Multicenter retrospective study of patients with PCDH19-related epilepsy: The first Hungarian cohort.

OBJECTIVE: PCDH19-related epilepsy occurs predominantly in girls and is caused by pathogenic variant of the protocadherin-19 gene. The initial seizures usually develop in association with fever, begin on average at 15 months of age, and often occur in clusters. Autistic symptoms, intellectual disability, and sleep disturbance are often associated. METHODS: In our retrospective, multicenter study, we reviewed clinical data of nine children with epilepsy genetically confirmed to be associated with PCDH19. RESULTS: In the Hungarian patient population aged 0-18 years, the prevalence of PCDH19-related epilepsy was found to be lower (1/100000 live births in females) than the reported international data (4-5/100000 live births in females). Four of our nine patients had positive family history of epilepsy (cousins, sister, and mother). We assessed brain anomalies in three patients (in one patient focal cortical dysplasia and left anterior cingulate dysgenesis, and in two children right or left hippocampal sclerosis) and in another three cases incidentally identified benign alterations on brain MRI were found. The first seizure presented as a cluster in seven out of nine children. In seven out of nine cases occurred status epilepticus. Six out of nine children had autistic symptoms and only one child had normal intellectual development. Seven of our patients were seizure free with combined antiseizure medication (ASM). The most effective ASMs were levetiracetam, valproate, and clobazam. SIGNIFICANCE: The prevalence of PCDH19-related epilepsy is presumably underestimated because of the lack of widely performed molecular genetic evaluations. Molecular genetic testing including PCDH19 pathogenic variants is recommended for female patients with an onset of seizures before the age of 3 years.

Perspectives of cerebral palsy experts on access to health care in Europe.

AIM: To explore the perspectives of cerebral palsy (CP) experts on access to healthcare and an analysis of socioeconomic and environmental determinants impacting young individuals with CP in Europe. METHOD: Cross-sectional survey designed by a convenience multi-disciplinary panel of invited experts and completed by clinicians, researchers and opinions leaders in the field of CP. RESULTS: Fifty-eight experts (response rate 85 %) from 39 regions in 26 European countries completed the survey. All countries provide care and financing through public systems. Long waiting lists were reported (mean 3 mo, range 1-12 mo), depending on type of specialist care and place of residence. Although diagnostic and therapeutic services were available, access within countries/regions were unevenly distributed, with children receiving better care than adults. Most experts reported a lack of transition services, although improvement is expected (62 % of responses). Hip and malnutrition surveillance, as well as educational and recreational activities were variably available. Public transportation, accessible roads and pavements, and urban green spaces for persons with disabilities were more available in larger cities. Overall, only 57 % of responders felt that most patients had adequate access to healthcare. CONCLUSION: The survey of CP experts' perspectives from the majority of European countries indicates discrepancies in the availability and accessibility of healthcare needed by people with CP and nonuniform implementation of policies across Europe.

Has the Incidence of Febrile Convulsions in Childhood Changed During the SARS-CoV-2 Pandemic?

Introduction: SARS-CoV-2 infection in children is usually asymptomatic or only mild symptoms are typical. The aim of our study was to assess the incidence of febrile convulsions in our own patients with COVID-19. Patients and Methods: In our retrospective study, we reviewed the data of children who presented at our University Hospital from March 2020 to March 2022 with febrile convulsion. The control group were children admitted to the hospital because of febrile convulsions from January 2018 to January 2020. Results: During the coronavirus pandemic, 51 patients were examined with febrile convulsions. The majority (86.3%) of children had their first febrile convulsion during this period. We diagnosed simple febrile convulsions in 40 cases and complicated ones in 11 cases. The family history of febrile convulsion or epilepsy was present in 12 (23.5%) patients. In addition to febrile convulsion, SARS-CoV-2 infection was confirmed by laboratory testing in 4 cases (7.8%). Three of them had febrile convulsion during the Omicron variant period. Conclusions: During the coronavirus pandemic, the number of children examined because of having febrile convulsions was not higher than in the control period. The coronavirus is unlikely to increase the risk of febrile convulsions.

