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1.  Patient assessment of physician performance of epilepsy quality-of-care measures 
Neurology. Clinical Practice  2012;2(4):335-342.
To identify gaps in physician practice of epilepsy care, an online survey was sent to members of a Web-based epilepsy community to ascertain whether their physician performed 8 quality measures for epilepsy care. A total of 221 of 348 recently active epilepsy patients (64%) completed the survey. More than 80% of patients agreed they knew their seizure type, epilepsy syndrome, current seizure frequency, and had an EEG and neuroimaging. Fewer (60%) recalled being asked about medication side effects at each visit and safety issues annually. Only 48% report referral to an epilepsy surgery specialist and only 46% of appropriate patients had discussed reproductive issues with epilepsy. This demonstrates some potential gaps in epilepsy care and these data have been submitted to the American Academy of Neurology and the National Quality Forum.
PMCID: PMC3613213  PMID: 23634376
Epilepsy & behavior : E&B  2008;13(4):607-613.
Epilepsy is associated with sleep disturbance, but little is known about how early this relationship develops and how it affects neuropsychological functioning. This study documented the frequency and types of sleep problems and examined how sleep problems are associated with seizures and neuropsychological functioning in 331 children following their first recognized seizure (ages 6 to 14) and in 225 sibling controls. Formal neuropsychological batteries were administered to all subjects. Sleep was measured using the Sleep Behavior Questionnaire and the Child Behavior Checklist. Sleep problems were more frequent in the seizure sample relative to siblings and previously published norms; bedtime difficulties, daytime somnolence and parasomnias were the most frequently occurring sleep problems. In the seizure group, sleep problems were related to seizure parameters and to neuropsychological functioning. Seizure patients with significant sleep problems had worse neuropsychological functioning on all measures. Findings demonstrate the significant impact of sleep disturbance on children with newly recognized seizures.
PMCID: PMC2647721  PMID: 18687412
Sleep; Sleep Disturbance; Epilepsy; Seizures; First recognized seizures; Children; Pediatric; Cognition; Neuropsychology; Prevalence
3.  A neuropsychological assessment, using computerized battery tests (CANTAB), in children with benign rolandic epilepsy before AED therapy  
Journal of Medicine and Life  2012;5(1):114-119.
Rationale: Benign rolandic epilepsy (BRE) is a form of partial idiopathic epilepsy according to the International League Against Epilepsy (ILAE) syndromes classification (1989). Recent studies have identified cases of BRE that do not meet the initial definition of ‘benign’; these included reports of cases with specific cognitive deficits. It is still a matter of debate, whether these deficits are due to epilepsy per se, to treatment or other associated factors.
Objectives: The aim of this study was to evaluate if BRE children have cognitive deficits at the onset of their seizures, prior to their participation in any anti-epileptic drug therapy (AED).
Methods and Results: We performed a neuropsychological assessment of 18 BRE children compared with a corresponding age-matched control group. We used the Cambridge Neuropsychological Test Automated Battery (CANTAB). Subjects were at their first neurological evaluation, before any AED therapy. We assessed: visual memory, induction and executive functions. In our group, the BRE children performed comparably with the control children for the induction and executive functions. Substantial differences were identified for the visual memory subtests: PRM percent correct (t = -2.58, p = 0.01) and SRM percent correct (t = -2.73, p = 0.01). Age of seizure onset had a negative impact on the visual memory subtest performances (PRM mean correct latency). We found significant correlations between the different CANTAB subtests results and characteristics of the centrotemporal spikes (CTS).
Discussion: Our results are consistent with the findings of other similar studies. This form of epilepsy is associated with subtle neuropsychological deficits, present at seizure onset. Neuropsychological deficits identified, suggest a more diffuse brain involvement in the epileptiform process.
Abbreviations: AED – AntiEpileptic Drug; BECTS - benign childhood epilepsy with centrotemporal spikes; BRE – Benign Rolandic Epilepsy; CANTAB - the Cambridge Neuropsychological Test Automated Battery; CTS – centrotemporal spikes; DMS – Delayed Matching Sample; EEG – electroencephalogram; ILAE – International league Against Epilepsy; MOT – Motor screening Test; PAL - Paired Associates Learning; PRM - Pattern Recognition Memory; SPSS - Statistical Package for the Social Sciences; SRM - Spatial Recognition Memory; SSP - Spatial Span, SWM - Spatial Working Memory
PMCID: PMC3307071  PMID: 22574100
benign; rolandic; children; epilepsy; CANTAB
4.  Epilepsy and Cognition 
Epilepsy Currents  2007;7(1):1-6.
