The association of cardiac asystole with partial seizures: does it result from ictal or interictal activity?

Bradycardia or asystole that occur during some seizures may be life threatening as a leading cause of SUDEP. A patient with right and left temporal lobe onset seizures and preceding bardycardia or asystole is presented. He had bilateral hippocampal atrophy on MRI. The unreliability of ictal bradycardia or asystole as a lateralizing sign in patients with partial epilepsy and the role of interictal autonomic activity in heart rate changes during seizures are discussed.     

Depressive symptoms predict the quality of sleep in patients with partial epilepsy — A combined retrospective and prospective study

IntroductionEpilepsy is one of the most common neurological diseases and has many detrimental effects on the patients' well-being as well as sleep quality. The aim of this study was to assess the subjective quality of sleep and influencing factors on subjective sleep quality in patients with partial epilepsy using a combined retrospective and prospective study design.MethodsWe conducted a combined retrospective and prospective study in patients with partial epilepsy and analyzed subjective ratings of sleep quality in 32 patients (17 female, 15 male; mean age: 40.41 ± 12.67 years, range: 20–64) with partial epilepsy (mean duration of epilepsy diagnosis: 18.31 ± 13.26 years) and 32 healthy gender-matched and age-matched controls. All patients filled out a seizure diary for 90 days, which included the number, duration, and type (partial vs. secondary generalized) of epileptic seizures and intake of antiepileptic and sleep medications. At baseline, all participants completed the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), Beck's Depression Inventory (BDI), and Beck Anxiety Inventory (BAI). Poor sleepers were defined by a PSQI score of ≥5.ResultsTwenty-three patients (72%) reported 15.17 ± 25.54 seizures in the previous three months, and nine (28%) patients reported being seizure-free. During the 90-day diary period, twenty-two patients (69%) documented a total of 319 epileptic seizures, while ten patients (31%) reported that they were seizure-free.The mean PSQI score of all patients was 4.88 ± 2.92 (range: 1–14) and the mean ESS score was 5.25 ± 2.98 (range: 0–10). The mean PSQI score of the control group was 3.25 ± 1.57 (range: 1–6), and their mean ESS score was 6.72 ± 3.48 (range: 0–14). The comparison of the two groups showed a significantly higher PSQI score in the patient group (t = 2.778, p = 0.008), but no statistically significant difference regarding their ESS score (t = − 1.811, p = 0.075). Sixteen (50%) patients were poor sleepers. Good sleepers showed a significantly lower PSQI (2.69 ± 1.08 vs. 7.06 ± 2.49; p < 0.001) and BDI scores (2.38 ± 2.50 vs. 9.63 ± 7.63; p < 0.002) than poor sleepers. Linear regression analysis showed that the BDI score was the significant predictor for the PSQI score (estimate: 0.2019; p = 0.00819) and for the ESS score (estimate: 0.2251; p = 0.0321).ConclusionIn patients with partial epilepsy, a higher depression score was the best predictor for a poor subjective sleep quality and increased daytime sleepiness.     

„The Borderland of Ictal Phenomenology and Pathophysiology“

The evaluation of episodic disorders of motor function, sensory awareness, cognition, alertness, and visceral integrity is among the core challenges of neurological work. Knowledge on the functional significance of symptoms and their differential diagnostic impact is of immense importance. Based on historical considerations of the basic pathophysiological principles of epileptic symptoms in addition to current results of neural networks investigations, the present review provides access to the broad spectrum of episodic disorders and their clinical presentation.     

Detrimental and synergistic role of epilepsy——Alzheimer's disease risk factors

<正>Identification of individuals at risk for developing Alzheimer's disease(AD)is an important issue for its pretreatment.Perspective studies predicting the disease are based on the genetic approaches emerging from the nucleotide polymorphism analysis in different loci through the entire genome in AD patients.Considerable diversity of genes located in these regions     

Lamotrigine in idiopathic epilepsy – increased risk of cardiac death?

OBJECTIVES: Lamotrigine (LTG) has recently been shown to inhibit the cardiac rapid delayed rectifier potassium ion current (Ikr). Ikr-blocking drugs may increase the risk of cardiac arrhythmia and sudden unexpected death. With this background, it may be of importance that in our outpatient clinic between August 1, 1995 and August 1, 2005 we registered four consecutive cases of sudden unexpected death in epilepsy (SUDEP) in non-hospitalized patients that were all being treated with LTG in monotherapy. Here we describe and discuss these cases, the relevant literature, and the reasons to question whether these events were as a result of coincidence alone. METHODS: All the cases were collected consecutively at the outpatient clinic, Department of Neurology, Stavanger University Hospital, Norway. Clinical and pathological data were obtained and the relevant literature reviewed. RESULTS: All were females with idiopathic epilepsy. CONCLUSIONS: A systematic study is needed to reveal whether LTG may increase the risk of SUDEP in certain groups of patients.     