Classification of events contributing to postneonatal cerebral palsy: Development, reliability, and recommendations for use.

AIM: This paper introduces the Surveillance of Cerebral Palsy in Europe (SCPE) classification of events contributing to postneonatally acquired cerebral palsy, presents its interrater reliability, and describes the cases identified in the SCPE database. METHOD: The development of the classification, based on literature review and expert discussions, resulted in six main categories and 19 subcategories. The first chronological event designated as the primary event was mainly reported. Interrater reliability was assessed through online exercise providing 24 clinical vignettes representing single/complex pathways. Percent agreement and Gwet's AC1 index of reliability were estimated. Primary events were described using data of 221 children born between 2008 and 2012. RESULTS: Thirty-nine professionals (21 registries) participated in the reliability exercise. Substantial overall agreement was reached (0.75), with some contrast between complex (0.48, moderate agreement) and single events involved (0.89, almost perfect). The distribution of primary events showed that 32.1% were infections (category A), 23.1% head injuries (B), 15.4% related to surgery or medical interventions (C), 13.1% cerebrovascular accidents (D), 9.1% hypoxic brain damaging events of other origins (E), and 7.2% miscellaneous (F). INTERPRETATION: This classification allows all the events involved to be recorded while consistently reporting the primary event, and may be used in different settings. WHAT THIS PAPER ADDS: A standardized classification enables the description of the events contributing to postneonatal cerebral palsy (CP). The first chronological event in complex pathway leading to CP is coded. Category choice and coding of the primary event identify preventable situations. The detailed 2-level classification is easy to use in various settings. Substantial overall interrater reliability shows that main categories can be consistently differentiated.

Prevalence, Clinical Features, Neuroimaging, and Genetic Findings in Children With Ataxic Cerebral Palsy in Europe.

BACKGROUND AND OBJECTIVES: To report on prevalence, associated impairments, severity, and neuroimaging findings in children with ataxic cerebral palsy (CP). METHODS: In children coded as having ataxic CP in the Central database of Joint Research Center-Surveillance of Cerebral Palsy in Europe (JRC-SCPE) and born during 1980-2010, birth characteristics, severity profiles including associated impairments, neuroimaging patterns, and the presence of syndromes were analyzed. Definitions were according to validated SCPE guidelines. Prevalence over time was estimated using Poisson regression. RESULTS: In total, 679 children with ataxic CP were identified in 20 European CP registers. The proportion with ataxic CP was 3.8% and varied from 0% to 12.9%. Prevalence over time showed no significant trend. Approximately 70% of children with ataxic CP were able to walk, and 40% had severe intellectual impairment and a high impairment index. Children with ataxic CP were mostly born at term (79%) and with normal birth weight (77%). Neuroimaging patterns revealed normal findings in 29%, brain maldevelopments in 28.5%, miscellaneous findings in 23.5%, and brain injuries in 19%, according to the SCPE classification. Genetic syndromes were described in 9%. DISCUSSION: This register-based multicenter study on children with ataxic CP provides a large sample size for the analysis of prevalence, severity, and origin of this rare CP subtype. Even with strict inclusion and classification criteria, there is variation between registers on how to deal with this subtype, and diagnosis of ataxic CP remains a challenge. Ataxic cerebral palsy differs from other CP subtypes: children with ataxic CP have a disability profile that is more pronounced in terms of cognitive than gross motor dysfunction. They are mostly term born and the origin rarely suggests acquired injuries. In addition to neuroimaging, a comprehensive genetic workup is particularly recommended for children with this CP type.

Total sleep deprivation decreases saliva ghrelin levels in adolescents.

