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.     

The epidemiology of the epilepsies in children

The epilepsies are a heterogeneous collection of neurological conditions and syndromes characterized by recurrent, unprovoked, paroxysmal seizure activity. There are several types of epileptic seizures and syndromes that are unique to children, including infantile spasms, Lennox-Gastaut syndrome and absence seizures. Febrile seizures and neonatal seizures, while not epilepsy, are relatively common types of seizures in infants and children and are likely markers of risk of later epilepsy. Thus, it is important to consider the epidemiological features of the epilepsies as they occur specifically in infants and children. The purpose of this review is to summarize what is currently known about the epidemiology of the childhood epilepsies and to identify promising areas for further population-based studies. The epilepsies are an important cause of neurological morbidity in children. The average annual rate of new cases (incidence) of epilepsy is approximately 5-7 cases per 10,000 children from birth to age 15 years, and in any given year, about 5 of every 1,000 children will have epilepsy. There is evidence that the incidence of the epilepsies in some populations of children may be decreasing over time, and this possibility merits further investigation. Factors that are known to increase risk of the epilepsies in children include congenital malformations of the central nervous system (CNS), moderate or severe head trauma, CNS infections, certain inherited metabolic conditions, and genetic factors. However, these account for only 25% to 45% of cases, and thus, the etiology of most cases of the epilepsies remains obscure. The paucity of well-controlled etiological studies is due largely to formidable methodological problems in conducting epidemiological studies of the epilepsies. The prognosis for seizure control is generally good, although children with remote symptomatic seizures and those with additional neurological disabilities do less well.     

Evaluation of a behaviour analysis and treatment of progressive myoclonus epilepsy, type Unverricht-Lundborg: a case study

This case study evaluates the clinical effectiveness of a psychological treatment within a behaviour medicine approach to epilepsy of a subject with progressive myoclonic epilepsy. The pattern of seizure behaviour was identified in the behaviour analysis. Self-management skills were taught to the client and in vivo exposure was subsequently used in a desensitization process. The main findings in this study were that the debilitating effects of the myclonic jerks can be reduced and functioning level improved when fear of seizures is reduced. These results indicate that psychological treatment can increase the level of functioning in clients with progressive myoclonic epilepsy.     

Evaluation of a Behaviour Analysis and Treatment of Progressive Myoclonus Epilepsy,Type Unverricht-Lundborg: A Case Study

This case study evaluates the clinical effectiveness of a psychological treatment within a behaviour medicine approach to epilepsy of a subject with progressive myoclonic epilepsy. The pattern of seizure behaviour was identified in the behaviour analysis. Self-management skills were taught to the client and in vivo exposure was subsequently used in a desensitization process. The main findings in this study were that the debilitating effects of the myclonic jerks can be reduced and functioning level improved when fear of seizures is reduced. These results indicate that psychological treatment can increase the level of functioning in clients with progressive myoclonic epilepsy.     

Possible uses of Convulsofin liquid in the treatment of pediatric epilepsies

The effect of Convulsofin-liquidum on several epilepsies and types of seizure in childhood is described. Typical and atypical absences, tonic-clonic seizures of primary generalised epilepsies as well as atonic or myoclonic seizures are considered to be the main indications for the treatment with Convulsofin. To the special advantage of the application in drops especially with infants is referred.     

Epilepsy by looking at patterns]

The author, after pointing out the rare occurence and peculiarities of pattern-sensitive forms of epilepsy, reports the case of a seven-year-old boy with infantile cerebral paresis characterized by symptomatic attacks, who was observed to show absence-like conditions when looking at small-checkered patterns of nylon fabric. Grasping and looking at such fabrics assumed the form of an addiction, with a sense of pleasure being believed to be derived therefrom. Although anticonvulsive therapy resulted in attacks of epilepsy being no longer detectable clinically and by electroencephalography, seizures were still triggered by looking at patterns. Also discussed in this article is the problem of transition from real to psychogenic attacks.     

One size does not fit all: face emotion processing impairments in semantic dementia, behavioural-variant frontotemporal dementia and Alzheimer's disease are mediated by distinct cognitive deficits

Patients with frontotemporal dementia (both behavioural variant [bvFTD] and semantic dementia [SD]) as well as those with Alzheimer's disease (AD) show deficits on tests of face emotion processing, yet the mechanisms underlying these deficits have rarely been explored. We compared groups of patients with bvFTD (n = 17), SD (n = 12) or AD (n = 20) to an age- and education-matched group of healthy control subjects (n = 36) on three face emotion processing tasks (Ekman 60, Emotion Matching and Emotion Selection) and found that all three patient groups were similarly impaired. Analyses of covariance employed to partial out the influences of language and perceptual impairments, which frequently co-occur in these patients, provided evidence of different underlying cognitive mechanisms. These analyses revealed that language impairments explained the original poor scores obtained by the SD patients on the Ekman 60 and Emotion Selection tasks, which involve verbal labels. Perceptual deficits contributed to Emotion Matching performance in the bvFTD and AD patients. Importantly, all groups remained impaired on one task or more following these analyses, denoting a primary emotion processing disturbance in these dementia syndromes. These findings highlight the multifactorial nature of emotion processing deficits in patients with dementia.     

