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Query: UMLS:C0036572 (seizures)
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Although psychotropic drugs are prescribed relatively often for childhood psychiatric and seizure disorders, relatively little is known about their use in everyday clinical settings--with the exception of children with ADHD and individuals with mental retardation. A legion of methodological and logistical obstacles are encountered when trying to gather information about pharmacotherapy for children, and this has often limited data collection to institutional settings or highly restricted geographic areas. Although initial efforts to study the extent of drug therapy focused primarily on the number of individuals receiving treatment and secondarily on drug dose, the breadth of topics quickly expanded to include numerous issues pertaining to the way psychotherapeutic agents are prescribed, evaluated, and monitored in typical clinical situations. This article presents an overview of treatment prevalence studies conducted during the past three decades and reviews a variety of clinical concerns raised by researchers in pediatric psychopharmacoepidemiology. The most salient findings from these studies are: (a) medication use continues to increase, (b) treatment prevalence rates in residential programs continue to be very high (40%-60%), and (c) many researchers continue to be concerned about the quality of treatment practices. The methodologies of this field show considerable promise for addressing pressing issues in health care for children and adolescents with emotional or behavioral disorders.
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PMID:An overview of three decades of research in pediatric psychopharmacoepidemiology. 954 94

Deletions within HSA band 4p16.3 cause Wolf-Hirschhorn syndrome (WHS), which comprises mental retardation and developmental defects. A WHS critical region (WHSCR) of approximately 165 kb has been defined on the basis of 2 atypical interstitial deletions; however, genotype-phenotype correlation remains controversial, due to the large size of deletion usually involving several megabases. We report on the first known patient with a small de novo interstitial deletion restricted to the WHSCR who presented with a partial WHS phenotype consisting only of low body weight for height, speech delay, and minor facial anomalies; shortness of stature, microcephaly, seizures and mental retardation were absent. The deletion was initially demonstrated by FISH analysis, and breakpoints were narrowed with a "mini-FISH" technique using 3-5 kb amplicons. A breakpoint-spanning PCR assay defined the distal breakpoint as disrupting the WHSC1 gene within intron 5, exactly after an AluJb repeat. The proximal breakpoint was not found to be associated with a repeated sequence or a known gene. The deletion encompasses 191.5 kb and includes WHSC2, but not LETM1. Thus, manifestations attributable to this deletion are reduced weight for height, minor facial anomalies, ADHD and some learning and fine motor deficiencies, while seizures may be associated with deletions of LETM1.
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PMID:First known microdeletion within the Wolf-Hirschhorn syndrome critical region refines genotype-phenotype correlation. 1125 5

Both children with epilepsy and children with ADHD may be characterized by slowing on reaction-time measurement. This is of particular interest, as neuropsychological assessment is often requested in the differential diagnosis between children with short non-convulsive epileptic seizures and children with ADHD. In this study we attempt to identify patterns of impairment on timed tasks that are specific for epilepsy, relative to ADHD. This study was an open, controlled parallel-group clinical investigation which included two groups of patients: 60 children with ADHD and 60 children with epilepsy. These children were compared with a control group (n=30) on two types of timed cognitive tasks: tasks with low information load (simple reaction-time measurement) and tasks with high information load (multiple decision reaction-time measurement). The simple reaction-time measurements show significant differences between ADHD and controls (all except for visual RT non-dominant hand) and between epilepsy and controls (only one test). No significant differences were found between epilepsy and ADHD. The two tests with high information load show significant slowing compared with the controls for epilepsy on the Binary Choice Reaction-Time Test and for ADHD on the Visual Searching Test. On both tests also the differences between epilepsy and ADHD are significant. The two tests in combination have a relatively satisfactory potential to classify the children with ADHD (75% correct classification) and the children with epilepsy (55% correct classification). We may conclude that complex reaction-time tests (i.e., timed tasks with high information load) have potential for assessing the differential impact of ADHD and epilepsy on attentional function. These tasks specifically reveal general slowing for children with epilepsy and slowing as an effect of failures of inhibitory self control on unstructured tasks for ADHD.
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PMID:A clinical comparative study evaluating the effect of epilepsy versus ADHD on timed cognitive tasks in children. 1140 98