It is well known that neuropsychological impairment can be associated with chronic epilepsy. This review suggests that a broad lifespan perspective of cognition in epilepsy should include consideration of: a) neurobiological factors that antedate the first seizure and influence cognition, b) epilepsy-related factors that influence brain growth and cognitive development after epilepsy is diagnosed and treated, c) clinical epilepsy and other risk factors associated with poor cognitive prognosis in the context of chronic pharmacoresistant epilepsy, and d) the modifiable and non-modifiable risk factors that influence cognitive aging in the general population.
PMCID: PMC1797884  PMID: 17304341
5.  Starting at the beginning: The neuropsychological status of children with new onset epilepsies 
This review examines the neurodevelopmental contributions to the cognitive and behavioral complications of epilepsy. Following a brief review of the lifespan complications of childhood epilepsies, attention turns to cognitive, psychiatric and social correlates of childhood epilepsies reported in population-based and tertiary care studies. The focus then becomes the neurobehavioral status of children with new onset epilepsy—a point in time not confounded by the effects of years of recurrent seizures, medications, and social reactions to epilepsy. Recent research shows that abnormalities in cognition, brain structure and behavior are present at or near the time of diagnosis. Further, careful history indicates that neurobehavioral problems may be present in advance of the first seizure suggesting the potential influence of epileptogenesis, antecedent neurodevelopmental abnormalities, genetic and environmental susceptibilities, and other risk factors. This becomes the substrate upon which to characterize the effects of epilepsy and its treatment on subsequent neurodevelopment. The review concludes with suggestions for future clinical care and research.
PMCID: PMC3701720  PMID: 22421240
The Clinical neuropsychologist  2010;24(3):429-453.
US demographic and sociopolitical shifts have resulted in a rapidly growing need for culturally competent neuropsychological services. However, clinical neuropsychology as a field has not kept pace with the needs of ethnic minority clients. In this discussion we review: historical precedents and the limits of universalism in neuropsychology; ethical/professional guidelines pertinent to neuropsychological practice with ethnic minority clients; critical cultural considerations in neuropsychology; current disparities germane to practice; and challenges to the provision of services to racial/ethnic minority clients. We provide a call to action for neuropsychologists and related organizations to advance multiculturalism and diversity within the field by increasing multicultural awareness and knowledge, multicultural education and training, multicultural neuropsychological research, and the provision of culturally competent neuropsychological services to racial/ethnic minority clients. Lastly, we discuss strategies for increasing the provision of culturally competent neuropsychological services, and offer several resources to meet these goals.
PMCID: PMC2909768  PMID: 20373222
Ethnic minority; Cultural competence; Diversity; Neuropsychology
7.  Prevalence and characteristics of epilepsy in the Belgian shepherd variants Groenendael and Tervueren born in Denmark 1995–2004 
The Belgian shepherd Groenendael and Tervueren is believed to be at higher risk of developing epilepsy than dogs of the common population. This epidemiological study was designed to estimate the prevalence of epilepsy in the Danish population of Groenendael and Tervueren born between 1995 and 2004. Furthermore, it was the intention to describe the clinical manifestation (seizure types and phenomenology) of epilepsy and to identify risk factors for euthanasia once the dog was diagnosed as having epilepsy.
All owners of Groenendael and Tervueren dogs born between January 1995 and December 2004 and registered in the Danish Kennel Club (1,248 dogs) were contacted and asked to answer a mailed questionnaire concerning epilepsy. Positive responders were subsequently validated in a follow-up interview conducted by telephone using a standardized questionnaire. Owners were questioned about age at first seizure, seizure frequency, seizure duration, a detailed description of seizure phenomenology, post-ictal signs and if a veterinarian had diagnosed the dog with epilepsy.
Prevalence of epilepsy was estimated at 9.5%. Mean age of epilepsy debut was 3.3 years (range 0.5–8.0 years). There was an almost equal number of Groenendael (25) and Tervueren (24). The distribution of females and males was 31 and 18 respectively. Twenty-five per cent experienced focal seizures, 53% experienced focal seizures with secondary generalization and 18% experienced primary generalized seizures. In four percent seizures were unclassifiable. The most commonly reported focal seizure phenomenology included ataxia, crawling, swaying, fearful behavior, salivation, excessive attention seeking and disorientation. In 16% of the cases, epilepsy led to euthanasia. Intact dogs with epilepsy had a significantly increased risk of being euthanized because of epilepsy compared to neutered dogs with epilepsy. In 22% of the cases the owners reported that anxiety/hyperactivity/stress could act as a seizure provoking factor.