Social rank and affiliation in social anxiety disorder

The present study examined the interpersonal lives of individuals with social anxiety disorder (SAD). According to evolutionary and interpersonal theories, we construed the interpersonal world using the social rank and the affiliation psychological systems. Two studies assessed measures of social rank, affiliation, social anxiety and depression among a population of treatment-seeking individuals with SAD. In study 1, individuals with SAD without major depressive disorder (MDD; n=42) were compared to healthy controls (n=47). In study 2, individuals with SAD and MDD (n=45) were compared to individuals with other anxiety disorders and MDD (n=31). Results indicated that SAD was related to perceiving oneself as having low social rank, being inferior, and behaving submissively, as well as to low perceived intimacy and closeness among peer relations, friendships and romantic relations. SAD was distinctly associated with these perceptions above and beyond the symptomatic (study 1) and the syndrome-level (study 2) effects of depression. These findings were further supported by a path analysis of the SAD participants from both studies. Our findings highlight the need to address both social rank and affiliation issues in the assessment and treatment of SAD.     

Cognitive-behavioral stress management increases free testosterone and decreases psychological distress in HIV-seropositive men.

The effects of a 10-week group-based cognitive-behavioral stress management (CBSM) intervention on psychological distress and plasma free testosterone in symptomatic, HIV-seropositive men were examined. Participants were randomized to either CBSM (n = 42) or a wait-list control group (n = 23). Men in the CBSM intervention showed significant increases in testosterone, whereas control participants showed significant decreases. Those participating in CBSM had significant distress reductions, whereas controls showed no such change. Alterations in free testosterone were inversely related to changes in distress states over time, independent of any changes in cortisol. These findings demonstrate that a short-term CBSM intervention increases free testosterone levels among symptomatic, HIV-seropositive men, and alterations in free testosterone are associated with changes in psychological distress observed during CBSM.     

Relation between cardiovascular and metabolic disease and cognition in very old age: cross-sectional and longitudinal findings from the berlin aging study.

This study documented findings on the relation between cognitive functioning (perceptual speed, memory, fluency, and knowledge) and cardiovascular and metabolic disease in a sample of very old adults (ages 70 and older), both cross-sectionally (n=516) and longitudinally (n=206) in a 4-year follow-up. After age, SES, sex, and dementia status were controlled for, 4 diagnoses were negatively associated with cognition: congestive heart failure, stroke, coronary heart disease, and diabetes mellitus, with a joint effect of 0.47 standard deviations. The impact of disease status was largest on perceptual speed and fluency, memory was impacted only by diabetes, and knowledge was not related to any somatic diagnosis. There was no differential decline in participants diagnosed with 1 of these 4 diseases and those who were not. The only cardiovascular risk factor associated with cognitive performance was alcohol consumption.     

A Systematic Review of Psychiatric and Psychosocial Comorbidities of Genetic Generalised Epilepsies (GGE)

Psychiatric disorders and associated poor psychosocial outcomes are recognised to be a common sequelae of epilepsy. The extent to which this is true of genetic generalised epilepsies (GGE), particularly syndromes other than juvenile myoclonic epilepsy (JME) is unclear. This systematic review synthesises findings regarding psychiatric and associated comorbidities in adults and children with GGE. Systematic review yielded 34 peer-reviewed studies of psychiatric and psychosocial outcomes in adults and children with GGE. Clinically significant psychiatric comorbidity was reported in over half of all children and up to a third of all adults with GGE. There was no evidence to support the presence of personality traits specific to JME or other syndromes; rather rates mirrored community samples. A small number of studies report poor psychosocial outcomes in GGE, however the interpretation of these findings is limited by paucity of healthy comparison groups. Some evidence suggests that anti-epileptic drug polytherapy in children and seizure burden at all ages may constitute risk factors for psychopathology. Findings highlight the importance of early screening so as not to overlook early or developing symptoms of psychopathology.     

