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Background: A relevant number of visits to pediatric emergency departments (pED) are associated to mild traumatic brain injury (mTBI). On March 16th 2020, the Bavarian government declared a first full lockdown (LD) related to the COVID-19 pandemic. Aim: The aim of the study was to investigate the impact of LD on pediatric mTBI. Methods: Retrospective chart review of presentations to a pED due to mTBI. Study periods covered LD (03/17/2020 through 05/05/2020) and the same time in 2017, 2018, and 2019 as reference period (RP). Comparative analyses were performed by Chi-square or Fisher's exact test. Results: Numbers of mTBI cases decreased by half. Age distribution did not differ. A significantly higher proportion of mTBI were related to falls at home (p= .001). Further, a higher rate of hospital admissions (p= .03), a higher proportion of ICU admissions (p= .001), a longer duration of hospital stay (p= .02), and a higher rate of intracranial pathologies on neuroimaging was observed during LD (p= .007). Conclusion: The decrease in mTBI presentations is likely due to an absolute decrease in numbers related to the LD measures, combined with a hesitation to present very minor mTBI to the hospital, because of fear of being infected or not to put additional strain on the health care system during this health care crisis. On the other hand, data of those that presented with mTBI tend to reflect the more severe spectrum of mTBI.

PURPOSE: A large prospective database from three Phase 3 studies allowed the study of spasticity-related pain (SRP) in pediatric cerebral palsy (CP). METHODS: Baseline (pretreatment) SRP data occurring during different activities in children/adolescents (aged 2–17 years, ambulant/nonambulant) with uni-/bilateral spastic CP was obtained using the Questionnaire on Pain caused by Spasticity (QPS; six modules specific to spasticity level [lower limb (LL) or upper limb (UL)] and type of respondent [child/adolescent, interviewer, or parent/caregiver]). RESULTS: At baseline, 331 children/adolescents with LL- and 155 with UL-spasticity completed at least one key item of their modules; LL/UL QPS modules of parent/caregivers were at least partially completed (key items) by 841/444 parents/caregivers. SRP with at least one activity at baseline was self-reported in 81.9% /69.7% (LLs/ULs) of children/adolescents with spasticity. Parents/caregivers observed LL/UL SRP behaviors in 85.9% /77.7% of their children, with multiple body regions affected. SRP negatively affected the great majority of the children in various ways. Child/adolescent-reported mean SRP intensity and parent/caregiver-observed mean SRP behavior frequencies were higher for LLs than ULs, and the level of SRP increased with more physically demanding activities. CONCLUSION: These data suggest SRP is more common and intense in pediatric CP than generally thought, emphasizing the need for effective, long-term pain management.

PURPOSE: This pooled analysis of data from three Phase 3 studies investigated the effects of incobotulinumtoxinA on spasticity-related pain (SRP) in children/adolescents with uni-/bilateral cerebral palsy (CP). METHODS: Children/adolescents (ambulant and non-ambulant) were evaluated for SRP on increasingly difficult activities/tasks 4 weeks after each of four incobotulinumtoxinA injection cycles (ICs) using the Questionnaire on Pain caused by Spasticity (QPS; six modules specific to lower limb [LL] or upper limb [UL] spasticity and respondent type [child/adolescent, interviewer, or parent/caregiver]). IncobotulinumtoxinA doses were personalized, with all doses pooled for analysis. RESULTS: QPS key item responses were available from 331 and 155 children/adolescents with LL- and UL-spasticity, respectively, and 841/444 (LL/UL) of their parents/caregivers. IncobotulinumtoxinA efficacy was evident with the first IC. Efficacy was sustained and became more robust with further subsequent ICs. By Week 4 of the last (i.e. fourth) IC, 33.8–53.3% of children/adolescents reported complete SRP relief from their baseline pain for respective QPS items. Children/adolescents reported reductions in mean LL SRP intensity at levels that surpassed clinically meaningful thresholds. Similarly, parents/caregivers observed complete SRP relief and less frequent SRP with incobotulinumtoxinA. Similar results were found for UL SRP. CONCLUSION: These findings indicate that incobotulinumtoxinA could bring considerable benefit to children/adolescents with spasticity by reducing SRP, even during strenuous activities.

