Clinico – diagnostic and therapeutic relevance of computed tomography scan of brain in children with partial seizures

Background:

Therapeutic relevance of computed tomography (CT) in children with partial seizures is reported to be remarkably low (1-2%). However, in the developing countries where infections involving the nervous system are common, routine CT scan of brain may help in finding treatable causes of seizures.

Objective:

Aim of this study was to evaluate the significance of CT scan of brain in the management of children with partial seizures.

Materials and Methods:

Children with partial epilepsy, whose predominant seizure type was focal motor seizures, were included in the study. CT scan of brain was done in all children aged between 1 month and 12 years with partial seizures of unknown etiology prospectively. The clinical findings of these children were noted along with the CT findings.

Results:

Between August 2001 and July 2002, of the 200 children with seizure disorder 50 children who satisfied the inclusion criteria were included in the study. CT scan of brain was normal in 16 children (32%) and was abnormal in 34 children (68%). Twenty children (~60% of abnormal scan) had potentially correctable lesions: Tuberculoma (n = 13), neurocysticercosis (n = 3), and brain abscess (n = 4). Five children had changes representing static pathology that did not influence patient management. The clinical features correlated with CT findings in 78% children.

Conclusion:

Children with partial motor seizures have high probability of having abnormal findings on CT scan of brain, especially, neuro-infections which are potentially treatable. Therefore, CT scan brain should be carried out in all children with partial motor seizures especially, in developing countries.     

Late-onset myoclonic epilepsy in Down syndrome (LOMEDS): A spectrum of progressive myoclonic epilepsy — Case report

Cognitive decline and epilepsy are well recognized complication of Down syndrome (DS). Here, we intend to present a case of 28 year old male who presented with progressive mental regression, gait ataxia and myoclonic jerking especially on awakening in morning. His EEG was normal and karyotyping revealed trisomy of chromosome 21. Very few cases had been described in literature of late-onset myoclonic epilepsy in DS. This is first case report from India and our aim is to propose the inclusion of this entity in the spectrum of progressive myoclonic epilepsies but still more cases are yet to be found.     

A tutorial on the use of temporal principal component analysis in developmental ERP research – Opportunities and challenges

Developmental researchers are often interested in event-related potentials (ERPs). Data-analytic approaches based on the observed ERP suffer from major problems such as arbitrary definition of analysis time windows and regions of interest and the observed ERP being a mixture of latent underlying components. Temporal principal component analysis (PCA) can reduce these problems. However, its application in developmental research comes with the unique challenge that the component structure differs between age groups (so-called measurement non-invariance). Separate PCAs for the groups can cope with this challenge. We demonstrate how to make results from separate PCAs accessible for inferential statistics by re-scaling to original units. This tutorial enables readers with a focus on developmental research to conduct a PCA-based ERP analysis of amplitude differences. We explain the benefits of a PCA-based approach, introduce the PCA model and demonstrate its application to a developmental research question using real-data from a child and an adult group (code and data openly available). Finally, we discuss how to cope with typical challenges during the analysis and name potential limitations such as suboptimal decomposition results, data-driven analysis decisions and latency shifts.     

Levels of C-reactive protein (CRP) in elderly patients with unipolar depression – case control analysis

Aim: C-reactive protein (CRP) is the major acute-phase plasma protein. Studies show that patients with depression have elevated levels of CRP. The aim of the study was to determine differences in CRP serum level in elderly patients with unipolar depression (DEP) compared with non-depressed elderly patients (nonDEP) using case-control analysis.

Methods: Serum level of CRP was measured in 404 (DEP: n?=?202, nonDEP: n?=?202) Caucasian inpatients aged ≥60 (350 women, 86.7%; mean age?=?76.7 years).

Results: Mean CRP level in the study groups was: DEP 2.67?±?2.56?mg/dL, nonDEP 2.41?±?2.19?mg/dL, the difference was not significant (p?=?0.96). The overall rate of being above the high level of CRP (set at 3.0?mg/L) was 33.2% for DEP and 29.2% for nonDEP groups (p?=?0.39). It was also found that, in the whole study group, CRP level was not correlated with age (p?=?0.10).