The objective of this study was to compare the clinical and electroencephalographic features of two groups of children with benign epilepsy of childhood with rolandic spikes: those in whom seizure control was attained either without the use of medication or with a single medication (group 1) and those requiring two medications for seizure control (group 2). A consecutive series of children with benign epilepsy of childhood with rolandic spikes was identified in a single pediatric neurology practice. Medical charts were then systematically retrospectively reviewed. A total of 66 children with benign epilepsy of childhood with rolandic spikes was identified; 52 (78.8%) required either no medication (n = 7) or a single medication (n = 45) for seizure control, whereas 14 (21.2%) required two medications. The two groups did not differ with respect to gender, age of onset, type (generalized or partial) of initial seizure, or whether EEG abnormalities were unilateral or bilateral. They did differ significantly with respect to the mean number of seizures experienced prior to initiating treatment (group 1, 2.06, versus group 2, 4.36; t = 3.40, P = .005). In those treated (n = 59), the initial medication selected (carbamazepine versus noncarbamazepine) in the two groups was significantly different: group 1, 40 (carbamazepine)/45, versus group 2, 9 (carbamazepine)/14; chi2 = 4.59; P = .03. The difference in frequency between the two groups of associated comorbid conditions (tics, attention-deficit hyperactivity disorder [ADHD], learning disability) almost reached the threshold of statistical significance: group 1, 7/52, versus group 2, 5/14 (chi2 = 3.67, P = .06). A subset of children with benign epilepsy of childhood with rolandic spikes may require more than one medication for effective seizure control. This subset experiences more seizures prior to the initiation of treatment, is more likely to be treated initially with a noncarbamazepine medication, and tends to have a higher frequency of associated conditions.
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PMID:Atypical benign epilepsy of childhood with rolandic spikes: features of a subset requiring more than one medication for seizure control. 1259 63

Children who suffer from childhood seizure disorders, especially epilepsy, have various potential psychiatric issues and concerns that the treating physician and psychiatric consultant should consider. These children are at increased risk of adjustment reactions, anxiety and mood disorders, ADHD, learning difficulties, and familial and social stress. Because of potential risks and vulnerabilities for the development of comorbid psychiatric conditions and the increased risk for individual, familial, and social impairment, a psychiatric consultation to children and families dealing with epilepsy may play an important role in the successful management of this complex disorder.
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PMID:Psychiatric issues of childhood seizure disorders. 1291 Aug 22

Initially described as a rare MCA/MR syndrome occurring only in boys, due to a recessive mutation on the X chromosome [Opitz and Kaveggia, 1974], the FG syndrome (FGS) now emerges as a more common disorder also occurring in girls. Based on over 50 reported cases, FGS is associated with developmental delay (especially speech), hypotonia, postnatal onset relative macrocephaly, prominent forehead, frontal hair upsweep, telecanthus, or ocular hypertelorism, thin vermilion border of the upper lip, relatively short fingers with broad thumbs and halluces, persistent fetal fingertip pads, anal anomalies, and/or constipation. Major malformations are rare, and include pyloric stenosis, anal agenesis, cryptorchidism, hypospadias, and congenital heart defects. Abnormal EEGs and seizures have been reported in almost 70% of patients. Brain MRI shows corpus callosum abnormalities associated with dilatation of lateral ventricles and, less frequently, periventricular nodular heterotopias, mild cerebellar defects, and reduced periventricular white matter. Chiari 1 malformation seems to be frequent. The behavior phenotype appears to be characterized by ADHD, and relatively less developed language, fine motor and executive function skills; whereas visual-spatial abilities seem to be a relative strength. Five candidate loci are already known but no gene identified. We describe 25 patients referred to the Stella Maris Institute for evaluation of DD/MR, and diagnosed as FGS. They were between 2 and 15 1/2 years at the first observation. High resolution banding, FRAXA/FRAXE DNA analysis, and subtelomere FISH analysis were performed in all of them, and all had normal results. Thirteen patients were followed-up from 6 months to 9 years. Our report focuses on physical, neurological, developmental findings, and natural history of FGS. Experience with our series of patients suggests that the syndrome may be common, and should be routinely considered in the evaluation of children and adolescents with DD/MR.
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PMID:The FG syndrome: report of a large Italian series. 1669

A search of the literature of studies on comorbidity in children with idiopathic and cryptogenic epilepsy revealed very few prospective longitudinal studies. The tendency was that children with epilepsy had a greater prevalence of comorbidity than healthy children, but more prospective longitudinal studies are needed to show the exact prevalence, grade and character. Behaviour problems seem to occur before the children have seizures, i.e. the behaviour problem seems to exist before the onset of epileptic seizures. There was no sound evidence for the prevalence of comorbidity in the form of ADHD. Children with epilepsy seem to have normal IQ.
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PMID:[Comorbidity in children with epilepsy. I: Behaviour problems, ADHD and intelligence]. 1753 48