Introduction Caring for individuals with fetal alcohol spectrum disorders (FASD) puts a substantial and often life-long burden on affected families. Caregivers’ specific needs and demands are, however, not well understood so far. We thus aimed at systematically collecting data on the needs of individuals caring for children and adolescents with FASD. Materials and methods Between May 2019 and November 2020, a quantitative survey among caregivers and professionals from across Germany was performed. Participants completed a questionnaire collecting information on the perceived support caregivers receive from various sources as well as the current fulfilment of caregivers’ needs. Specifically, the fulfilment of a variety of specific needs summarised in five categories was rated by the participants on a scale ranging from 1 (very good) to 6 (insufficient). Results Both caregivers and professionals rated the overall fulfilment of needs rather poorly (mean: 3.94 and 4.27, respectively). Caregivers indicated needs concerning coordination of support (4.74) and relief services (4.44) to be fulfilled the least while needs in the relief services category also received the lowest average grade among professionals (4.57). The needs that the caregivers regarded as most sufficiently fulfilled were their own knowledge about FASD (mean: 1.95) and their knowledge about the causes of their child's problems (mean: 1.87). Conclusions The results of the present study indicate that FASD caregivers are supported insufficiently, while most of their needs remain unmet. Health care planners and providers thus urgently need to identify and implement measures to better address FASD caregivers’ needs and demands.

PURPOSE: Investigate the efficacy and safety of multipattern incobotulinumtoxinA injections in children/adolescents with lower-limb cerebral palsy (CP)-related spasticity. METHODS: Phase 3 double-blind study in children/adolescents (Gross Motor Function Classification System – Expanded and Revised I–V) with unilateral or bilateral spastic CP and Ashworth Scale (AS) plantar flexor (PF) scores ? 2 randomized (1:1:2) to incobotulinumtoxinA (4, 12, 16 U/kg, maximum 100, 300, 400 U, respectively) for two 12- to 36-week injection cycles. Two clinical patterns were treated. Pes equinus (bilateral or unilateral) was mandatory; if unilateral, treatment included flexed knee or adducted thigh. Endpoints: Primary: AS-PF change from baseline to 4 weeks; Coprimary: investigator-rated Global Impression of Change Scale (GICS)-PF at 4 weeks; Secondary: investigator’s, patient’s, and parent’s/caregiver’s GICS, Gross Motor Function Measure-66 (GMFM-66). RESULTS: Among 311 patients, AS-PF and AS scores in all treated clinical patterns improved from baseline to 4-weeks post-injection and cumulatively across injection cycles. GICS-PF and GICS scores confirmed global spasticity improvements. GMFM-66 scores indicated better motor function. No significant differences between doses were evident. Treatment was well-tolerated, with no unexpected treatment-related adverse events or neutralising antibody development. CONCLUSION: Children/adolescents with lower-limb spasticity experienced multipattern benefits from incobotulinumtoxinA, which was safe and well-tolerated in doses up to 16 U/kg, maximum 400 U.

Childhood arterial ischemic stroke differs in essential aspects from adult stroke. It is rare, often relatively unknown among laypersons and physicians and the wide variety of age-specific differential diagnoses (stroke mimics) as well as less established care structures often lead to a considerable delay in the diagnosis of stroke. The possible treatment options in childhood are mostly off-label. Experiences in well-established acute treatment modalities in adult stroke, such as thrombolysis and mechanical thrombectomy are therefore limited in children and only based on case reports and case series. The etiological clarification is time-consuming due to the multitude of risk factors which must be considered. Identifying each child's individual risk profile is mandatory for acute treatment and secondary prevention strategies and has an influence on the individual outcome. In addition to the clinical neurological outcome the residual neurological effects of stroke on cognition and behavior are decisive for the integration of the child into its educational, later professional and social environment.