Conclusions: Elderly patients with depression have no increased CRP levels. A high percentage (～30%) of all subjects had a CRP level >3?mg/L, which is the cut-off point for increased cardiovascular risk.     

Detection of fast (40–150 Hz) oscillations from the ictal scalp EEG data of myoclonic seizures in pediatric patients

Objective

We explored fast (40–150?Hz) oscillations (FOs) from the ictal scalp electroencephalogram (EEG) data of myoclonic seizures in pediatric patients to obtain insight into the pathophysiological mechanisms involved in the generation of myoclonic seizures.

Generalized onset seizures with focal evolution (GOFE) — A unique seizure type in the setting of generalized epilepsy

PurposeWe report clinical and electrographic features of generalized onset seizures with focal evolution (GOFE) and present arguments for the inclusion of this seizure type in the seizure classification.MethodsThe adult and pediatric Epilepsy Monitoring Unit databases at Vanderbilt Medical Center and Children's Hospital were screened to identify generalized onset seizures with focal evolution. We reviewed medical records for epilepsy characteristics, epilepsy risk factors, MRI abnormalities, neurologic examination, antiepileptic medications before and after diagnosis, and response to medications. We also reviewed ictal and interictal EEG tracings, as well as video-recorded semiology.ResultsTen patients were identified, 7 males and 3 females. All of the patients developed generalized epilepsy in childhood or adolescence (ages 3–15 years). Generalized onset seizures with focal evolution developed years after onset in 9 patients, with a semiology concerning for focal seizures or nonepileptic events. Ictal discharges had a generalized onset on EEG, described as either generalized spike-and-wave and/or polyspike-and-wave discharges, or generalized fast activity. This electrographic activity then evolved to focal rhythmic activity most commonly localized to one temporal or frontal region; five patients had multiple seizures evolving to focal activity in different regions of both hemispheres. The predominant interictal epileptiform activity included generalized spike-and-wave and/or polyspike-and-wave discharges in all patients. Taking into consideration all clinical and EEG data, six patients were classified with genetic (idiopathic) generalized epilepsy, and four were classified with structural/metabolic (symptomatic) generalized epilepsy. All of the patients had modifications to their medications following discharge, with three becoming seizure-free and five responding with > 50% reduction in seizure frequency.ConclusionGeneralized onset seizures may occasionally have focal evolution with semiology suggestive of focal seizures, leading to a misdiagnosis of focal onset. This unique seizure type may occur with genetic as well as structural/metabolic forms of epilepsy. The identification of this seizure type may help clinicians choose appropriate medications, avoiding narrow spectrum agents known to aggravate generalized onset seizures.     

Training of goal directed arm movements with motion interactive video games in children with cerebral palsy – A kinematic evaluation

Objective: The main aim of this study was to evaluate the quality of goal-directed arm movements in 15 children with cerebral palsy (CP) following four weeks of home-based training with motion interactive video games. A further aim was to investigate the applicability and characteristics of kinematic parameters in a virtual context in comparison to a physical context.

Method: Kinematics and kinetics were captured while the children performed arm movements directed towards both virtual and physical targets.

Results: The children’s movement precision improved, their centre of pressure paths decreased, as did the variability in maximal shoulder angles when reaching for virtual objects. Transfer to a situation with physical targets was mainly indicated by increased movement smoothness.

Conclusion: Training with motion interactive games seems to improve arm motor control in children with CP. The results highlight the importance of considering both the context and the task itself when investigating kinematic parameters.     

Impact of sleep on the localizing value of video EEG in patients with refractory focal seizures – A prospective video-EEG with EOG and submental EMG study