In this paper we explore the prevalence of ictal and interictal epileptiform discharges (IEDs) and sleep disorders in ADHD children referred to a sleep clinic for all night video-PSG. Forty-two ADHD outpatients (35 males and 7 females) underwent video-PSG and a behavioural/neuropsychological assessment. Spearman correlation coefficients (p<0.05 criterion level) were used to assess the association between cognitive, behavioural, clinical (co-morbidity), sleep (sleep efficiency) and EEG (seizures, IEDs, localization of IEDs foci) variables. Sleep disorders were found in 86% of ADHD children; among these, 26% had RLS. 53.1% of ADHD children had IEDs (28.2% centro-temporal spikes, 12.5% frontal spikes, 9.3% temporal-occipital spikes and 2.3% generalized S-W). Nocturnal seizures were recorded in three patients: two with atypical interictal rolandic spikes and one with left frontal slow abnormalities. A significant relationship (p<0.05) emerges between nocturnal seizures and WISC-R IQ score and visual-spatial memory test and between some cognitive variables and interictal rolandic spikes. High levels of inattention, impulsivity/hyperactivity and oppositional behaviours were related (p<0.01 or 0.05) with Restless Leg Syndrome diagnosis. In conclusion, ADHD is a condition often associated with EEG epileptiform abnormalities. Seizures/IEDs presence seems to play a role on cognitive abilities, conversely sleep disorders have a stronger impact on behavioural rather than cognitive indicators.
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PMID:Ictal and interictal EEG abnormalities in ADHD children recorded over night by video-polysomnography. 1758 23

XXYY syndrome occurs in approximately 1:18,000-1:40,000 males. Although the physical phenotype is similar to 47,XXY (tall stature, hypergonadotropic hypogonadism, and infertility), XXYY is associated with additional medical problems and more significant neurodevelopmental and psychological features. We report on the results of a cross-sectional, multi-center study of 95 males age 1-55 with XXYY syndrome (mean age 14.9 years), describing diagnosis, physical features, medical problems, medications, and psychological features stratified by age groups. The mean age of diagnosis was 7.7 years. Developmental delays and behavioral problems were the most common primary indication for genetic testing (68.4%). Physical and facial features varied with age, although hypertelorism, clinodactyly, pes planus, and dental problems were common across all age groups. Tall stature was present in adolescents and adults, with a mean adult stature of 192.4 cm (SD 7.5; n = 22). Common medical problems included allergies and asthma (>50%), congenital heart defects (19.4%), radioulnar synostosis (17.2%), inguinal hernia and/or cryptorchidism (16.1%), and seizures (15%). Medical features in adulthood included hypogonadism (100%), DVT (18.2%), intention tremor (71%) and type II diabetes (18.2%). Brain MRI (n = 35) showed white matter abnormalities in 45.7% of patients and enlarged ventricles in 22.8%. Neurodevelopmental and psychological difficulties were a significant component of the behavioral phenotype, with developmental delays and learning disabilities universal but variable in severity. Twenty-six percent had full-scale IQs in the range of intellectual disability (MR), and adaptive functioning was significantly impacted with 68% with adaptive composite scores <70. Rates of neurodevelopmental disorders, including ADHD (72.2%), autism spectrum disorders (28.3%), mood disorders (46.8%), and tic disorders (18.9%), were elevated with 55.9% on psychopharmacologic medication overall. Recommendations for evaluation and treatment are summarized.
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PMID:A new look at XXYY syndrome: medical and psychological features. 1848 Dec 71

Psychiatric disorders, such as mood, anxiety, attention deficit, and psychotic disorders, are among the most frequent comorbidities experienced by patients with epilepsy. While these psychiatric disorders have typically been considered as one of its complications, there is increasing evidence of a bidirectional relationship between the seizure disorder and mood and ADHD. Indeed, not only are patients with epilepsy at greater risk of developing these two disorders, but patients with mood and attention deficit disorders are at greater risk of developing epilepsy. Comorbid psychiatric disorders have a negative impact on the quality of life of patients with epilepsy. For patients with pharmacoresistant epilepsy, mood disorders are a stronger predictor of a worse perception of their quality of life than is their seizure frequency and severity. Thus, the use of psychotropic drugs is often necessary in patients with epilepsy, be they children or adults. Unfortunately, there are many misconceptions regarding the safety of psychotropic drugs, particularly of antidepressants and central nervous system stimulants, which are often erroneously thought of as being "proconvulsant." Such misconceptions have resulted in the undertreatment of psychiatric comorbidities in patients with epilepsy. This article provides a practical review of the use of antidepressants, central nervous system stimulants, and antipsychotic drugs in patients with epilepsy.
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PMID:The use of psychotropic drugs in epilepsy: what every neurologist should know. 1877 84


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