Migrane ist eine haufige neurologische Erkrankung im Kindes- und Jugendalter, deren Komplexitat und Alltagsrelevanz regelhaft unterschatzt wird. Die Pravalenz im Kindes- und Jugendalter ist in den letzten Jahren zunehmend. Typisch fur das junge Alter ist der Mischtyp mit Uberlappung von Migrane und Spannungskopfschmerzen. Eine fruhe und korrekte Diagnose ist entscheidend, um eine rasche und adaquate Behandlung zu initiieren, einen bestmoglichen Verlauf uber die kindliche Entwicklung zu unterstutzen sowie der Gefahr fur somatische Belastungsstorungen und Chronifizierung vorzubeugen. Dabei kommen (1) bio-psycho-sozialem Krankheitsverstandnis, (2) Assoziation von nuchalen Muskelverspannungen und Migrane (uber den sog. trigemino-zervikalen Komplex) sowie (3) strukturierter, interdisziplinarer, multiprofessioneller und multimodaler Therapie besondere Bedeutung zu. Dies wird anhand der „moma“-Intervention („modules-on-migraine-activity“), einem Innovationsfonds-Projekt des Gemeinsamen Bundesausschusses (G-BA), veranschaulicht.

Repetitive neuromuscular magnetic stimulation (rNMS) was previously applied in adult patients with episodic migraine, showing beneficial effects on headache characteristics, high safety, and convincing satisfaction. This study aims to assess rNMS as a personalized intervention in pediatric headache.Retrospective chart review including patients with migraine, TTH, mixed type headache, or PTH, who had received at least one test rNMS session targeting the upper trapezius muscles (UTM).33 patients (13.9 ± 2.5 years; 61% females) were included in the primary analysis, resulting in a total of 182 rNMS sessions. 43 adverse events were documented for 40 of those sessions (22%). Most common side effects were tingling (32.6%), muscle sore (25.5%), shoulder (9.3%) and back pain (9.3%). Secondly, in patients (n = 20) undergoing the intervention, headache frequency (p = 0.017) and minimum and maximum intensities (p = 0.017; p = 0.023) significantly decreased from baseline to 3-month after intervention. 11 patients (44%) were classified as ≥25% responders, with 7 patients (28%) experiencing a ≥75% reduction of headache days. After 73% of interventions, patients reported rNMS helped very well or well. A majority of patients would repeat (88.5%) and recommend rNMS (96.2%) to other patients.rNMS seems to meet the criteria of safety, feasibility, and acceptance among children and adolescents with three age-typical headache disorders. A significant reduction in headache frequency and intensity during a 3 months follow-up was documented. Larger, prospective, randomized, sham-controlled studies are urgently needed to confirm if rNMS may become a new valuable non-invasive, non-pharmacological treatment option for pediatric headache disorders.

PURPOSE: The open-label phase 3 “Treatment with IncobotulinumtoxinA in Movement Open-Label” (TIMO) study investigated longer-term safety and efficacy of incobotulinumtoxin A in children/adolescents with cerebral palsy (CP). METHODS: Patients on standard treatment, with unilateral or bilateral lower limb (LL) or combined upper limb (UL)/LL spasticity received four incobotulinumtoxinA injection cycles (16 or 20 Units/kg bodyweight total [maximum 400 or 500 Units] per cycle depending on ambulatory status/clinical pattern treated), each followed by 12–16 weeks’ observation. Treatment for pes equinus was mandatory; flexed knee or adducted thigh were options for unilateral treatment and/or ULs for unilateral/bilateral treatment. The primary endpoint was safety; changes in Ashworth Scale and Gross Motor Function Measure-66 scores, and Global Impression of Change Scale scores at week 4 of each injection cycle were also evaluated. RESULTS: IncobotulinumtoxinA (≤500 Units for ≤98 weeks) was safe, well-tolerated, and effective across all endpoints for multipattern treatment of LL and combined LL/UL spasticity in ambulant/nonambulant children/adolescents with CP. Treatment effects increased with each injection cycle. No new/unexpected safety concerns were identified. CONCLUSION: IncobotulinumtoxinA showed a good safety and tolerability profile, with efficacy over multiple clinical presentations. As an adjunct treatment, it offers an effective, individualized treatment option for pediatric CP-related spasticity.

