A new approach for assessing executive functions in everyday life, among adolescents with Genetic Generalised Epilepsies.

Studies have characterised relationships between cognitive status and a variety of clinical epilepsy factors. The aim of this study was to describe a new approach for assessing executive functions in everyday life and its unique expression in adolescents with Genetic Generalised Epilepsies (GGEs) compared with typical peers. Twenty adolescents with a diagnosis of GGEs and 20 typical healthy peers, matched by age and gender, were studied. Assessment of everyday executive function was carried out using: (1) the Weekly Calendar Planning Activity (WCPA), a direct performance based and outcome measure of strategy use and cognitive performance; and (2) Behavior Rating Inventory of Executive Function (BRIEF) parental report. Adolescents with GGEs demonstrated significantly less accuracy, less efficiency and fewer strategies used, as measured by the WCPA. Parents of adolescents with GGEs rated their child's daily performance as less efficient compared with typical peers. Better ratings of executive function (low BRIEF score) were associated with greater WCPA accuracy in the entered appointments. The WCPA provides a useful evaluation of cognitive performance for adolescents with GGEs and a functionally relevant information on task efficiency, self-monitoring and effective strategy use. Direct observation of performance supplements parental ratings and has strong potential to guide intervention and measure outcomes.

Long-term motor, cognitive and behavioral outcome of acute disseminated encephalomyelitis.

OBJECTIVE: The purpose of this study was to evaluate the long-term motor and neurocognitive outcome of children with acute disseminated encephalomyelitis and to identify prognostic risk factors. METHODS: The study included 43 children who were hospitalized due to acute disseminated encephalomyelitis during the years 2002-2012. The children underwent full neurological examinations, along with comprehensive neurocognitive and behavioral assessments. RESULTS: Twenty-six (61%) children had different degrees of neurological sequelae after a mean follow-up of 5.5 ± 3.5 years. The most common residual impairment included attention-deficit hyperactivity disorder (44%), behavioral problems (32%), and learning disabilities (21%). Five (12%) children had a full-scale IQ of 70 or less, compared to 2.2% in the general population. CONCLUSIONS: Neurocognitive sequelae were found even in children who were considered as fully recovered at the time of discharge. Risk factors for severe neurological sequelae were older age at diagnosis and male gender. We suggest neuropsychological testing and long-term follow-up for all children with acute disseminated encephalomyelitis, even in the absence of neurological deficits at discharge.

Visual Outcome and Recurrence Rate in Children With Idiopathic Intracranial Hypertension.

The purpose of this retrospective study was to evaluate the visual outcome and recurrence rate of idiopathic intracranial hypertension in children. The study included 68 patients who were diagnosed with idiopathic intracranial hypertension according to the modified Dandy criteria. Permanent visual impairment was rare. Three percent remained with mild visual impairment, 4% with minimal visual field defects, and only 1 patient had severe visual impairment. However, 26% had either a prolonged course of disease or a recurring condition. Higher cerebrospinal fluid opening pressure was the only clinical predictor at presentation (P = .04). Recurrence rate was 18%, and in most cases, the second episode occurred during the first year after remission. There was no significant difference between the group of patients with only 1 episode and the group of patients with more than 1 episode. We suggest long-term follow-up after remission, for at least a year, for all children with idiopathic intracranial hypertension.

Childhood-onset primary generalized epilepsy--impacts on children's preferences for participation in out-of-school activities.

