (9.0%) involving soft tissue and neurostimulator explantation (4.7%). Conclusions: The RNS System is the first direct brain responsive neurostimulator. Acute and sustained efficacy and safety were demonstrated in adults with medically refractory partial onset seizures arising from 1 or 2 foci over a mean follow-up of 5.4 years. This experience supports the RNS System as a treatment option for refractory partial seizures. Classification of evidence: This study provides Class IV evidence that for adults with medically refractory partial onset seizures, responsive direct cortical stimulation reduces seizures and improves quality of life over a mean follow-up of 5.4 years.

Determination of Brain Temperatures for Safe Circulatory Arrest during Cardiovascular Operation

Profound hypothermia protects cerebral function during circulatory arrest in the surgical treatment of a variety of cardiac and aortic abnormalities. Despite its importance, techniques to determine the appropriate level of hypothermia vary; studies of temperatures recorded from multiple peripheral body sites show inconsistent findings. The purpose of this study is to establish objective criteria to consistently identify intraoperatively the safe level of hypothermia. Our studies are based on experimental evidence showing a correlation between brain temperature and development of electrocerebral silence (ECS) on the electroencephalogram (EEG), and the recognition that the EEG, as an objective measure of brain function, can easily be recorded intraoperatively. We studied 56 patients who required circulatory arrest during operation for replacement of the ascending aorta or aortic arch (N = 55) or aortic valve replacement (N = 1). Peripheral body temperatures from the nasopharynx, esophagus, and rectum and the EEG were continuously recorded during body cooling. Circulatory arrest time ranged from 14 to 109 minutes. No peripheral body temperature from a single site or from a combination of sites consistently predicted ECS. There was a wide variation in temperature among body sites when ECS occurred: nasopharyngeal, 10.1 degrees to 24.1 degrees C; esophageal, 7.2 degrees to 23.1 degrees C; rectal, 12.8 degrees to 28.6 degrees C. Fifty-one (91%) of the 56 patients survived. Three had neurological deficits, none clearly related to hypothermia. Two patients (3.6%) required reexploration for postoperative bleeding. We conclude that monitoring the EEG to identify ECS is a safe, consistent, and objective method of determining the appropriate level of hypothermia.

Anterior Temporal Lobectomy and Medically Refractory Temporal Lobe Epilepsy of Childhood

Summary: The evaluation and outcome of 22 patients who had onset of complex partial seizures (CPS) of temporal lobe origin in childhood and subsequently underwent anterior temporal lobectomy are described. All patients showed improved seizure control; 81.8% had a reduction ≥95% in seizure frequency. However, many patients had difficulty adjusting to a seizure‐free life. Psychosocial, behavioral, and educational problems occurred more frequently in patients whose surgery was delayed until adult life. We conclude that attempts should be made early in the course of CPS of childhood to determine whether the seizures are truly intractable to medical management so that surgical intervention can be expedited.

Evaluation of an automatic seizure detection method for the newborn EEG.

In another publication, we described a set of methods for automatic detection of EEG seizures in the newborn. We describe here the evaluation of these methods using a completely new set of data, which were not used in developing the method. This testing data set consisted of recording from 54 patients, lasting an average of 4.4 h. Recordings had 8-16 channels and were obtained, in approximately equal numbers, from 3 institutions in Canada, the USA and Australia. Recording conditions varied from short recordings fully attended by a technologist to overnight recordings largely unattended. The average seizure detection rate was 69% (77%, 53%, 84% in the 3 institutions). False detections occurred at the average rate of 2.3/h (4.1, 1.0, 2.7 in the 3 institutions), with fluctuations that reflected largely the technical quality and level of supervision of the recordings. The results are similar to those obtained in the commonly used method of epilepsy monitoring in adults and allow us to envisage clinical application.

Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics

Evidence‐based guidelines, or recommendations, for the management of infants with seizures are lacking. A Task Force of the Commission of Pediatrics developed a consensus document addressing diagnostic markers, management interventions, and outcome measures for infants with seizures. Levels of evidence to support recommendations and statements were assessed using the American Academy of Neurology Guidelines and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. The report contains recommendations for different levels of care, noting which would be regarded as standard care, compared to optimal care, or “state of the art” interventions. The incidence of epilepsy in the infantile period is the highest of all age groups (strong evidence), with epileptic spasms the largest single subgroup and, in the first 2 years of life, febrile seizures are the most commonly occurring seizures. Acute intervention at the time of a febrile seizure does not alter the risk for subsequent epilepsy (class 1 evidence). The use of antipyretic agents does not alter the recurrence rate (class 1 evidence), and there is no evidence to support initiation of regular antiepileptic drugs for simple febrile seizures (class 1 evidence). Infants with abnormal movements whose routine electroencephalography (EEG) study is not diagnostic, would benefit from video‐EEG analysis, or home video to capture events (expert opinion, level U recommendation). Neuroimaging is recommended at all levels of care for infants presenting with epilepsy, with magnetic resonance imaging (MRI) recommended as the standard investigation at tertiary level (level A recommendation). Genetic screening should not be undertaken at primary or secondary level care (expert opinion). Standard care should permit genetic counseling by trained personal at all levels of care (expert opinion). Genetic evaluation for Dravet syndrome, and other infantile‐onset epileptic encephalopathies, should be available in tertiary care (weak evidence, level C recommendation). Patients should be referred from primary or secondary to tertiary level care after failure of one antiepileptic drug (standard care) and optimal care equates to referral of all infants after presentation with a seizure (expert opinion, level U evidence). Infants with recurrent seizures warrant urgent assessment for initiation of antiepileptic drugs (expert opinion, level U recommendation). Infantile encephalopathies should have rapid introduction and increment of antiepileptic drug dosage (expert opinion, level U recommendation). There is no high level evidence to support any particular current agents for use in infants with seizures. For focal seizures, levetiracetam is effective (strong evidence); for generalized seizures, weak evidence supports levetiracetam, valproate, lamotrigine, topiramate, and clobazam; for Dravet syndrome, strong evidence supports that stiripentol is effective (in combination with valproate and clobazam), whereas weak evidence supports that topiramate, zonisamide, valproate, bromide, and the ketogenic diet are possibly effective; and for Ohtahara syndrome, there is weak evidence that most antiepileptic drugs are poorly effective. For epileptic spasms, clinical suspicion remains central to the diagnosis and is supported by EEG, which ideally is prolonged (level C recommendation). Adrenocorticotropic hormone (ACTH) is preferred for short‐term control of epileptic spasms (level B recommendation), oral steroids are probably effective in short‐term control of spasms (level C recommendation), and a shorter interval from the onset of spasms to treatment initiation may improve long‐term neurodevelopmental outcome (level C recommendation). The ketogenic diet is the treatment of choice for epilepsy related to glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency (expert opinion, level U recommendation). The identification of patients as potential candidates for epilepsy surgery should be part of standard practice at primary and secondary level care. Tertiary care facilities with experience in epilepsy surgery should undertake the screening for epilepsy surgical candidates (level U recommendation). There is insufficient evidence to conclude if there is benefit from vagus nerve stimulation (level U recommendation). The key recommendations are summarized into an executive summary. The full report is available as Supporting Information. This report provides a comprehensive foundation of an approach to infants with seizures, while identifying where there are inadequate data to support recommended practice, and where further data collection is needed to address these deficits.

A multistage system for the automated detection of epileptic seizures in neonatal electroencephalography.

This paper describes the design and test results of a three-stage automated system for neonatal EEG seizure detection. Stage I of the system is the initial detection stage and identifies overlapping 5-second segments of suspected seizure activity in each EEG channel. In stage II, the detected segments from stage I are spatiotemporally clustered to produce multichannel candidate seizures. In stage III, the candidate seizures are processed further using measures of quality and context-based rules to eliminate false candidates. False candidates because of artifacts and commonly occurring EEG background patterns such as bifrontal delta activity are also rejected. Seizures at least 10 seconds in duration are considered for reporting results. The testing data consisted of recordings of 28 seizure subjects (34 hours of data) and 48 nonseizure subjects (87 hours of data) obtained in the neonatal intensive care unit. The data were not edited to remove artifacts and were identical in every way to data normally processed visually. The system was able to detect seizures of widely varying morphology with an average detection sensitivity of almost 80% and a subject sensitivity of 96%, in comparison with a team of clinical neurophysiologists who had scored the same recordings. The average false detection rate obtained in nonseizure subjects was 0.74 per hour.

Agenesis of the vermis with fusion of the cerebellar hemispheres, septo-optic dysplasia and associated anomalies

SummaryAgenesis of the cerebellar vermis with fusion of the dentate nuclei and cerebellar hemispheres (rhombencephalosynapsis) is a rare cerebral malformation. We report the case of a 7-h-old girl whose mother had taken the drug phencyclidine during the first 6 weeks of pregnancy. Absence of the septum pellucidum, hypoplasia of the commissural system, optic nerves, chiasm and tracts, moderate hydrocephalus, and agenesis of the posterior lobe of the pituitary were also found. Extracranial congenital anomalies involved the cardiovascular, respiratory, urinary, and musculoskeletal systems. Death was secondary to severe respiratory distress and bradycardia. The literature concerning this rare form of cerebellar malformation is reviewed.

Neonatal seizures : Early-onset seizure syndromes and their consequences for development

The determination of the developmental consequences of seizure syndromes in the neonate is based upon a number of factors which include: understanding of the clinical and electroencephalographic (EEG) features of neonatal seizures; current theories of the mechanisms by which neonatal seizures are generated; a current classification of neonatal seizures; potential etiologic and risk factors for seizures; and therapies. In addition, different seizure types, mechanisms of generation and etiologies of cerebral dysfunction may vary with conceptional age of the infant. There are a few distinct neonatal epileptic syndromes, which are rare, have been well described: benign neonatal convulsions; benign neonatal familial convulsions; early myoclonic encephalopathy and early infantile epileptic encephalopathy. The prognosis for the first two is relatively good while the outcome for the other two with encephalopathy is catastrophic. However, the majority of neonatal seizures occur as acute, reactive events in association with a wide range of etiologic factors. These etiologic factors, as well as those of the more traditionally defined syndromes, are the main determinants of eventual developmental outcome of neonates who experience seizures. Although experimental data suggests that some epileptic seizures eventually may have physiological, histological, metabolic, or behavioral consequences, there is yet direct evidence in humans to suggest that the occurrence of seizures themselves in the neonate is the main determinant of long-term outcome.