ObjectivesTo examine the role of sleep and its stages on the localizing value of video EEG in the evaluation of refractory focal seizures.MethodsVideo-electroencephalographic (VEEG) evaluation with additional polygraphic recording was carried out for 70 consecutive patients with refractory focal epilepsy, undergoing pre-surgical evaluation, over a two-year period. Localization of video EEG for each seizure was made based on clinical, ictal and interictal data. Seizure localization in each patient was assessed for concordance with MRI and other imaging data (SPECT, PET) for both wake and sleep seizures. Interictal discharges in sleep and wake were similarly compared for concordance with imaging data.ResultsA total of 608 seizures were recorded in 70 patients, 289 in sleep. Overall, concordance with imaging data was found in 218 out of 322 wake seizures (67.8%) and in 157 out of 286 sleep seizures (54.8%) (p = 0.0314). On analyzing the subset of patients with seizures recorded in both wake and sleep states (total 279 seizures recorded, 113 out of sleep), concordance was observed in 93 out of 166 (56%) wake seizures and in 80 out of 113 (70.7%) sleep seizures (OR 2.03, 95% CI 1.17 to 3.56; p 0.007). Interictal discharges were more common and more precisely localizing in sleep, mostly in stage N2.ConclusionsThis prospective VEEG-PSG study demonstrates the role of sleep versus wake state in the localizing value of different components of long-term VEEG recording for patients with medically refractory epilepsy. Our findings show that while wake state ictal EEG has more localizing value in a mixed group of patients, sleep ictal and interictal EEG is significantly more useful in patients who have seizures recorded both during wake and sleep states. In addition, interictal discharges recorded during NREM sleep have high localizing value.SignificanceThis is only the second study elucidating the effect of sleep on the localizing value of video-electroencephalographic evaluation of patients with medically refractory focal epilepsy; mainly revealing high value of sleep interictal discharges and that sleep ictal recording has two times higher localizing value than wake ictal recording, among patients in whom seizures are recorded in both states.     

Bilateral pallidal stimulation in children and adolescents with primary generalized dystonia – Report of six patients and literature-based analysis of predictive outcomes variables

Introduction: Primary generalized dystonia is a rare movement disorder. Medical treatment rarely relieves symptoms. The aim of this study was to investigate the efficacy and safety of bilateral pallidal stimulation in 6 children and adolescents with primary generalized dystonia. In addition, we strived to find predictors for treatment outcome by review and analysis of previously published studies. Methods: Six patients with primary generalized dystonia underwent chronic bilateral stimulation of the globus pallidus internus. A PubMed and MEDLINE search was performed in order to identify children and adolescents who underwent deep brain stimulation for primary generalized dystonia. The primary efficacy endpoint was the relative change of the Burke-Fahn-Marsden-Dystonia-Rating-Scale (movement score) after surgery. Results: Forty-four patients were found to meet the inclusion criteria. The mean age at onset of the disease was 7.8 ± 2.8 years and the mean age at surgery was 14.2 ± 3.5 years. The mean Burke-Fahn-Marsden-Dystonia-Rating-Scale (movement score) was 56.9 ± 22.7 before surgery and 23.7 ± 23.2 at a mean follow up of 13.0 ± 4.8 months (p < 0.001). The improvement in the DYT1-positive group was significantly higher compared to the DYT1-negative group (77% ± 24% and 44% ± 30%, respectively, p < 0.001). A positive correlation between the movement score before and after surgery was found in both the DYT1-positive and DYT1-negative cohort (rs = 0.624, p < 0.001 and rs = 0.734, p < 0.001, respectively). Conclusion: DBS is an effective treatment in children and adolescents with primary generalized dystonia. Predictive factors for a better treatment outcome are DYT1-positive status and minor motor impairment before surgery.     

A case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome with positive anti-galactocerebroside (Gal-C) IgM antibody]

A 49-year-old man presented with hoarseness, dysphagia, muscle atrophy and weakness of deltoid, trapezius, sternocleidomastoid, rhomboid, anterior serratus, infraspinatus and supraspinatus. Anti-Gal-C IgM antibody was positive in the serum. The other antiganglioside antibodies (GM1, GM2, GM3, GD1a, GD1b, GD3, GT1a, GT1b, GQ1b, GA1, GalNAc-GD1a, GM1b) were negative. Patient contracted pneumonia but whether it was due to mycoplasma was not evident. Plasmapheresis improved his clinical state including a decrease of the antibody. This case was diagnosed pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, and anti-Gal-C antibody seemed to be correlated with the pathogenesis of this syndrome. Gal-C is a major glycolipid of myelin and the cell membrane of the myelin-forming cell (oligodendrocytes and Schwann cells) and is free of specific localization and distribution. The mechanism how the anti-Gal-C IgM antibody induced bulbar paralysis and the symptoms localizing neck and upper limbs remains to be known.     