A high prevalence of epilepsy appears to be present in the Danish Groenendael and Tervueren population. The relatively late debut age of epilepsy in this breed contributes greatly to the increased prevalence of epileptic individuals, because dogs developing epilepsy late in life are used for breeding unintended.
PMCID: PMC2633289  PMID: 19102738
8.  John Hughlings-Jackson: a sesquicentennial tribute. 
One hundred and fifty years have elapsed since the birth of John Hughlings-Jackson, a pivotal figure in the development of clinical neuroscience. In this review the origin of Jackson's postulate of a hierarchical organisation of function in the nervous system is described in the context of his education and his contacts with contemporaries, both in his clinical practice at The London Hospital and at the National Hospital, Queen Square, and in relation to the evolutionary approach to the organisation and ideas on biology and society set out by the philosopher Herbert Spencer.
PMCID: PMC1028998  PMID: 3531410
9.  Temporal Lobe Epilepsy Semiology 
Epilepsy represents a multifaceted group of disorders divided into two broad categories, partial and generalized, based on the seizure onset zone. The identification of the neuroanatomic site of seizure onset depends on delineation of seizure semiology by a careful history together with video-EEG, and a variety of neuroimaging technologies such as MRI, fMRI, FDG-PET, MEG, or invasive intracranial EEG recording. Temporal lobe epilepsy (TLE) is the commonest form of focal epilepsy and represents almost 2/3 of cases of intractable epilepsy managed surgically. A history of febrile seizures (especially complex febrile seizures) is common in TLE and is frequently associated with mesial temporal sclerosis (the commonest form of TLE). Seizure auras occur in many TLE patients and often exhibit features that are relatively specific for TLE but few are of lateralizing value. Automatisms, however, often have lateralizing significance. Careful study of seizure semiology remains invaluable in addressing the search for the seizure onset zone.
PMCID: PMC3420439  PMID: 22957241
10.  Care of patients with epilepsy in the community: will new initiatives address old problems? 
Epilepsy is a serious neurological condition that has important medical, psychological and social consequences. Up to 90% of patients with epilepsy are not under hospital supervision at any one time; the role of the general practitioner is therefore of central importance. There seems little doubt that community care of people with epilepsy must be improved. This article reviews the research findings on the quality of care of people with epilepsy, examines the barriers to effective community care, and looks at local and national initiatives to improve the care of patients with epilepsy in the community. Finally, the review considers how care can best be provided in an efficient, effective and acceptable manner within the resources of present day general practice. A patient-centred model is proposed that would tailor care to the needs of the individual patient.
PMCID: PMC1239510  PMID: 8745851
11.  Injuries in epilepsy: a review of its prevalence, risk factors, type of injuries and prevention 
Neurology International  2009;1(1):e20.
Currently, there is intense clinical research into various aspects of the medical risks relating to epilepsy, including total and cause-specific mortality, accidents and injuries in patients with epilepsy and mortality related with seizures. Seizures occurring in precarious situations and resulting in injuries are still an important concern for patients with epilepsy, their employers and their care-givers. Submersion injuries, motor vehicle accidents, burns, and head injuries are among the most feared epilepsy-related injuries. These concerns seem valid because the hallmark of epilepsy, episodic impairment of consciousness and motor control, may occur during interictal EEG epileptiform discharges, even in the absence of a clinical seizure. In addition, psychomotor comorbidity and side effects of antiepileptic drugs may contribute to the risk of injuries in patients with epilepsy. Published risk factors for injuries include the number of antiepileptic drugs, history of generalized seizures, and seizure frequency. In general, epidemiological information about incidence of injuries has been conflicting and sparse. In general, studies focusing on populations with more severe forms of epilepsy tend to report substantially higher risks of injuries than those involving less selected populations. On the other hand, studies based on non-selected populations of people with epilepsy have not shown an increased frequency of injuries in people with epilepsy compared with the general population. Some studies have shown that patients with epilepsy are more frequently admitted to the hospital following an injury. Possible explanations include: more cautious attitude of clinicians toward injuries occurring in the setting of seizures; hospitalization required because of seizures and not to the injuries themselves; and hospitalization driven by other issues, such as comorbidities, which are highly prevalent in patients with epilepsy. Potentially the high rate of hospitalizations could be related with the severity of the injury. This article reviews the best available epidemiological information about injuries, including incidence and risk factors. Also this article reviews information about specific types of injuries such as fractures, burns, concussions, dislocations, etc. Information about accidents in people with epilepsy is also discussed.