Ghrelin, a regulator of food intake and energy expenditure, has been shown to be associated with insufficient sleep. The goal of the present study was to investigate the effect of a single night of total sleep deprivation on fasting saliva ghrelin and on nocturnal variation of saliva ghrelin concentration. A further aim of the study was to investigate the influence of body mass index on changes in saliva ghrelin levels. Altogether 35 adolescents (18 boys; age: 13.8 ± 1.14 years) were studied on two subsequent days (sleep and total sleep deprivation). Saliva samples were collected during the two experimental nights at 21:00 hours, 01:00 hours and 06:00 hours. Total-ghrelin concentration showed a continuous increase from the evening until 06:00 hours. This increase was blunted significantly (p = 0.003) by total sleep deprivation. Total-ghrelin level was significantly lower (p = 0.02) during total sleep deprivation at 06:00 hours (median 403.6 pg ml-1 ; 95% confidence interval: 343.1-468.9 pg ml-1 ) as compared with values during the sleep condition (median 471.2 pg ml-1 ; 95% confidence interval: 205.4-1578.7 pg ml-1 ). Acyl-ghrelin levels did not present any change at the three time points, and were not affected by total sleep deprivation. Stratifying the study population according to body mass index (normal weight and overweight/obese groups), the blunting effect of total sleep deprivation was more pronounced in the obese/overweight group (sleep: median 428.2 pg ml-1 ; 95% confidence interval: 331.3-606.9 pg ml-1 versus total sleep deprivation: median 333.1 pg ml-1 ; 95% confidence interval: 261.5-412.9 pg ml-1 ; p = 0.0479). Saliva total-ghrelin concentrations gradually increased during the night, and total sleep deprivation significantly blunted this increase. This blunting effect was mainly observed in subjects with overweight/obesity. The physiological and clinical implications of the present observation are to be clarified by further studies.

Trends in Prevalence and Severity of Pre/Perinatal Cerebral Palsy Among Children Born Preterm From 2004 to 2010: A SCPE Collaboration Study.

Aim: To report on prevalence of cerebral palsy (CP), severity rates, and types of brain lesions in children born preterm 2004 to 2010 by gestational age groups. Methods: Data from 12 population-based registries of the Surveillance of Cerebral Palsy in Europe network were used. Children with CP were eligible if they were born preterm (<37 weeks of gestational age) between 2004 and 2010, and were at least 4 years at time of registration. Severity was assessed using the impairment index. The findings of postnatal brain imaging were classified according to the predominant pathogenic pattern. Prevalences were estimated per 1,000 live births with exact 95% confidence intervals within each stratum of gestational age: ≤27, 28-31, 32-36 weeks. Time trends of both overall prevalence and prevalence of severe CP were investigated using multilevel negative binomial regression models. Results: The sample comprised 2,273 children. 25.8% were born from multiple pregnancies. About 2-thirds had a bilateral spastic CP. 43.5% of children born ≤27 weeks had a high impairment index compared to 37.0 and 38.5% in the two other groups. Overall prevalence significantly decreased (incidence rate ratio per year: 0.96 [0.92-1.00[) in children born 32-36 weeks. We showed a decrease until 2009 for children born 28-31 weeks but an increase in 2010 again, and a steady prevalence (incidence rate ratio per year = 0.97 [0.92-1.02] for those born ≤27 weeks. The prevalence of the most severely affected children with CP revealed a similar but not significant trend to the overall prevalence in the corresponding GA groups. Predominant white matter injuries were more frequent in children born <32 weeks: 81.5% (≤27 weeks) and 86.4% (28-31 weeks), compared to 63.6% for children born 32-36 weeks. Conclusion: Prevalence of CP in preterm born children continues to decrease in Europe excepting the extremely immature children, with the most severely affected children showing a similar trend.

The impact of disease and sociodemographic background on children suffering from cerebral palsy / A betegség és a szociodemográfiai háttér hatása a cerebralis bénulásban szenvedo gyermekek életminoségére.