The purpose of this study was to compare preferences for participation in out-of-school activities between children with childhood-onset primary generalized epilepsy and their healthy peers. Overall, participants were 56 children aged 6-11 years. The study group included 26 children with childhood-onset primary generalized epilepsy. The controls were 30 healthy children. Parents of all participants completed a demographic and health status questionnaire. All children completed the Preference Assessment of Children (PAC) that profiles the out-of-school activities the child wishes to participate in. Scores are calculated for five activity types, namely, recreational, active physical, social, skill-based, and self-improvement and for two domains of formal and informal activities. Children with generalized epilepsy showed a similar preference for participation in out-of-school activities as did their healthy peers. The study group showed a lower preference for participation in social activities but showed a higher preference for participation in self-improvement activities. In both groups, younger children (aged 6-8 years) showed a lower preference for participation in most PAC scales. Older children (aged 9-11 years) showed a higher preference for participation in social activities. Difference between genders was close to being statistically significant in the skill-based activities (F(1,21)=3.84, p=.06), where girls showed a higher preference compared with boys. Intervention policies need to be undertaken in order to encourage children with epilepsy to participate in activities together with their healthy peers, aiming to enhance the well-being of children with primary generalized epilepsy.

Long-term motor and cognitive outcome of acute encephalitis.

OBJECTIVES: To examine the long-term motor and neurocognitive outcome of children with acute encephalitis and to look at possible prognostic factors. METHODS: Children who were treated for acute encephalitis in 2000-2010 were reevaluated. All children and their parents were interviewed by using structured questionnaires, and the children underwent full neurologic examinations, along with comprehensive neurocognitive, attention, and behavioral assessments. RESULTS: Of the 47 children enrolled, 1 died and 29 had neurologic sequelae, including motor impairment, mental retardation, epilepsy, and attention and learning disorders. Children with encephalitis had a significantly higher prevalence of attention-deficit/hyperactivity disorder (50%) and learning disabilities (20%) compared with the reported rate (5%-10%) in the general population of Israel (P < .05) and lower IQ scores. Lower intelligence scores and significantly impaired attention and learning were found even in children who were considered fully recovered at the time of discharge. Risk factors for long-term severe neurologic sequelae were focal signs in the neurologic examination and abnormal neuroimaging on admission, confirmed infectious cause, and long hospital stay. CONCLUSIONS: Encephalitis in children may be associated with significant long-term neurologic sequelae. Significant cognitive impairment, attention-deficit/hyperactivity disorder, and learning disabilities are common, and even children who were considered fully recovered at discharge may be significantly affected. Neuropsychological testing should be recommended for survivors of childhood encephalitis.

Oxidative stress, metalloproteinase and LDH in children with intractable and non-intractable epilepsy as reflected in salivary analysis.

UNLABELLED: Oxidative stress and metalloproteinase (MMPs) may have a pivotal role in the pathogenesis of epilepsy. This study examined the underlying mechanism of epilepsy in children and especially in its intractable form, with respect to the roles of MMP's and free radicals in general and in saliva in particular. We also explored the possible diagnostic role of a compositional salivary analysis in these children, as salivary collection is simple, non-invasive (and thus 'children-friendly') and requires almost no expertise. We analyzed saliva parameters of 33 epileptic children: 22 with non-intractable (E's) and 11 with intractable epilepsy (IE's), and compared them with 16 healthy controls. Mean salivary LDH concentration in controls was 213.1±34.0IU/L, dropping by 38% (p=0.014) in E's and by 76% (p=0.0003) in IE's. Mean salivary values of peroxidase activity, SOD activity and carbonyls level were 480±14mU/mL, 1.30±0.15U/mL and 0.34±0.04nmol/mg, respectively, in controls, increasing by 6% (p=0.03), by 37% (p=0.04) and by 59% (p=0.003) in E's, and by 10% (p=0.02), by 29% (p=0.03) and by 56% (p=0.004) in IE's. Mean salivary MMP 9 concentration was 0.062±0.003 (OD) in controls, decreased by 12% (p=0.048) in E's, and by 23%, (p=0.009) in IE's. Our results enhance our understanding of epilepsy's biological underlying mechanism as reflected in the saliva of children with both intractable and non intractable disease. SIGNIFICANCE: The currently reported salivary analysis and the demonstrated salivary alterations in children suffering from epilepsy represent a novel direction. We found various salivary alterations demonstrated in the general composition as well as the oxidative and metalloproteinase analyses and more so in the intractable epilepsy group than in the non intractable epilepsy group. Hence, salivary oxidative components and MMP levels were found useful in the detection and follow-up of children with epilepsy. As such, we recommend using this non-invasive salivary analysis for diagnosis and monitoring of epileptic activity in children.