Illuminating patients with children up to 18 years of age – A 1-day-inventory study in a psychiatric service

This study reports the possibility of accomplishing a survey in an ordinary psychiatric service organization to identify the children and the adolescents in families with a parent with mental illness and to highlight the number of patients in psychiatric treatment with little or virtually no contact with their minor children. The prevalence of patients being parents to minor children was 36% in the total sample consisting of 137 patients, from both inpatient and outpatient services, participating in the survey. Three of four patients were living together with the children. A higher proportion of patients in the outpatient unit were parents to minor children, and more often lived together with them. There were no differences in prevalence of patients with minor children according to sex or diagnostic subgroup. However, female patients more often and patients with a psychosis diagnosis more seldom had the custody of the children. A majority of the patients had communicated with their children about their own situation, showing that psychiatric patients care a lot about the situation of their children, although, according to the patients, the psychiatric services only take an active part in this information in a minority of the cases. The study may be found to be a basis for inspiring structured interventions and treatments programmes, including the minor children of the adult patients seeking psychiatric treatment. Prevention of mental disorders in the oncoming generation is an important issue for all psychiatric professionals, especially in co-operation between adult and child/adolescent psychiatric services.     

The neural correlates of gait improvement by rhythmic sound stimulation in adults with Parkinson's disease – A functional magnetic resonance imaging study

IntroductionAdults with Parkinson's disease (PD) experience gait disturbances that can sometimes be improved with rhythmic auditory stimulation (RAS); however, the underlying physiological mechanism for this improvement is not well understood. We investigated brain activation patterns in adults with PD and healthy controls (HC) using functional magnetic resonance imaging (fMRI) while participants imagined gait with or without RAS.MethodsTwenty-seven adults with PD who could walk independently and walked more smoothly with rhythmic auditory cueing than without it, and 25 age-matched HC participated in this study. Participants imagined gait in the presence of RAS or white noise (WN) during fMRI.ResultsIn the PD group, gait imagery with RAS activated cortical motor areas, including supplementary motor areas and the cerebellum, while gait imagery with WN additionally recruited the left parietal operculum. In HC, the induced activation was limited to cortical motor areas and the cerebellum for both the RAS and WN conditions. Within- and between-group analyses demonstrated that RAS reduced the activity of the left parietal operculum in the PD group but not in the HC group (condition-by-group interaction by repeated measures analysis of variance, p < 0.05).ConclusionDuring gait imagery in adults with PD, the left parietal operculum was less activated by RAS than by WN, while no change was observed in HC, suggesting that rhythmic auditory stimulation may support the sensory-motor networks involved in gait, thus alleviating the overload of the parietal operculum and compensating for its dysfunction in these patients.     

The diagnostic utility of cerebrospinal fluid alpha-synuclein analysis in dementia with Lewy bodies – A systematic review and meta-analysis

BackgroundDementia with Lewy Bodies (DLB) can be difficult to distinguish clinically from other dementias.ObjectiveTo investigate the diagnostic utility of CSF alpha-synuclein in differentiating between DLB and other dementias.MethodsElectronic databases were systematically searched for studies investigating reproducible alpha synuclein quantification methods. Random effects model was used to calculate weighted mean difference (WMD) and 95% confidence intervals between DLB and other groups.ResultsA total of 13 studies, comprising 2728 patients were included. Mean CSF alpha-synuclein concentration was significantly lower in DLB patients compared to those with Alzheimers disease (AD) [WMD ?0.24; 95% CI, ?0.45, ?0.03; p = 0.02]. No significant difference was found between patients with DLB compared to Parkinsons disease [WMD 0.05; 95% CI, ?0.17, 0.28; p = 0.65] or other neurodegenerative conditions.ConclusionCSF alpha synuclein may be of diagnostic use in differentiating between DLB and AD. We propose several recommendations to guide better design of future studies.     