Összefoglaló. Bevezetés: A kutatócsoport 99 fo, cerebralis paresisben (CP) szenvedo gyermek (8-18 éves) önállóan közölt életminoségét értékelte, és az eredményeket összehasonlította egy 237 fos kontrollcsoport adataival, amelyek hasonló életkorú, egészséges általános populációhoz tartozó gyermekektol származtak. Célkituzés: A kutatás célja annak megismerése volt, hogy a CP-ben szenvedo gyermekek hogyan vélekednek egészségi állapotukról és társadalmi helyzetükrol. Módszer: Életminoség-kérdoív alkalmazása. Betegségspecifikus és társadalmi-demográfiai mutatók mérése, kiértékelése. Eredmények: Az CP-ben szenvedo gyermekek és szüleik az egészséggel kapcsolatos életminoséget rosszabbnak ítélték meg, mint társaik. Eredményeink azt mutatják, hogy a noi nem, a rosszabb motoros funkció és a komorbiditások (epilepszia, incontinentia és intellektuális károsodás) negatív hatású. A szüloi vélemény alkalmas volt proxyjelentésként a korreláció mért erossége miatt. Figyelemre méltó, hogy az agyi bénulás típusai közül az egyoldali spasticus CP-ben szenvedo gyermekek életminoség-értéke a legalacsonyabb. A válaszadók valószínuleg a test két oldala között lévo funkcionális különbséget érezték. A szellemi fogyatékosság a betegpopuláció több mint felénél fordult elo. Testvéreik között a mentális betegség 5,7-szer gyakoribb. A CP-s gyerekek családi környezete sokkal hátrányosabb volt, mint az egészséges gyermekeké. A kutatás eredményei alapján megállapítható, hogy a szülo alacsonyabb iskolai végzettsége és munkaeropiaci inaktivitása, valamint az egyszülos család a CP-s gyerekeknél szignifikánsan magasabb arányban fordult elo, és ezek a tényezok negatív hatást gyakoroltak az életminoségre. Következtetés: A fogyatékkal élo gyermekek életminoségét a betegség és a szociodemográfiai környezet egyaránt befolyásolja. Orv Hetil. 2021; 162(7): 269-279. INTRODUCTION: Self-reported health-related quality of life (HRQoL) of 99 children (8-18 years) with cerebral palsy (CP) was assessed and compared with 237, age-matched healthy control children from the general population. OBJECTIVE: The aim was to find out the opinions of children with CP about their health status and social condition. METHOD: Assessment of quality of life questionnaire was carried out. Measurements of disease-specific and sociodemographic variables were done. RESULTS: Children with CP and their parents rated HRQoL poorer than their counterparts. Our results show that female sex, worse gross motor function and comorbidities (epilepsy, incontinence and intellectual impairment) had negative impact. The parental opinion was suitable as proxy report because of the measured strength of the correlation. Among the types of CP, interestingly, children with unilateral spastic CP had the poorest HRQoL. They were likely to feel a functional difference between the two sides of the body. Intellectual disability occurred in more than half of our patient population. Among their siblings, mental illness is 5.7 times more common. The family environment was much more disadvantageous than in the case of healthy children. As our study shows, lower education, inactive status in the labour market and single-parent family occurred at a much higher rate and worsened the quality of life. CONCLUSION: Quality of life of children with disability was influenced by both the sociodemographic background and the disease. Orv Hetil. 2021; 162(7): 269-279.

The training and organization of Paediatric Neurology in Europe: Special report of the European Paediatric Neurology Society & Committee of National Advisors.