The objective of this study was to assess the burden of disease and prevalence of lifestyle factors for adolescents and young adults with frequent episodic migraine. We conducted a secondary comparative analysis of data collected during two previous studies. Inclusion criteria for this analysis were age 15–35 years, 15 to 44 migraine episodes within 12 weeks, and completeness of Migraine Disability Assessment and lifestyle questionnaire data. Datasets of 37 adults (median age [interquartile range]: 25 [6]) and 27 adolescents (median age [interquartile range]: 15 [1]) were analyzed. 81% ( n = 30) of adults reported severe disability (16% [ n = 3] of adolescents; p < 0.001). Headache frequency (24 vs. 17 days; p = 0.005) and prevalence of regular analgesic use (60% [ n = 22] vs. 18% [ n = 5]; p = 0.002) were significantly higher in adults. In adults, sleep duration on weekdays was significantly lower (8.5 vs. 10 h; p < 0.001). Any consumption of caffeine tended to be higher in adolescents and alcohol consumption tended to be higher in adults ( p > 0.05). This study underlines the importance of educating adolescents and young adults with migraine about lifestyle habits that are likely to interfere with the condition.

Aim To assess the improvement in gross motor function following three blocks of a three-week, intensive robot-enhanced treadmill therapy (ROBERT-Program). Method retrospective chart review in a before-after interventional trial in children with cerebral palsy attending a university hospital outpatient rehabilitation centre. Patients received three blocks of a three-week, 12 sessions ROBERT-Program over a mean period of 24 months. Outcome measures were block specific and cumulative improvement in GMFM 66, D and E. Longterm GMFM 66 improvements were compared to the individuals' expected increment as derived from previously published GMFM-66 percentiles. 95% confidence intervals (CI) and paired t-test were calculated. Results 20 children (8 GMFCS Level II; 12 GMFCS Level III, mean age 5.9 years (CI: [5.0; 6.7])) were treated. For each block a significant increase in motor performance in similar size could be observed without deterioration between blocks. The cumulative improvement during 21 months observation period was: 6.5 (CI: [4.8; 8.2]) in GMFM 66, which represents a clinically meaningful effect size of 3.6 (CI: [1.4; 5.8]) above the expected improvement. Interpretation Progressive clinically meaningful improvement in motor performance for three blocks of ROBERT-Program was observed. Cumulative GMFM 66 improvements exceeded the individuals' age-specific expected course.

Introduction Brain function deficits cause strong negative impacts for the everyday lives of children and adolescents with fetal alcohol spectrum disorders (FASD). Therefore, evidence-based intervention programs that are specifically designed for patients with FASD are needed but still scarce. The aim of the presented article is a systematic literature review of evidence-based intervention strategies for children and adolescents with FASD. Materials and methods A comprehensive systematic literature search was conducted in several relevant databases to identify randomized-controlled intervention studies for children and adolescents with FASD. Results We identified 25 randomized-controlled studies regarding interventions in FASD. Overall, evidence indicating that some therapeutic interventions are effective in children and adolescents with FASD was found. Even though evidence-based interventions rarely lead to improvements of performance into a “normal range”, those measures can alleviate negative consequences of prenatal alcohol exposure and relieve daily burdens. Conclusion There are only a few randomized-controlled trials regarding therapy research for children and adolescents with FASD. Their results indicate that especially the combination of parent and child sessions present a promising approach for the treatment of FASD. Positive treatment effects of interventions seem to be domain specific, except for interventions regarding self-regulation or social interaction.