Features of animal models of complex partial epilepsy established through unilateral, bilateral and alternate-side kindling at hippocampus of rats

IN T R O D U C T IO N At present, it is thoughtthatelectrophysiologicalm echanism of kin- dling is related to enhancem enteffectand burstspikes [1]. E nhance- m enteffectis long-term potentiation(LTP). Kindling anim alm odels of epilepsy has been w elldev…     

An appraisal of the new operational definition of epilepsy—Then and now

The focus to define epilepsy in the newly proposed classification has shifted from the conceptual perspective to practical application thought to better reflect that which is happening to the patient. Within the new definition, a single unprovoked or reflex seizure can be considered as epilepsy if the recurrence risk is similar to that following two unprovoked seizures. Epilepsy is considered to be resolved if the individual had an age-dependent epilepsy syndrome and has passed the applicable age or if the person has remained seizure-free for the last ten years without seizure medications for the last five years. This new operational definition of epilepsy may change the epileptologist's approach regarding when and how long to treat patients with seizures. The new definition also has significant psychosocial and employment-related implications for the patients. With regard to etiology, the terms idiopathic, symptomatic, and cryptogenic have been replaced by genetic, structural/metabolic, and unknown. This reflects a better understanding of the underlying cause of epilepsy based on genetic tests and better neuroimaging. The terms ‘simple partial’ and ‘complex partial’ seizures have been replaced by ‘focal motor/sensory’ and ‘focal dyscognitive’ seizures, thereby ending the ambiguity associated with the former terms and the difficulty encountered with definitions of altered states of consciousness. These changes, reflective of a better insight into the pathogenesis of seizures and epilepsy, are expected to be more pragmatic and assist when managing patients with epilepsy.     

Clinical manifestation and neurosurgical intervention of encephalocraniocutaneous lipomatosis—a case report and review of the literature

Introduction

Currently, there are only a few reported cases of symptomatic or asymptomatic subpial (intramedullary) spinal lipoma, and therefore no guidelines are available to indicate surgery. These lesions are infrequently associated with spina bifida.

Case report

Herein, we provide our experience in the neurosurgical intervention of compressive myeloradiculopathy for encephalocraniocutaneous lipomatosis (ECCL). The patient initially presented with bilateral upper hand paralysis, then regained muscle power after surgery and during 1 year of follow-up. We discuss the neurosurgical indications and intervention, imaging studies, other associated symptoms, and the pathogenesis of ECCL in an infant.     

Editor's Choice — Electrophysiological characteristics of epilepsy

Electroencephalographic signalscan act as an indicator of abnormaldischarge during epileptic seizures,providing important evidence fordiagnosing epilepsy. Abnormalelectroencephalographic activitywas detected during seizures, and     

Marijuana: an effective antiepileptic treatment in partial epilepsy? A case report and review of the literature

Although more data are needed, animal studies and clinical experience suggest that marijuana or its active constituents may have a place in the treatment of partial epilepsy. Here we present the case of a 45-year-old man with cerebral palsy and epilepsy who showed marked improvement with the use of marijuana. This case supports other anecdotal data suggesting that marijuana use may be a beneficial adjunctive treatment in some patients with epilepsy. Although challenging because of current federal regulations, further studies are needed to examine the role of marijuana in the treatment of this disorder.     

The genetics of epilepsy—The past,the present and future

A brief history of human geneticsSixty years is an appropriate yardstick for many reasons, not least for the remarkable advances in medicine, public health, psychology and biological disciplines. Particularly relevant is the approaching 60th anniversary of the discovery of the structure of DNA, which unlocked the driving force of nature and spawned a plethora of scientific discoveries and economic development through the Bitoech industry. Prior to 1953, and before Watson and Crick burst into the Cambridge pub with their eureka moment, it was known that chromosomes were important, the first principles of clinical cytogenetics were emerging and the rules of heritable traits were well-advanced, but without the basic framework or mechanism. Human Molecular Genetics arrived when the first mutations were linked to human disorders reflecting the advances in understanding the genetic code, assembly of protein building blocks and methodological advances in reading the physical code (all be it very difficult process at the time). Accelerated by the introduction of recombinant gene technology in the 1980s, and in conjunction with the development of linked genetic marker maps, the catalogue of genes associated with disease has risen exponentially with classical examples such as sickle cell disease, cystic fibrosis and Huntington's disease. The advances approached super-sonic dimensions when genes were found in Mendelian families, and mapping strategies were adopted using the variation map of the human genome (SNP's, di-nucleotide repeats), in addition to targeted candidate gene approaches aided by the significant database resources available to investigators. Super-sonic gave way to light-speed with the publication of the 3 billion letters of the genetic code which constitutes the human genome, followed quickly by genomes in plants, bacteria, pathogens, fruits and vegetables, and a menagerie of eukaryotic and prokaryotic animals, often representing model systems for genomic and pathophysiological research. In short don’t blink or you’ll miss the next revolution – too late, it's just happened!     