BACKGROUND: Paediatric Neurology (PN) is a discipline focused on diagnosis, comprehensive management and research into diseases of the central and peripheral nervous system from fetal life to transition into adulthood. The European Paediatric Neurology Society first designed and published the European PN training programme in the European Paediatric Neurology Syllabus in 2002. This was important in gaining recognition for the sub-specialty from the European Academy of Paediatrics and the European Academy of Neurology and in 2003 PN was recognized as a sub-specialty of paediatrics and neurology by the Board of the European Union of Medical Specialties. In 2004, the EPNS founded the Committee of National Advisors (CNA) that comprised representatives from national Paediatric Neurology societies, in order to further enhance Europe wide standards in training and practice., The EPNS Training Advisory Board (TAB) offers nation specific advice/support to PN societies on developing training and care systems. In 2019, the 2nd revision of the Paediatric Neurology Syllabus was approved by the EPNS Board and CNA. We aim to give an overview of the training of Paediatric Neurology (PN) specialists (i.e. Paediatric Neurologists), the relevant professional bodies and the current practice of Paediatric Neurology in Europe, as defined geographically by the World Health Organization. METHODS: A structured online data collection form was completed by CNA representatives from European countries. The data included training routes and structure of training, epidemiological data, nature of professional societies, organization of Paediatric Neurology care, research, academic life and recognition of the specialty. RESULTS: Data was collected from 43 European countries of which 38 have a national PN Society. In 10 (6 European Union (EU) and 4 non-EU countries) PN is recognized as a core specialty. In 26 countries PN is recognized as a sub-specialty of Paediatrics, Neurology or both (15 EU-11 non-EU). PN is not recognized as a core or sub-specialty in 7 countries (4 EU and 3 non-EU). In 35 countries paediatric neurologists begin their training from Paediatrics, but in 19 countries PN training from Neurology is also possible or the preferred route. Training in PN differs, but in over 50% of countries the three main training modules named in the 2019 2nd revision of the European PN Syllabus (PN, Paediatrics and adult Neurology) are included. Many countries have already adapted their curriculum to the suggestions in the European PN syllabus. CONCLUSIONS: There is diversity among European countries in terms of professional organization and PN training. The European PN syllabus has had impact on the development of PN training throughout Europe, independent of duration of training or route from paediatrics or neurology. The syllabus provides a basis for the future development of PN training, the recognition of PN as a (sub) specialty in individual countries and for improving the care of children with neurological disorders in Europe.

The usefulness of MRI Classification System (MRICS) in a cerebral palsy cohort.

AIM: Our aim was to investigate perinatal and clinical factors associated with children with cerebral palsy (CP) using magnetic resonance imaging (MRI). The distribution of MRI patterns was based on the MRI classification system (MRICS). Associations between perinatal/clinical characteristics and MRI patterns were also investigated. METHODS: A population-based cohort study was performed; those 257 children (58.0% male) were enrolled from our CP database who born between 1990 and 2015 in Southwest Hungary and had at least one MRI scan. RESULTS: Brain maldevelopments were found in 18.7% of our patients, 83.7% of those born at term. Grey matter lesions were found in 19.8% of our patients, and 80.0% of those children were born at term. The rate of white matter injuries was the highest (35.4%); 69.0% of these patients were born before 37th week of gestation. MRI revealed no abnormalities in 13.6% of children with CP. The best values of gross/fine motor and cognitive function tests were found in children with normal MRI and with grey matter injuries. The prevalence of epilepsy was above 60% in every group with an abnormal MRI. CONCLUSION: MRI results were conclusive in 86.4% of children with CP. It is highly encouraged to perform cranial MRI in every patient with CP.

Does Co-occurred Cerebral Palsy Change the Prognosis of West Syndrome?

AIM: We aimed to examine the occurrence of cerebral palsy (CP) in children with West syndrome (WS), to estimate the possible causative factors by analyzing the neuroimaging examinations of patients, to evaluate their cognitive/motor function and epileptic status and to compare the prognosis of children with double pathology of WS and CP and of those without CP. METHODS: The clinical and magnetic resonance imaging (MRI) data of 62 patients with West syndrome were evaluated. A total of 39 of 62 patients (63%) suffered from CP (CP group). The non-CP group included 23 patients. RESULTS: Abnormal MRI was found in 55/62 (89%) patients. Main anomalies were: brain malformation (21), hypoxic-ischemic encephalopathy (13), cerebrovascular insult (8), infection (7), and other anomalies (6). In the CP group, the most common MRI abnormalities included pre/perinatal hypoxia/ischemia, brain malformation, cerebrovascular insult, and infection. In the non-CP group, brain malformations were the most frequent. Significantly more negative MRIs were found in the non-CP group. More than 60% of the patients were severely cognitively impaired, almost 90% of them had CP. Not only the occurrence of intellectual disability was lower in the non-CP group, but its severity was milder as well. A total of 78% of the children with CP had a very severe motor disability. Fifty-four percent in the CP and 67% in the non-CP group had therapy-resistant epilepsy. CONCLUSION: WS has an especially unfavorable prognosis: cerebral anomaly was confirmed in 89% of our patients. CP was present in almost two-thirds of the children with WS, most of them had severe cognitive and motor deficits.

Paroxysmal non-epileptic events in infancy: five cases with typical features.