PMCID: PMC3093233  PMID: 21577358
injuries; epilepsy.
12.  Health-related quality of life in childhood epilepsy: Moving beyond 'seizure control with minimal adverse effects' 
Childhood epilepsy is one of the most important and prevalent neurological conditions in the developing years. Persons with childhood onset epilepsy are at a high risk for poor psychosocial outcomes, even without experiencing co-morbidities. The goal of management of children with epilepsy should be to enable the child and the family to lead a life as free as possible from the medical and psychosocial complications of epilepsy. This comprehensive care needs to go beyond simply trying to control seizures with minimal adverse drug reactions. Seizure frequency and severity is only one important outcome variable. Other factors such as social, psychological, behavioural, educational, and cultural dimensions of their lives affect children with epilepsy, their families and their close social networks.
A number of epilepsy-specific health-related quality of life (HRQL) scales for children have been developed with the aim to include and measure accurately the impact and burden of epilepsy. Their target populations, details of the origin of the items, and psychometric properties vary significantly. Their strengths and weaknesses will be identified more clearly through their continued use in the clinical setting and in research studies. Only a few studies to date have utilized these or generic HRQL measures to assess the HRQL of specific populations with epilepsy.
Future research needs to develop theory driven models of HRQL and identify measurable factors that have important correlations with outcomes. Since biomedical variables like seizure frequency and severity have only moderate correlations with HRQL, other independent factors including the child's resilience, co-morbid conditions, parental well-being, family factors and societal/cultural variables may play a major role. We also need to learn what encompasses comprehensive patient care, define the goals of management and evaluate the impact of different interventions. Future studies need to include the children's own perspectives of their HRQL in addition to parental reports.
Finally, clinicians need to familiarize themselves with outcome measures, be able to evaluate them, and use them routinely in their day-to-day practice.
PMCID: PMC201010  PMID: 14498989
review; health-related quality of life; outcome; measures; children; epilepsy; self-report measure; parents; proxy; goals of care
13.  SPECT Imaging of Epilepsy: An Overview and Comparison with F-18 FDG PET 
Epilepsy surgery is highly effective in treating refractory epilepsy, but requires accurate presurgical localization of the epileptogenic focus. Briefly, localization of the region of seizure onset traditionally dependents on seizure semiology, scalp EEG recordings and correlation with anatomical imaging modalities such as MRI. The introduction of noninvasive functional neuroimaging methods, including single-photon emission computed tomography (SPECT) and positron emission tomography (PET) has dramatically changed the method for presurgical epilepsy evaluation. These imaging modalities have become powerful tools for the investigation of brain function and are an essential part of the evaluation of epileptic patients. Of these methods, SPECT has the practical capacity to image blood flow functional changes that occur during seizures in the routine clinical setting. In this review we present the basic principles of epilepsy SPECT and PET imaging. We discuss the properties of the SPECT tracers to be used for this purpose and imaging acquisition protocols as well as the diagnostic performance of SPECT in addition to SPECT image analysis methods. This is followed by a discussion and comparison to F-18 FDG PET acquisition and imaging analysis methods.
PMCID: PMC3139140  PMID: 21785722
14.  Characteristics of late-onset epilepsy and EEG findings in children with autism spectrum disorders 
Korean Journal of Pediatrics  2011;54(1):22-28.
To investigate the clinical characteristics of late-onset epilepsy combined with autism spectrum disorder (ASD), and the relationship between certain types of electroencephalography (EEG) abnormalities in ASD and associated neuropsychological problems.
Thirty patients diagnosed with ASD in early childhood and later developed clinical seizures were reviewed retrospectively. First, the clinical characteristics, language and behavioral regression, and EEG findings of these late-onset epilepsy patients with ASD were investigated. The patients were then classified into 2 groups according to the severity of the EEG abnormalities in the background rhythm and paroxysmal discharges. In the severe group, EEG showed persistent asymmetry, slow and disorganized background rhythms, and continuous sharp and slow waves during slow sleep (CSWS).