Viral-induced intracranial hypertension mimicking pseudotumor cerebri.

BACKGROUND: Pseudotumor cerebri or idiopathic intracranial hypertension is characterized by normal spinal fluid composition and increased intracranial pressure in the absence of a space-occupying lesion. METHODS: This study describes a subgroup of 10 patients with the same typical presenting symptoms (headache, vomiting, and papilledema) but without nuchal rigidity, meningeal signs, or change in mental status. Patients had normal neuroimaging studies and intracranial hypertension but also pleocytosis in the cerebrospinal fluid, suggesting central nervous system infection. From the results it can be hypothesized that those children represent a unique subgroup of viral-induced intracranial hypertension when comparing their risk factors, clinical course, treatment, and outcome with 58 patients who had idiopathic intracranial hypertension. RESULTS: All patients with viral-induced intracranial hypertension presented with papilledema but none had reduced visual acuity or abnormal visual fields, compared with 20.7% of patients who had idiopathic intracranial hypertension. They also responded better to treatment with acetazolamide, needed a shorter duration of treatment (7.7 ± 2.6 months vs 12.2 ± 6.3 months, P = 0.03), and had no recurrences. CONCLUSIONS: The results suggest that children who fulfill the typical presenting signs and symptoms and all diagnostic criteria for pseudotumor cerebri other than the normal cerebrospinal fluid component may represent a unique subgroup of viral-induced intracranial hypertension and should be managed accordingly. The overall prognosis is excellent.

Primary headaches, attention deficit disorder and learning disabilities in children and adolescents.

BACKGROUND: Primary headaches and Learning difficulties are both common in the pediatric population. The goal of our study was to assess the prevalence of learning disabilities and attention deficit disorder in children and adolescents with migraine and tension type headaches. METHODS: Retrospective review of medical records of children and adolescents who presented with headache to the outpatient pediatric neurology clinics of Bnai-Zion Medical Center and Meyer Children's Hospital, Haifa, during the years 2009-2010. Demographics, Headache type, attention deficit disorder (ADHD), learning disabilities and academic achievements were assessed. RESULTS: 243 patients met the inclusion criteria and were assessed: 135 (55.6%) females and 108 (44.4%) males. 44% were diagnosed with migraine (35.8% of the males, 64.2% of the females, p = 0.04), 47.7% were diagnosed with tension type headache (50.4% of the males, 49.6% of the females). Among patients presenting with headache for the first time, 24% were formerly diagnosed with learning disabilities and 28% were diagnosed with attention deficit disorder (ADHD). ADHD was more prevalent among patients with tension type headache when compared with patients with migraine (36.5% vs. 19.8%, p = 0.006). Poor to average school academic performance was more prevalent among children with tension type headache, whereas good to excellent academic performance was more prevalent among those with migraine. CONCLUSIONS: Learning disabilities and ADHD are more common in children and adolescents who are referred for neurological assessment due to primary headaches than is described in the general pediatric population. There is an association between headache diagnosis and school achievements.

Obesity in children with headaches: association with headache type, frequency, and disability.