The differential diagnosis of paroxysmal non-epileptic events in early childhood is one of the most challenging tasks in paediatrics, and may be difficult even for specialized child neurologists. Parents are usually concerned by every unusual movement of their children and consult paediatric general practitioners immediately. We investigated five infants/toddlers (aged 1-30 months) referred by their general practitioners with a suspicion of epilepsy. None of them were ultimately shown to have epilepsy. Our aim was to determine the main reasons for referral and describe, through images and video, the typical features of five non-epileptic paroxysmal events (benign neonatal sleep myoclonus, jitteriness, shuddering attack, paroxysmal tonic upgaze, and infantile masturbation). The review of these events reveals the significance of the circumstances within the history of the patients. A detailed history is of considerable help in the differentiation of epileptic paroxysmal events from non-epileptic events, avoiding unnecessary investigations. Video-EEG examination is necessary only in cases when epilepsy is strongly suspected. [Published with video sequences].

[Epidemiology, cost and economic impact of cerebral palsy in Hungary]. / A cerebralis paresis epidemiológiája, költségei és közgazdasági hatásai Magyarországon.

BACKGROUND AND PURPOSE: The purpose of our communication was to determine the total cost of cerebral paretic patients in Hungary between 0 and 18 years and to assess their impact on the national budget. METHODS: Based on the data of Borsod county we calculated the CP characteristics. The cost of CP was determined by routine care of individuals. Lost Parental Income and Tax were calculated on the basis of average earnings. The ratio of GDP, Health and Social Budget and Health Budget to CP is based on CP annual average cost and frequency. We have developed a repeatable computational model. RESULTS: Of the risk groups, premature birth (30.97%), low birth weight (29.64%), perinatal asphyxia (19.47%) were the most common. Source is unknown of 37.61% of the cases. CP prevalence was 2.1‰. The two-sided (59.7%) and the one-sided (19.0%) spastic pareses dominated. The most serious form is the two-sided spastic paresis (42.5% GMFCS 3-5 degrees). Epilepsy was 22.0%, incontinence was 27%, mental involvement was 46%. Care for one child up to 18 years of age costs an average of 73 million HUF (€ 251,724). The lost family income was 27.36 million HUF (€ 94,345), and lost tax and health care contributions were 14.46 million HUF (€ 49,862). Additionally, 0.525% of the GDP, 0.88% of the full health and social budget and 1.83% of direct medical costs were spent for CP families. CONCLUSION: The cost of CP disease is significant. Costs can be reduced by improving primary prevention. From the perspective of the family and government, it is better to care for families so they can take care of their disabled children.

[The evaluation of paroxysmal events in neonates and infants]. / Paroxysmalis jelenségek megítélése az újszülöttés csecsemokorban.

BACKGROUND AND PURPOSE: Differential diagnosis of neonatal and infantile seizures based only on inspection poses a challenge even for specialists. Aims - To investigate the evaluations of neonatal and infantile paroxysmal events based only on inspection. Research question - Is there any difference in the opinion of neonatologists, paediatric neurologists and neurologists about the assessment of common paroxysmal events in infancy? METHODS: Video recordings about paroxysmal movements of 15 neonates or infants (aged 2 days- 5 months) were displayed for 47 paediatric neurologists, 35 neonatologists and nurses working in Neonatal or Perinatal Intensive Care Units and 43 neurologists. They had to decide without knowing the past medical history or EEG results whether events presented were epileptic or nonepileptic in nature. RESULTS: Answers of neonatologists and paediatric neurologists were correct in 67% of cases (824/1230), no significant difference was found between their results. The largest uncertainty was in the judgement of discrete hand movements and very rapid clonus with epileptic origin, they were judged correctly by only one third of participants. The result of neurologists was only slightly, but not significantly different from that of paediatric neurologists. CONCLUSION: In most cases, the correct diagnosis of neonatal and infantile paroxysmal events requires video-EEG recording. No significant difference was revealed between the evaluation of neonatologists and paediatric neurologists about the differential diagnosis of movements. The ongoing cooperation of paediatric neurologists and neurologists going back to several decades facilitates the shaping of a common perspective.