Between the two groups, there was no statistically significant difference in mean age (P=0.259), age of epilepsy diagnosis (P=0.237), associated family history (P=0.074), and positive abnormal magnetic resonance image (MRI) findings (P=0.084). The severe EEG group tended to have more neuropsychological problems (P=0.074). The severe group statistically showed more electrographic seizures in EEG (P=0.000). Rett syndrome was correlated with more severe EEG abnormalities (P=0.002). Although formal cognitive function tests were not performed, the parents reported an improvement in neuropsychological function on the follow up checkup according to a parent's questionnaire.
Although some ASD patients with late-onset epilepsy showed severe EEG abnormalities, including CSWS, they generally showed an improvement in EEG and clinical symptoms in the long-term follow up. In addition, severe EEG abnormalities tended to be related to the neuropsychological function.
PMCID: PMC3040362  PMID: 21359057
Autism spectrum disorder; Epilepsy; Electroencephalography; Regression; Child
15.  Epileptic seizures in a population of 6000. I: Demography, diagnosis and classification, and role of the hospital services. 
A total of 122 patients with a history of non-febrile epileptic seizures were identified in a survey of 6000 persons from a single general practice. The lifetime prevalence was 20.3/1000 including single seizures and 17.0/1000 excluding single seizures; 5.3/1000 had active epilepsy. Diagnosis and classification of seizures in such a survey were difficult. Most patients were seen by a hospital specialist at some point, but hospital follow up was sporadic. Overall patient assessment and monitoring was poor. There is a place for specialised epilepsy clinics that could be organised along the lines of the clinics for diabetes. These provide facilities for initial diagnosis and assessment, for planning long term management, for the selected follow up of difficult cases, and for referral for specific problems. Such a system would improve the long term care of patients with epilepsy.
PMCID: PMC1548792  PMID: 6411263
16.  Commentary: Pediatric Epilepsy: A Good Fit for Pediatric Psychologists 
Journal of Pediatric Psychology  2010;36(4):461-465.
While there are an abundance of pediatric neuropsychologists working with youth with epilepsy (YWE), other subspecialty psychologists have played minimal roles in clinical and research endeavors in pediatric epilepsy. Thus, the purpose of this commentary was to describe (a) the needs of YWE due to the intermittent nature of seizures and difficulties with disease management, (b) increased risk for psychosocial comorbidities, (c) limited access to care, and (d) provide recommendations for how pediatric psychologists can become involved in the clinical care and research activities for YWE.
PMCID: PMC3079127  PMID: 21148174
17.  Febrile seizures: Mechanisms and relationship to epilepsy 
Brain & development  2009;31(5):366-371.
Studies of febrile seizures have been driven by two major enigmas: first, how these most common of human seizures are generated by fever has not been known. Second, epidemiological studies have linked prolonged febrile seizures with the development of temporal lobe epilepsy, yet whether long or recurrent febrile seizures cause temporal lobe epilepsy has remained unresolved. To investigate these questions, a model of prolonged (complex) febrile seizures was developed in immature rats and mice, permitting mechanistic examination of the potential causal relationships of fever and seizures, and of febrile seizures and limbic epilepsy. Although the model relied on hyperthermia, it was discovered that the hyperthermia-induced secretion of endogenous fever mediators including interleukin-1β, which contributed to the generation of these ‘febrile’ seizures. In addition, prolonged experimental febrile seizures provoked epilepsy in a third of the animals. Investigations of the mechanisms of this epileptogenesis demonstrated that expression of specific ion (HCN) channels and of endocannabinoid signaling, may be involved. These may provide novel drug targets for intervention in the epileptogenic process.
PMCID: PMC2698702  PMID: 19232478
Fever; Epileptogenesis; Animal models; Seizures; Hippocampus; Ion channels; Inflammation; Development
18.  Epilepsy (partial) 
Clinical Evidence  2011;2011:1214.
About 3% of people will be diagnosed with epilepsy during their lifetime, but about 70% of people with epilepsy eventually go into remission.
Methods and outcomes
We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of starting antiepileptic drug treatment following a single seizure? What are the effects of drug monotherapy in people with partial epilepsy? What are the effects of additional drug treatments in people with drug-resistant partial epilepsy? What is the risk of relapse in people in remission when withdrawing antiepileptic drugs? What are the effects of behavioural and psychological treatments for people with epilepsy? What are the effects of surgery in people with drug-resistant temporal lobe epilepsy? We searched: Medline, Embase, The Cochrane Library, and other important databases up to July 2009 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA).