Semantic priming effects in normal versus poor readers

The authors studied sensitivity to semantic priming, as distinct from semantic judgment, in poor readers. Association strength (high vs. low semantic association) was manipulated factorially with semantic association type (categoric vs. thematic association). Participants were 11-year-old poor readers (n = 15) who were matched with a group of chronological-age controls (n = 13), and also matched with a group of reading-age controls (n = 15). Three priming conditions were used: related, unrelated, and neutral prime. Neutral primes consisted of a row of hash marks. Related primes elicited shorter decision latencies than did unrelated primes. Neutral primes elicited the slowest responses in all groups. Poor readers showed an additional delay in the neutral prime condition. No effects of association type (categorical vs. thematic) or association strength (high vs. low) were found, nor were any relationship with reading ability found. The delayed performance of the poor readers on neutral primes is explained in terms of orthographic processing and dependency on grapheme phoneme relationships. The findings are discussed with reference to F. R. Vellutino, D. M. Scanlon, and D. Spearing's (1995) work on semantic processing and reading ability.     

Response inhibition in children with DSM-IV subtypes of AD/HD and related disruptive disorders: the role of reward.

The current study had four aims: (a) to replicate previous findings of slow response inhibition in Attention Deficit/Hyperactivity Disorder (AD/HD), (b) to explore whether poor response inhibition in children with AD/HD is a core problem or rather a result of an underlying problem related to reward, (c) to investigate the specificity of poor response inhibition and the role of reward in relation to AD/HD, and (d) to study whether findings would be different for three subtypes of AD/HD. In order to address these issues, a stop paradigm was administered under a reward condition and under a nonreward condition to an AD/HD group (n=24), an Oppositional Defiant Disorder (ODD)/Conduct Disorder (CD) group (n=21), a comorbid AD/HD+ODD/CD group (n=27), and a normal control (NC) group (n=41). Firstly, contrary to prediction, none of the Disruptive Behavior Disorder (DBD) groups differed from the NC group with respect to the speed of the inhibition process. Secondly, it was shown that children with AD/HD and children with comorbid AD/HD+ODD/CD, but not children with ODD/CD alone, slowed down more dramatically in the reward condition than normal controls. This finding was interpreted as a strategy to increase the chance of being rewarded in children with AD/HD and children with comorbid AD/HD+ODD/CD, but not in children with pure ODD/CD. Finally, analysis of AD/HD subtypes did not change the main findings of this study.     

Response Inhibition in Children With DSM-IV Subtypes of AD/HD and Related Disruptive Disorders: The Role of Reward

The current study had four aims: (a) to replicate previous findings of slow response inhibition in Attention Deficit/Hyperactivity Disorder (AD/HD), (b) to explore whether poor response inhibition in children with AD/HD is a core problem or rather a result of an underlying problem related to reward, (c) to investigate the specificity of poor response inhibition and the role of reward in relation to AD/HD, and (d) to study whether findings would be different for three subtypes of AD/HD. In order to address these issues, a stop paradigm was administered under a reward condition and under a nonreward condition to an AD/HD group (n = 24), an Oppositional Defiant Disorder (ODD)/Conduct Disorder (CD) group (n = 21), a comorbid AD/HD+ODD/ CD group (n = 27), and a normal control (NC) group (n = 41). Firstly, contrary to prediction, none of the Disruptive Behavior Disorder (DBD) groups differed from the NC group with respect to the speed of the inhibition process. Secondly, it was shown that children with AD/HD and children with comorbid AD/HD+ODD/CD, but not children with ODD/CD alone, slowed down more dramatically in the reward condition than normal controls. This finding was interpreted as a strategy to increase the chance of being rewarded in children with AD/HD and children with comorbid AD/HD+ODD/CD, but not in children with pure ODD/CD. Finally, analysis of AD/HD subtypes did not change the main findings of this study.     

Residential Water Use: Predicting and Reducing Consumption1

This project had two goals: to explain variation in residential water consumption and to evaluate methods of encouraging residents to reduce their consumption. Survey data for both studies were collected by mail questionnaire in early 1991, and water consumption figures were recorded between June and August of that year. In Study 1 (n = 264) a three-variable regression model (number of residents, clothes washing machine loads, and property value) accounted for 60% of the variance. Attitudes, habits and values were very poor predictors of water consumption. In Study 2 (n =226) households were divided into three treatment groups: feedback only, feedback and dissonance, and a control group. Repeated-measures ANOVA revealed that high consumers receiving dissonance and feedback or feedback alone had significantly reduced their water consumption in the treatment period. The implications of these findings are discussed.     