Epileptic seizure or not? Proportion of correct judgement based only on a video recording of a paroxysmal event.

PURPOSE: Our study was intended to measure the proportion of correct seizure recognition among different medical and non-medical groups based on only a video recording. METHODS: Video recordings about paroxysmal movements of 15 very young infants (2days - 5 months of age) were displayed for six groups: 159 1st-year medical students, 65 4-5th-year medical students, 52 paediatric residents, 18 paediatric neurologists from different European countries, 43 adult neurologists and 37 parents whose children were treated at our Department. All participants were asked to decide which recording they considered as of epileptic origin or a non-epileptic event. Correct answer rate (CAR) was calculated in each group for every video. RESULTS: The average CAR was the lowest in the group of 1st-year medical students (36.6%), the best results were reached by paediatric neurologists (67.4%). The CAR was significantly different between the groups of 1st-year medical students and paediatric neurologists (p=0.02), and between the groups of 1st-year medical students and residents (p=0.045). The CAR of the most deceptive epileptic seizure was only 18.2%. The judgement of parents proved to be better than that of the 1st-year medical students. CONCLUSIONS: Recognising epileptic seizures in very young infants without EEG is extremely inaccurate. Even trained paediatric neurologists were able to judge correctly the different movement types in only 67.4% of the cases. The role of education and experience is clearly indicated by the increase in CAR from 1st-year medical students through well-trained paediatric neurologists.

MRI classification system (MRICS) for children with cerebral palsy: development, reliability, and recommendations.

AIM: To develop and evaluate a classification system for magnetic resonance imaging (MRI) findings of children with cerebral palsy (CP) that can be used in CP registers. METHOD: The classification system was based on pathogenic patterns occurring in different periods of brain development. The MRI classification system (MRICS) consists of five main groups: maldevelopments, predominant white matter injury, predominant grey matter injury, miscellaneous, and normal findings. A detailed manual for the descriptions of these patterns was developed, including test cases (www.scpenetwork.eu/en/my-scpe/rtm/neuroimaging/cp-neuroimaging/). A literature review was performed and MRICS was compared with other classification systems. An exercise was carried out to check applicability and interrater reliability. Professionals working with children with CP or in CP registers were invited to participate in the exercise and chose to classify either 18 MRIs or MRI reports of children with CP. RESULTS: Classification systems in the literature were compatible with MRICS and harmonization possible. Interrater reliability was found to be good overall (k=0.69; 0.54-0.82) among the 41 participants and very good (k=0.81; 0.74-0.92) using the classification based on imaging reports. INTERPRETATION: Surveillance of Cerebral Palsy in Europe (SCPE) proposes the MRICS as a reliable tool. Together with its manual it is simple to apply for CP registers.

[EEG-based cerebral networks in 14 neurological disorders]. / EEG-alapú agyi hálózatok 14 neurológiai betegségben.

Background - Brain networks have not been systematically investigated yet in most neurological disorders. Purpose - To investigate EEG functional connectivity (EEGfC) networks in 14 neurological disorders. Patients - Potentially eligible patients were collected from clinical and EEG databases. All the available clinical data and EEG records were critically revised. All the patients who suffered of a single neurological disorder (out of the 14) and had a good quality EEG recording entered the study. Confoundig factors as comorbidity and CNS-active drug effects were eliminated as far as possible. EEG analysis - Three minutes of resting-state, waking EEG activity were selected for analysis. Current source density (CSD) values were computed for 2394 cortical voxels by Low Resolution Electromagnetic Tomography (LORETA). Thereafter, Pearson correlation coefficients were computed between all pairs of 23 cortical regions of interest (ROI) in each hemisphere (LORETA Source Correlation, LSC software). Computation was carried out for conventional EEG broad bands and very narrow bands (1 Hz bandwidth) between 1 and 25 Hz as well. Correlation coefficients of each group were statistically compared to our normative EEG (LSC) database by two-talied t-tests. Bonferroni-corrected p<0.05 values were accepted as statistically significant, and were graphically displayed as topographical networks. Results and conclusion - Group-specific networks were demonstrated. However, non-specific networks, charasteristic for most groups, were detected as well. Common finding were: decreased connectivity in the alpha band and increased connectivity in the delta, theta bands and upper-beta band. Decreased alpha-band connectivity presumably reflected primary lesional effects and on the other hand, non-specific vulnerability of "rich club connections". Increased connectivity in the slow bands presumably indicated adaptive-compensatory activity of brain homeostasis.