OBJECTIVE: To examine the association between obesity and the different types of primary headaches, and the relation to headache frequency and disability BACKGROUND: The association between obesity and headache has been well established in adults, but only a few studies have examined this association in children, in particular, the relationship between obesity and different types of primary headaches. METHODS: The authors retrospectively evaluated 181 children evaluated for headaches as their primary complaint between 2006 and 2007 in their Pediatric Neurology Clinic. Data regarding age, gender, headache type, frequency, and disability, along with height and weight were collected. Body mass index was calculated, and percentiles were determined for age and sex. Headache type and features were compared among normal weight, at risk for overweight, and overweight children. RESULTS: A higher prevalence (39.8%) of obesity was found in our study group compared with the general population. The diagnosis of migraine, but not of tension-type headache, was significantly associated with being at risk for overweight (odds ratio [OR] = 2.37, 95% confidence interval 1.21-4.67, P = .01) or overweight (OR = 2.29, 95% confidence interval 0.95-5.56, P = .04). A significant independent risk for overweight was present in females with migraine (OR = 4.93, 1.46-8.61, P = .006). Regardless of headache type, a high body mass index percentile was associated with increased headache frequency and disability, but not with duration of attack. CONCLUSIONS: Obesity and primary headaches in children are associated. Although obesity seems to be a risk factor for migraine more than for tension-type headache, it is associated with increased headache frequency and disability regardless of headache type.

Headache in children: young age at onset does not imply a harmful etiology or predict a harsh headache disability.

The purpose of this retrospective study was to compare headache etiology, type, and burden and the learning and behavioral profile in children with early-onset (under 6 years) and late-onset (8-12 years) headaches. The study included 133 patients, 35 in the early-onset group and 98 in the late-onset group. Headache diagnosis was based on International Classification of Headache Disorders -II (ICHD-II) criteria. Learning profile and behavioral problems were assessed by parental reports. Tension headache was the most common diagnosis in the early-onset headache group (51.4%). No significant differences were found between the age groups with regard to headache etiology, disability, abnormal neuroimaging results, school performance, or attention problems. Nevertheless, the early-onset group patients had a significantly higher prevalence of behavioral problems: 25.7% versus 11.2% (P < .02). The authors suggest that early age of headache onset does not imply a harmful etiology or a relentless headache disability or burden.

Diagnostic value of Hoover sign and motor-evoked potentials in acute somatoform unilateral weakness and sensory impairment mimicking vascular stroke.

Acute unilateral weakness along with sensory impairment is commonly caused by obstruction of major cortical arteries in either adults or children. A somatoform presentation mimicking acute vascular stroke is very rare, especially in the pediatric age group. Here we report three adolescents presenting with acute unilateral weakness and sensory impairment along with diminished tendon reflexes who were suspected to have an acute stroke but who had developed a somatoform psychogenic disorder. Two adolescents had complete hemiplegia and one had weakness of the left leg - two had moved the alleged paralytic limbs during sleep. A normal Hoover sign was suggestive of a somatoform psychogenic etiology rather than true vascular stroke. Cortical and spinal MRI, motor-evoked potentials (MEP) and somatosensory-evoked potentials were normal. All adolescents recovered completely. Therefore, a somatoform conversion reaction should be considered in children presenting with acute unilateral weakness and sensory alterations, which is corroborated by a normal Hoover sign and intact MEP.

Reactivation of varicella presenting as pseudotumor cerebri: three cases and a review of the literature.

The possible association of pseudotumor cerebri and varicella infection was previously mentioned in a few case reports. In those cases, the history and clinical features of active varicella were obvious, and signs were directly related to the varicella infection. We describe three immunocompetent children with pseudotumor cerebri as the only manifestation of Varicella zoster virus reactivation, with a review of the literature. We suggest considering Varicella zoster virus in children with pseudotumor cerebri, even in the absence of a history of recent varicella infection.

Impaired social behavior in children with benign childhood epilepsy with centrotemporal spikes.

The aim of the present study was to assess the emotional and cognitive aspects of social cognition among patients with rolandic epilepsy. A computerized neuropsychological battery was used for cognitive evaluation. Affective and cognitive social cognition were evaluated using two computerized Theory of Mind tasks. Cognitive abilities and social behavior of 15 children, diagnosed with rolandic epilepsy, ages 7 to 13 years were assessed and compared with 15 age- and education-matched healthy controls. Compared with controls, the epileptic patients had lower scores on verbal and visual learning rate parameters and on verbal processing and were significantly impaired on "affective Theory of Mind" tasks but not on "cognitive Theory of Mind" conditions. Our findings raise the possibility that rolandic epilepsy may affect neural networks affecting cognition and mediating social cognition essential for social behavior, thus challenging the benign nature of the condition.