Epilepsy genes: the link between molecular dysfunction and pathophysiology

Our understanding of the genetic basis of epilepsy is progressing at a rapid pace. Gene mutations causing several of the inherited epilepsies have been mapped, and several more are likely to be added in coming years. In this review, we summarize the available information on the genetic basis of human epilepsies and epilepsy syndromes, emphasizing how genetic defects may correlate with the pathophysiological mechanisms of brain hyperexcitability. Mutations leading to epilepsy have been identified in genes encoding voltage- and ligand-gated ion channels (benign familial neonatal convulsions, autosomal dominant nocturnal frontal lobe epilepsy, generalized epilepsy with febrile seizures "plus"), neurotransmitter receptors (Angelman syndrome), the molecular cascade of cellular energy production (myoclonic epilepsy with ragged red fibers), and proteins without a known role in neuronal excitability (Unverricht-Lundborg disease). Gene defects can lead to epilepsy by altering multiple and diverse aspects of neuronal function.     

The impact of premature childbirth on parental bonding

The development of an affectionate parent-infant bond is essential for a newborn infant's survival and development. However, from evolutionary theory it can be derived that parental bonding is not an automatic process, but dependent on infants' cues to reproductive potential and parents' access to resources. The purpose of the present study was to examine the process of bonding in a sample of Dutch mothers (n = 200) and fathers (n = 193) of full-term (n = 69), moderately premature (n = 68), and very premature infants (n = 63). During the first month postpartum parents completed the Pictorial Representation of Attachment Measure (PRAM) and Postpartum Bonding Questionnaire (PBQ). Longitudinal analyses revealed that mothers' PRAM scores decreased after moderately preterm delivery, whereas decreases in PRAM scores occurred in both parents after very preterm delivery. As lower PRAM scores represent stronger feelings of parent-infant connectedness, our findings suggest a higher degree of bonding after premature childbirth. Results of the PBQ analysis were in line with PRAM outcomes, as parents of preterm infants reported less bonding problems compared to parents of full-terms. These findings support the hypothesis that in affluent countries with adequate resources, bonding in parents of preterm infants on average may be higher than in parents of full-term infants.     

APPLYING RANGE RESTRICTION CORRECTIONS USING PUBLISHED NORMS: THREE CASE STUDIES

This study evaluated the accuracy of range restriction corrections. Initial range restriction corrections used published norms as a proxy for unrestricted applicant data. The accuracy of the initial correction was evaluated using two large independent follow-up samples ( n = 983 and n = 1,389). The author found the initial norm-based correction to be very consistent with empirical findings. The application of these findings to range restriction corrections with customized (i.e., non-published) selection tests is also discussed.     

An examination of the ECST-R as a screen for feigned incompetency to stand trial

Psychological assessments of competency-to-stand-trial (CST) referrals must consider whether the defendants' impairment is genuine or feigned. This study addressed feigning on the Evaluation of Competency to Stand Trial--Revised (ECST-R), a standardized interview designed for assessing dimensions of CST and screening for feigned CST. In particular, this study examined the effectiveness of the ECST-R's Atypical Presentation (ATP) scales as screens for feigned incompetency. It examined ATP scales for (a) jail detainees (n=96) in simulation and control conditions and (b) inpatient competency cases (n=56) in clinical comparison and probable malingering groups. Comparisons of ATP scales yielded very large effect sizes for feigners when compared with jail controls (mean d=2.50) and genuine inpatient competency cases (mean d=1.83). Several cut scores were established with very few false negatives and robust sensitivity estimates. In summary, the ECST-R ATP scales appear to be homogenous scales with established clinical use as feigning screens in CST evaluations.     

Massively multiplayer online role-playing game-induced seizures: a neglected health problem in Internet addiction.

As the Internet has become rapidly and widely integrated into society, Internet addiction has become a growing psychosocial problem. However, epileptic seizure, another out-of-the-ordinary health problem, is often neglected in this regard. Ten patients who experienced epileptic seizures while playing the newest genre of electronic games -- Massively Multiplayer Online Role-Playing Games (MMORPGs) -- were investigated. Patients were predominantly male young adults, and most of the events were generalized tonic-clonic seizures, myoclonic seizures, and absences. These patients should be categorized into idiopathic generalized epilepsies. Even though photosensitivity was an important factor, behavioral and higher mental activities also seemed to be significant seizure precipitants. Results demonstrated that MMORPG-induced seizures were not analogous to the ordinary video game-induced seizures. Significantly, an epileptic seizure warning did not always appear on the websites of MMORPGs and instructions for the software. While the prevalence of MMORPG-induced seizures remains unknown, it may exceed our expectations and impact our society. Not only for clinical neurologists but also for the primary physicians, educators, sociologists, and global online game publishers, there should be an awareness of this special form of reflex seizures in order to provide an appropriate health warning to MMORPG players.