Anti-N-methyl-D-aspartate receptor encephalitis and drug abuse - the probable role of molecular mimicry or the overstimulation of CB receptors in a 17-year-old adolescent - case report.

Anti-N-methyl-D-aspartate encephalitis is an autoimmune disorder characterized by autoantibodies produced against NMDA receptors. We report the case of a 17-year-old drug user teenager who presented with altered mental scale, psychiatric symptoms and autonomic dysfunction. In the background we diagnosed NMDA encephalitis. We supposed that synthetic cannabinoids/drugs may have lead to the of trigger NMDA encephalitis via the altered activation of the immune system and molecular mimicry mechanism.

[INDIVIDUAL EVALUATION OF LORETA ABNORMALITIES IN IDIOPATHIC GENERALIZED EPILEPSY]. / EGYEDI LORETA-ABNORMALITÁSOK VIZSGÁLATA IDIOPATHIÁS GENERALIZÁLT EPILEPSZIÁKBAN.

BACKGROUND: Contemporary neuroimaging methods disclosed structural and functional cerebral abnormalities in idiopathic generalized epilepsies (IGEs). However, individual electrical (EEG) abnormalities have not been evaluated yet in IGE patients. METHODS: IGE patients were investigated in the drug-free condition and after 3-6 month of antiepileptic treatment. To estimate the reproducibility of qEEG variables a retrospective recruited cohort of IGE patients was investigated. 19-channel resting state EEG activity was recorded. For each patient a total of 2 minutes EEG activity was analyzed by LORETA (Low Resolution Electromagnetic Tomography). Raw LORETA values were Z-transformed and projected to a MRI template. Z-values outside within the [+3Z] to [-3Z] range were labelled as statistically abnormal. RESULTS: 1. In drug-free condition, 41-50% of IGE patients showed abnormal LORETA values. 2. Abnormal LORETA findings showed great inter-individual variability. 3. Most abnormal LORETA-findings were symmetrical. 4. Most maximum Z-values were localized to frontal or temporal cortex. 5. Succesfull treatment was mostly coupled with disappearence of LORETA-abnormality, persistent seizures were accompanied by persistent LORETA abnormality. DISCUSSION: 1. LORETA abnormalities detected in the untreated condition reflect seizure-generating property of the cortex in IGE patients. 2. Maximum LORETA-Z abnormalities were topographically congruent with structural abnormalities reported by other research groups. 3. LORETA might help to investigate drug effects at the whole-brain level.

Increased resting-state EEG functional connectivity in benign childhood epilepsy with centro-temporal spikes.

PURPOSE: To explore intrahemispheric, cortico-cortical EEG functional connectivity (EEGfC) in benign childhood epilepsy with rolandic spikes (BECTS). METHODS: 21-channel EEG was recorded in 17 non-medicated BECTS children and 19 healthy controls. 180s of spike- and artifact-free activity was selected for EEGfC analysis. Correlation of Low Resolution Electromagnetic Tomography- (LORETA-) defined current source density time series were computed between two cortical areas (region of interest, ROI). Analyses were based on broad-band EEGfC results. Groups were compared by statistical parametric network (SPN) method. Statistically significant differences between group EEGfC values were emphasized at p<0.05 corrected for multiple comparison by local false discovery rate (FDR). RESULTS: (1) Bilaterally increased beta EEGfC occurred in the BECTS group as compared to the controls. Greatest beta abnormality emerged between frontal and frontal, as well as frontal and temporal ROIs. (2) Locally increased EEGfC emerged in all frequency bands in the right parietal area. CONCLUSIONS: Areas of increased EEGfC topographically correspond to cortical areas that, based on relevant literature, are related to speech and attention deficit in BECTS children.