[Acute hemiplegia and hemianesthesia together with decreased tendon reflexes mimicking acute stroke representing a conversion disorder].

Acute hemiplegia and hemianesthesia is commonly caused by obstruction of major cortical arteries. Such a presentation secondary to a conversion reaction is very rare, especially in the pediatric age group. The authors report an adolescent presenting with acute complete left-sided hemiplegia and sensory loss together with decreased tendon reflexes mimicking an acute arterial stroke. Examination revealed Hoover's sign was present and the patient was oblivious to his stern neurological state. Movement of his paralytic limbs was observed during sleep. Cortical and spinal CT, cortical MRI, motor and somatosensory evoked potentials and a PET study were all normal. As such, the diagnosis of psychogenic hemiplegia was established, apparently within a period that the patient had experienced severe emotional stress while questioning his gender identity. After three days, the adolescent began to move the paralytic limbs along gradual resolution of sensory deficit, leading to complete clinical recovering within two months. Although extremely rare, a conversion reaction should be taken into account in children presenting with acute hemiplegia and anaesthesia, even accompanied with decreased tendon reflexes, when the patient is oblivious to his alleged grave state, and when clinical observations such as Hoover's sign remain intact, substantiated by normal extensive radiological and neurophysiological investigation. Intact motor evoked potentials serve as a key for the diagnosis of psychogenic hemiplegia and, should therefore be performed in suspected cases.

Acute transient deafness representing a negative epileptic phenomenon.

We report herein 2 children who presented with acute deafness heralding an epileptic event manifesting thereafter by loss of consciousness and tonic generalized posturing, possibly reflecting a negative epileptic phenomenon. The first previously healthy male had 2 paroxysmal episodes 7 months apart, starting with acute deafness lasting for a few minutes followed by loss of consciousness and generalized tonic posturing for 10 minutes. Electroencephalography (EEG) during the second episodes demonstrated generalized epileptiform discharges. The second with previously controlled partial complex seizures presented with episodes of complete deafness lasting for a few minutes followed by loss of consciousness and focal tonic posturing lasting 10 minutes. Such acute deafness represented an aura of a focal seizure substantiated by right focal temporal epileptic discharges within the region of the primary auditory cortex. Therefore, EEG should be performed in any case of acute transient deafness, even in the absence of accompanying overt clinical seizures.

The complementary value of sleep-deprived EEG in childhood onset epilepsy.

BACKGROUND: Although EEG is an important diagnostic tool in suspected childhood onset epilepsy, as many as 50% of wakefulness records remain normal. Sleep-deprived EEG has been reported in adults to serve as an activator of epileptic discharges but such effect is still not agreed upon in children reporting small series. PURPOSE: Assess the complementary diagnostic value of sleep deprivation on the induction of epileptic discharges in childhood onset epilepsy having a normal awake record within a period of 5 years. EEG recording was performed during the awake, drowsiness and sleep states following sleep deprivation of 6h. BACKGROUND RESULTS: Fifty five children of whom the initial record failed to detect epileptiform discharges, were assessed at age 5-17 years (mean: 10+/-3.7), 27 boys and 28 girls. Sleep occurred in 51 (92.7%) after sleep deprivation and in only 1 (1.8%) during an awake record. Epileptic discharges were detected in 15 of 55 (27.2%) previous non-epileptic awake records during the sleep-deprived EEG either during wakefulness and more frequent during sleep. Eight abnormal records were detected in 18 (44%) children presenting with a focal seizure and 7 of 35 (20%) associated with generalized seizures. Epileptic discharges were recorded mainly and more frequent during sleep. CONCLUSIONS: Our data suggests that sleep deprivation imposes an apparent activating impact uncovering epileptic discharges children corroborating with overt clinical seizures even beyond the sampling effect of repeat records.