Non‐word repetition in Dutch children with (a risk of) dyslexia and SLI

It has been proposed that poor non‐word repetition is a marker of specific language impairment (SLI), and a precursor and marker of dyslexia. This study investigated whether a non‐word repetition deficit underlies both disorders. A group of Dutch preschool SLI children and children at familial risk of dyslexia, as well as school‐going groups of SLI and dyslexic children were presented with a non‐word repetition task. The results showed that the SLI and the (at‐risk of) dyslexia groups performed more poorly than the control children. Furthermore, with the exception of one child, all preschool SLI children scored significantly below the mean of the preschool control group, suggesting that non‐word repetition performance is a marker of SLI. Approximately half of the at‐risk group were poor performers, which was expected on the basis of the familial risk factor of the at‐risk group. The results show that a non‐word repetition deficit is attested early in life and underlies both dyslexia and SLI.     

The German version of the revised Adolescent Sleep-Wake Scale (rASWS) – A validation study in pediatric pain patients and school children

ObjectiveSleep problems are common in children and adolescents with chronic pain. The revised Adolescent Sleep-Wake Scale (rASWS) is an internationally well-established instrument to assess sleep quality in adolescents. So far, no German version is available. The study aimed to provide a validated German version of the rASWS, specifically for use in children and adolescents with chronic pain.MethodsThe translated questionnaire was validated in a sample of N = 159 pediatric outpatients with chronic pain (8–17 years; 65.4% female), who presented to a specialized pediatric pain center. For cross-validation a community sample of N = 1348 school children was analyzed.ResultsConfirmatory factor analysis was conducted to examine the factor structure of the original 10-item 3-factor model in the sample of children and adolescents with chronic pain, which showed poor model fit. Model modifications were carried out by deleting 3 items with low factor loadings stepwise. The overall model fit of the final 3-factor model containing 7 items was excellent. Cronbach's α of the derived scales ranged from 0.74 to 0.86. Cross-validation in a community sample of school children confirmed the superiority of the 7-item model. The convergent validity of the measure was proved by moderate correlations between the rASWS and self-reported sleep problems. Associations with chronic pain characteristics were evident for pain-related disability.ConclusionsThe use of the 7-item version of the rASWS for German-speaking children and adolescents with and without chronic pain is recommended as a self-report measure of sleep quality.     

A case of Gerstmann-Str?ussler-Scheinker syndrome (GSS) with late onset--a haplotype analysis of Glu219Lys polymorphism in PrP gene]

We report a 74-year-old man with late onset Gerstmann-Str?ussler-Scheinker syndrome (GSS). In this family, 3 out of 6 siblings and his father developed cerebellar ataxia and mental deterioration in their fifth decades. He complained of unsteady walking and tingling pain in the legs at the age of 70. Neurological examination revealed moderate truncal ataxia, mild limb ataxia, ataxic speech, sensory impairment, paresthesia and areflexia in the lower extremities. CSF examination showed elevated CSF and 14-3-3 proteins with a normal cell count. EEG and brain MRI demonstrated no abnormality. Somatosensory evoked potential (SEP) study showed delayed N13-N20 interpeak latencies in the upper extremities and delayed N20 at 12th thoracic spinous process, indicating dysfunction of the posterior roots or columns of the spinal cord including the dorsal horns and proximal peripheral nerve. Analysis of the prion protein gene demonstrated a Pro102Leu amino acid substitution, which is compatible with classical GSS. Haplotype analysis of the PrP gene identified a Glu219Lys polymorphism on another allele. Recently, it was confirmed that protein X, which accelerates the conversion of the normal type of PrP (PrPC) into a pathological type of PrP (PrPSc), binds to the 219th amino acid residue of PrP. Therefore, the 219Lys polymorphism theoretically inhibited formation of PrPSc and may thus have delayed the onset of the disease in this patient.     

The impact of attention deficit/hyperactivity disorder on adaptive functioning in children diagnosed late with autism spectrum disorder—A comparative analysis

This study investigated the relationship between ASD, ADHD and adaptive behavior in children aged 7–17 years at the time of their first ASD diagnosis. Results showed that 68.1% of the participants had a clinical diagnosis of ADHD in addition to ASD. A hypothesis of an additive negative effect of ADHD on adaptive behavior of children with ASD was partly supported. When controlling for age, gender, IQ, and autistic symptoms, communication was the only adaptive behavior domain that remained significant. Further analyses of the data showed that this effect was limited to high functioning boys (IQ ≥ 80). The reasons why ADHD did not impinge on the adaptive behavior of low functioning boys and low and high functioning girls are discussed.