We found 83 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions.
In this systematic review we present information relating to the effectiveness and safety of the following interventions: antiepileptic drugs after a single seizure; monotherapy for partial epilepsy using carbamazepine, gabapentin, lamotrigine, levetiracetam, phenobarbital, phenytoin, sodium valproate, or topiramate; addition of second-line drugs for drug-resistant partial epilepsy (allopurinol, eslicarbazepine, gabapentin, lacosamide, lamotrigine, levetiracetam, losigamone, oxcarbazepine, retigabine, tiagabine, topiramate, vigabatrin, or zonisamide); antiepileptic drug withdrawal for people with partial or generalised epilepsy who are in remission; behavioural and psychological treatments for partial or generalised epilepsy (biofeedback, cognitive behavioural therapy (CBT), educational programmes, family counselling, relaxation therapy (alone or plus behavioural modification therapy, yoga); and surgery for drug-resistant temporal lobe epilepsy ( lesionectomy, temporal lobectomy, vagus nerve stimulation as adjunctive therapy).
Key Points
During their lifetime, about 3% of people will be diagnosed with epilepsy, but about 70% of people with epilepsy eventually go into remission.
After a first seizure, antiepileptic drugs may delay or prevent subsequent seizures, but they can cause adverse effects, and their long-term benefit is unknown. Antiepileptic drug treatment after a single seizure does not reduce the risk of drug refractory epilepsy in the long term.
Carbamazepine, gabapentin, lamotrigine, levetiracetam, phenobarbital, phenytoin, sodium valproate, and topiramate are widely considered effective in controlling seizures in newly diagnosed partial epilepsy, but we found no RCTs comparing them with placebo, and a placebo-controlled trial would now be considered unethical. Systematic reviews found no reliable evidence on which to base a choice among antiepileptic drugs. Adding second-line drugs to usual treatment reduces seizure frequency in people with drug-resistant partial epilepsy, but it increases adverse effects such as dizziness and somnolence. We don't know if any one antiepileptic drug is more likely to reduce seizures compared with the others.
CAUTION: Vigabatrin, which may be used as second-line treatment, causes concentric visual-field abnormalities in about 40% of people, which are probably irreversible.
In people who have been seizure free for at least 2 years on treatment, almost 60% of those with partial or generalised epilepsy who withdraw from antiepileptic treatment will remain seizure free, compared with almost 80% of people who continue treatment.
Educational programmes may reduce seizure frequency and improve psychosocial functioning in people with partial or generalised epilepsy, but we don't know whether relaxation, yoga, biofeedback, CBT, relaxation plus behavioural modification, or family counselling are beneficial.
There is consensus that temporal lobectomy or amygdalohippocampectomy can improve seizure control and quality of life in people with drug-resistant temporal lobe epilepsy, but they can cause neurological adverse effects.
High-level vagus nerve stimulation may reduce seizure frequency in people with drug-resistant partial seizures, but it may cause hoarseness and dyspnoea, and long-term effects are unknown. We don't know whether different stimulation cycles are more effective at reducing seizure frequency or at increasing the proportion of responders.
We don't know whether lesionectomy improves seizure control in people with drug-resistant temporal lobe epilepsy.
PMCID: PMC3217777  PMID: 21549021
19.  Pseudosleep events in patients with psychogenic non-epileptic seizures: prevalence and associations 
Objectives: To determine the prevalence and clinical associations of a history of events during sleep in patients with psychogenic non-epileptic seizures (PNES, pseudoseizures), and to compare the prevalence of a history of sleep events with that in poorly controlled epilepsy.
Methods: Prospective study by semistructured interview of the history of event patterns and their clinical associations in 142 patients with video EEG confirmed PNES, and 100 patients with poorly controlled epilepsy.
Results: 84/142 patients with PNES (59%) and 47/100 with epilepsy (47%) gave a history of events during sleep (p = 0.062). In patients with PNES, significant associations were found between a history of sleep events and: convulsive clinical semiology, antiepileptic drug treatment, fatigue, suicide attempts, mood disorder, and physical abuse. A particularly strong association with social security benefit was also found (odds ratio 4.0, p<0.001).
Conclusions: The prevalence of a history of sleep events is similar in PNES and epilepsy, and is of no value in discriminating between the two, although a history of events occurring exclusively during sleep does suggest epileptic seizures. The clinical associations found indicate that a combination of psychopathological and external influences may be important in determining whether or not a patient with PNES gives a history of events during sleep.