[Identical, for better and for worse].

We report on identical premature twins (monochorionic diamniotic). As fetuses, they both demonstrated sonographic and MRI evidence of cerebral bilateral ventriculomegaly. Neonatal brain US showed bilateral ventriculomegaly, similar in both twins. During follow-up, these physical and imaging similarities persisted. To the best of our knowledge, this is the first report on similar bilateral ventriculomegaly in Identical Twins (IT). A genetic origin of this finding in our IT is suggested. Should more cases of ventriculomegaly be reported in the future in several sets of IT, cerebral ventriculomegaly can then be added to the list of similar CNS features observed in identical twins.

Kindergarten children's failure to qualify for first grade could result from sleep disturbances.

Every year, 7% to 15% of preschool children are found to be underqualified for first grade. We examined whether sleep disturbances are factors in school readiness and their association with neurocognitive skills and behavior. The population included 148 kindergarten students. The study group consisted of 50 students who were assessed by the educational authority as unready for first grade. Children who were scheduled to attend first grade (n = 98) were in the control group. All children/parents filled in a sleep questionnaire and underwent a week of actigraphic sleep/wake study as well as cognitive and behavioral assessments. Children in the study group had significantly shorter total sleep time, reduced sleep efficiency, and increased number of nighttime awakenings. There were significant correlations between sleep variables, and cognitive and behavioral scores. In conclusion, children who fail to qualify for first grade have significantly inferior sleep patterns. Sleep disturbances were associated with cognitive and emotional immaturity.

Sleep disturbances are associated with reduced school achievements in first-grade pupils.

Several previous studies have demonstrated deteriorated scholastic achievements in school-aged children who experience sleep disturbances. In the current study, we examined the association between sleep disturbances during kindergarten (fifth to sixth year of children), and behavior, neurocognitive skills, as well as the children's future academic performance in first grade. The population included 98 kindergarten students who were scheduled to attend first grade. All children/parents filled out a sleep questionnaire and underwent one week of actigraphic sleep/wake study, as well as cognitive and behavioral assessments. Toward the end of first grade achievement evaluation in reading, writing, and arithmetic as well as teachers' evaluations were administrated. Of the 98 pupils, 6 failed the end of first grade's achievement tests (6.1%). When looking at their sleep patterns a year earlier, they had significantly longer sleep latencies (41 +/- 14 vs. 21 +/- 12 min, p < .05), increased arousals from sleep (4.1 +/- 0.9 vs. 1.8 +/- 1.1, p < .01), and lower sleep efficiencies (89.5 +/- 1.1 vs. 94.3 +/- 2.7%, p < .05) than pupils who passed the tests. There were significant correlations between sleep variables and cognitive and behavioral scores. The correlation between sleep efficiency in kindergarten and grades at the end of first year of school was r = .64 (p < .01). Children who failed first grade had significantly inferior sleep patterns. Sleep disturbances were associated with cognitive and emotional immaturity.

Apparent life-threatening events: neurological correlates and the mandatory work-up.

The purpose of this study was to estimate the prevalence of neurological causes for apparent life-threatening events in infants and assess the neurological work-up mandatory to diagnose such diagnosis, hypothesizing that acute neurological disorders constitute the most common etiology for such an acute event. A retrospective chart review allocated 93 infants fulfilling the criteria of the National Institutes of Health presenting at 1 week to 8 months with apnea, color changes, gagging, unresponsiveness, and alterations of body tone. An underlying neurological etiology was detected in 18 (19%), whereby 15 had seizures and 3 had central apnea. Only one electroencephalography demonstrated epileptic discharges, and brain imaging was intact. Thus, it has been concluded that neurological impairment, mainly that of seizures inducing an apparent life-threatening event, are relatively uncommon. As such, recommendations are that history taking and repeat physical examination still remain the major diagnostic tools before resorting to extensive laboratory studies.