Analysis of cardiac parameters in animals with epilepsy: possible cause of sudden death?

Among the causes for sudden death in epilepsy, cardiac dysfunction has been an area of interest. Based on this, the aim of our study was to evaluate the heart rate (in vivo and in vitro) and ventricular pressure in vitro of rats with epilepsy induced by pilocarpine. Adult male Wistar rats (n=6) were given pilocarpine hydrochloride to induce status epilepticus. Control rats (n=6) received saline solution instead pilocarpine. Our results showed significant differences in the mean of heart rate in vivo between the groups. In contrast, we did not find differences during in vitro experiments. Our results suggest a central nervous system modulation on the heart, which could explain the sudden unexpected death in epilepsy.     

CPD — Education and self-assessment The epidemiology of epilepsy: the size of the problem

The prevalence of epilepsy is generally taken as between 5 and 10 cases per 1000 persons, and the overall incidence as about 50 cases per 100 000 persons. The rates are dependent on case ascertainment and on definitions used. The prognosis depends on many factors, including the number of seizures at presentation, the seizure type and the use of anti-epileptic drugs. Epilepsy carries an excess mortality; the cause of death can be unrelated to epilepsy, related to the underlying disease causing epilepsy, or related to epilepsy itself.     

The relevance of neuropsychiatric symptoms and cognitive problems in new-onset epilepsy — Current knowledge and understanding

Neurobehavioral and cognition problems are highly prevalent in epilepsy, but most research studies to date have not adequately addressed the precise nature of the relationship between these comorbidities and seizures. To address this complex issue and to facilitate collaborative, innovative research in the rising field of neurobehavioral comorbidities and cognition disturbances in new-onset epilepsy, international epilepsy experts met at the 3rd Halifax International Epilepsy Conference & Retreat at White Point, South Shore, Nova Scotia, Canada from September 18 to 20, 2014. This Conference Proceedings provides a summary of the conference proceedings. Specifically, the following topics are discussed: (i) role of comorbidities in epilepsy diagnosis and management, (ii) role of antiepileptic medications in understanding the relationship between epilepsy and neurobehavioral and cognition problems, and (iii) animal data and diagnostic approaches. Evidence to date, though limited, strongly suggests a bidirectional relationship between epilepsy and cognitive and psychiatric comorbidities. In fact, it is likely that seizures and neurobehavioral problems represent different symptoms of a common etiology or network-wide disturbance. As a reflection of this shared network, psychiatric comorbidities and/or cognition problems may actually precede the seizure occurrence and likely get often missed if not screened.     

Schizophrenia and sudden cardiac death—A review

Schizophrenia is a devastating mental disorder, which is often associated with severe loss of functioning and shortened life expectancy. Suicides and accidents are well-known causes of the excess mortality, but patients with schizophrenia have also been reported to be three times as likely to experience sudden unexpected death as individuals from the general population. This review is aimed to offer an update of the prevalence and mechanisms for sudden cardiac death in schizophrenia. The PubMed database was searched from 1966 up to May 2007 with key words schizophrenia AND “ sudden cardiac death” OR “autonomic dysfunction” OR “torsades de pointes”. Part of the high death rates may be explained by long-lasting negative health habits, disease- and treatment-related metabolic disorders, and consequent increased frequencies of cardiovascular diseases. The antipsychotic medications may also increase the risk as some antipsychotics may cause prolongation of QT-time, serious ventricular arrhythmias and predispose to sudden death. Autonomic dysfunction seen as low heart rate variability and decreased baroreflex sensitivity may also contribute via malignant arrhythmias. Due to the complex interaction of various risk factors for sudden death, the patients need a comprehensive follow-up of their physical health. In addition, more studies on the role and prevalence of autonomic dysfunction in psychotic patients are needed.