PMCID: PMC1739122  PMID: 15201361
20.  Natural History of Temporal Lobe Epilepsy: Antecedents and Progression 
Temporal lobe epilepsy represents the largest group of patients with treatment resistant/medically intractable epilepsy undergoing epilepsy surgery. The underpinnings of common forms of TLE in many instances begin in early life with the occurrence of an initial precipitating event. The first epileptic seizure often occurs after a variable latency period following this event. The precise natural history and progression following the first seizure to the development of TLE, its subsequent resolution through spontaneous remission or the development of treatment resistant epilepsy remain poorly understood. Our present understanding of the role played by these initial events, the subsequent latency to development of temporal lobe epilepsy, and the emergence of treatment resistance remains incomplete. A critical analysis of published data suggest that TLE is a heterogeneous condition, where the age of onset, presence or absence of a lesion on neuroimaging, the initial precipitating event, association with febrile seizures, febrile status epilepticus, and neurotropic viral infections influence the natural history and outcome. The pathways and processes through which these variables coalesce into a framework will provide the basis for an understanding of the natural history of TLE. The questions raised need to be addressed in future prospective and longitudinal observational studies.
PMCID: PMC3420774  PMID: 22937237
21.  Psychomotor seizures, Penfield, Gibbs, Bailey and the development of anterior temporal lobectomy: A historical vignette 
Psychomotor seizures, referred to as limbic or partial complex seizures, have had an interesting evolution in diagnosis and treatment. Hughlings Jackson was the first to clearly relate the clinical syndrome and likely etiology to lesions in the uncinate region of the medial temporal lobe. With the application of electroencephalography (EEG) to the study of human epilepsy as early as 1934 by Gibbs, Lennox, and Davis in Boston, electrical recordings have significantly advanced the study of epilepsy. In 1937, Gibbs and Lennox proposed the term "psychomotor epilepsy" to describe a characteristic EEG pattern of seizures accompanied by mental, emotional, motor, and autonomic phenomena. Concurrently, typical psychomotor auras and dreamy states were produced by electrical stimulation of medial temporal structures during epilepsy surgery by Penfield in Montreal. In 1937, Jasper joined Penfield, EEG was introduced and negative surgical explorations became less frequent. Nevertheless, Penfield preferred to operate only on space occupying lesions. A milestone in psychomotor seizure diagnosis was in the year 1946 when Gibbs, at the Illinois Neuropsychiatric Institute, Chicago, reported that the patient falling asleep during EEG was a major activator of the psychomotor discharges and electrographic ictal episodes becoming more prominently recorded. Working with Percival Bailey, Gibbs was proactive in applying EEG to define surgical excision of the focus in patients with intractable psychomotor seizures. By early 1950s, the Montreal group began to clearly delineate causative medial temporal lesions such as hippocampal sclerosis and tumors in the production of psychomotor seizures.
PMCID: PMC2924506  PMID: 20814492
Electroencephalography; epilepsy; ictus; temporal lobe; temporal lobectomy
22.  The neurological founding fathers of the National Society for Epilepsy and of the Chalfont Centre for Epilepsy. 
The National Society for Epilepsy is the largest epilepsy charity in the United Kingdom, and administers the Chalfont Centre for Epilepsy. The Society was founded in London in 1892 and its first task was to establish an agricultural colony where people with epilepsy could live and work; and this was the origin of the Chalfont Centre. Recently, details of the early history of the Society have come to light showing that neurologists from the National Hospital, Queen Square were instrumental in its foundation. The meeting in which the society was constituted was held in the house of Thomas Buzzard, chaired by David Ferrier, and its first resolution was proposed by John Hughlings-Jackson. Other neurologists associated with its early history include William Gowers, Victor Horsley, Howard Tooth, and W Aldren Turner. In this paper we review the society's history and the light it throws on the attitudes to epilepsy and neurology in London in this exciting late Victorian period.
PMCID: PMC489607  PMID: 8509771
23.  Familial clustering of epilepsy and behavioral disorders: Evidence for a shared genetic basis 
Epilepsia  2011;53(2):301-307.
To examine whether family history of unprovoked seizures is associated with behavioral disorders in epilepsy probands, thereby supporting the hypothesis of shared underlying genetic susceptibility to these disorders.
We conducted an analysis of the 308 probands with childhood onset epilepsy from the Connecticut Study of Epilepsy with information on first degree family history of unprovoked seizures and of febrile seizures whose parents completed the Child Behavior Checklist (CBCL) at the 9-year follow-up. Clinical cut-offs for CBCL problem and DSM-Oriented scales were examined. The association between first degree family history of unprovoked seizure and behavioral disorders was assessed separately in uncomplicated and complicated epilepsy and separately for first degree family history of febrile seizures. A subanalysis, accounting for the tendency for behavioral disorders to run in families, adjusted for siblings with the same disorder as the proband. Prevalence ratios were used to describe the associations.
Key findings
In probands with uncomplicated epilepsy, first degree family history of unprovoked seizure was significantly associated with clinical cut-offs for Total Problems and Internalizing Disorders. Among Internalizing Disorders, clinical cut-offs for Withdrawn/Depressed, and DSM-Oriented scales for Affective Disorder and Anxiety Disorder were significantly associated with family history of unprovoked seizures. Clinical cut-offs for Aggressive Behavior and Delinquent Behavior, and DSM-Oriented scales for Conduct Disorder and Oppositional Defiant Disorder were significantly associated with family history of unprovoked seizure. Adjustment for siblings with the same disorder revealed significant associations for the relationship between first degree family history of unprovoked seizure and Total Problems and Agressive Behavior in probands with uncomplicated epilepsy; marginally significant results were seen for Internalizing Disorder, Withdrawn/Depressed and Anxiety Disorder.
There was no association between family history of unprovoked seizure and behavioral problems in probands with complicated epilepsy. First degree family history of febrile seizure was not associated with behavioral problems in probands with uncomplicated or in those with complicated epilepsy.
Increased occurrence of behavioral disorders in probands with uncomplicated epilepsy and first degree family history of unprovoked seizure suggests familial clustering of these disorders. This supports the idea that behavioral disorders may be another manifestation of the underlying pathophysiology involved in epilepsy or closely related to it.
PMCID: PMC3267857  PMID: 22191626
epilepsy; psychiatric disorders; family history; epidemiology
24.  Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report 
This is the first report of the case of a patient with a natural history of dysembryoplastic neuroepithelial tumor associated with probable sudden unexplained death in epilepsy. These tumors are benign, arising within the supratentorial cortex. Over 100 cases have been reported in the literature since the first description by Daumas-Duport in 1988.
Case presentation
A 24- year-old Caucasian woman had a long period of intractable complex partial seizures, sometimes with tonic-clonic generalization and neuropsychological abnormalities. Magnetic resonance imaging showed a cortico-subcortical parietal tumor with all the characteristics of these types of tumors. After 14 years of evolution, our patient died suddenly during sleep.
To the best of our knowledge, this is the first case of probable sudden unexplained death in symptomatic epilepsy due to dysembryoplastic neuroepithelial tumor with natural history. Early and complete excision, with functional studies before and during the surgery, leads to better control of seizures, avoiding neuropsychological changes and the risk of death. Patients with refractory epilepsy should be evaluated for any sleep disorders and should have complete cardiology assessments including electrocardiographic evaluation of cardiac rhythm disturbances.
PMCID: PMC3183037  PMID: 21899755
25.  Cognitive Impairment in Juvenile Myoclonic Epilepsy 
Background and purpose
Cognitive impairments are frequent consequences of epilepsy, with intellectual ability reportedly being lower in patients with idiopathic generalized epilepsies than in the general population. However, neuropsychological investigations have been rarely performed in patients with juvenile myoclonic epilepsy (JME). We aimed to quantify the cognitive function in JME patients using various neuropsychological tests.
We compared cognitive function in 27 JME patients with that in 27 healthy volunteers using tests examining cognitive performance, such as the verbal and visual memory, frontal function, attention, IQ score, and mood. In the JME group, we examined risk factors for cognitive function such as age, sex, family history, education level, age at seizure onset, seizure frequency, EEG abnormality, disease duration, and previous intake of antiepileptic drugs.
Verbal learning was significantly lower in JME patients than in controls, and attention and verbal fluency were impaired in JME patients compared with controls. However, general intellectual ability and mood did not differ between the groups. Early onset of seizure and long duration of disease were closely related to impaired cognitive function.
JME patients may exhibit impaired cognitive function, in terms of memory and execution, despite having normal intelligence and mood.
PMCID: PMC2686860  PMID: 19513297
Juvenile myoclonic epilepsy; Cognition; Neuropsychological tests; Mood

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