Background This randomized phase 3 study with double-blind main period (MP) and open-label extension (OLEX; NCT02002884 ) assessed incobotulinumtoxinA safety and efficacy for pediatric upper-limb spasticity treatment in ambulant/nonambulant (Gross Motor Function Classification System [GMFCS] I-V) patients, with the option of combined upper- and lower-limb treatment. Methods Patients were aged two to 17 years with unilateral or bilateral spastic cerebral palsy (CP) and Ashworth Scale (AS) score ≥2 in treatment-selected clinical patterns. In the MP, patients were randomized (2:1:1) to incobotulinumtoxinA 8, 6, or 2 U/kg body weight (maximum 200, 150, 50 U/upper limb), with optional lower-limb injections in one of five topographical distributions (total body dose ≤16 to 20 U/kg, maximum 400 to 500 U, depending on body weight and GMFCS level). In the OLEX, patients received three further treatment cycles, at the highest MP doses (8 U/kg/upper limb group). Outcomes included AS, Global Impression of Change Scale (GICS), and adverse events (AEs). Results AS scores improved from baseline to week 4 in all MP dose groups (n = 350); patients in the incobotulinumtoxinA 8 U/kg group had significantly greater spasticity improvements versus the 2 U/kg group (least-squares mean [standard error] for upper-limb main clinical target pattern −1.15 [0.06] versus −0.93 [0.08]; P = 0.017). Investigator's, child/adolescent's, and parent/caregiver's GICS scores showed improvements in all groups. Treatment benefits were sustained over further treatment cycles. AE incidence did not increase with dose or repeated treatment across GMFCS levels. Conclusions Data provide evidence for sustained efficacy and safety of multipattern incobotulinumtoxinA treatment in children and adolescents with upper-limb spasticity.

Migraine is a common and invalidating disorder worldwide. Patients of all ages experience the disorder as very impairing regarding their personal and occupational lives. The current approach in migraine therapy is multimodal including lifestyle management, psychoeducation and, if available, psychotherapeutic interventions, and pharmacotherapy. The lack of non-pharmacological and non-invasive treatment options call for new and innovative therapeutic approaches. Peripheral neurostimulation is a relatively new method in migraine management offering a painless and non-pharmacological way of targeting specific mechanisms involved in migraine. This review summarizes 15 recent randomized clinical trials to provide an overview of non-invasive peripheral neurostimulation methods currently available for the treatment of migraine. Efficacy, tolerability, and safety of the different interventions and their feasibility in the pediatric setting are evaluated. Vagal nerve stimulation (VNS), remote electrical neuromodulation (REN) and supraorbital nerve stimulation (SNS) are considered effective in treating acute migraine attacks, the latter being more pronounced in migraine without aura. Regarding migraine prevention, occipital nerve stimulation (ONS) and supraorbital nerve stimulation (SNS) demonstrated efficacy, whereas repetitive neuromuscular magnetic stimulation (rNMS) may represent a further effective option in episodic migraine. REN and rNMS were found to be well-accepted with fewer patients discontinuing treatment than those receiving direct cranial nerve stimulation. In summary, peripheral neurostimulation represents a promising option to complement the multimodal therapy concept for pediatric migraine. In particular, rNMS opens a new field for research and treatment fitting the requirements of "non-invasiveness" for children. Given the reported efficacy, safety, and feasibility, the therapy decision should be made on an individual level.

Acute treatment of childhood arterial ischaemic stroke and prevention strategies for recurrent stroke episodes depend strongly on each child's individual risk profile. The aim of this study is to characterize risk factors for childhood stroke, their occurrence in isolation or combination, and to identify possible common risk factor patterns.This population-based study was conducted via ESPED, a surveillance unit for rare paediatric diseases in Germany. Children aged28days and18 years with an acute arterial ischaemic stroke occurring between January 2015 and December 2016 were included.Among 99 reported children with arterial ischaemic stroke, 56 children were male. Male predominance was significant in adolescents from 12 years old onward. Arterial ischaemic stroke was more common in very young children2 years of age and in adolescence. No risk factor was identified in 27 children. Hypercoagulable states (29%), cardiac disorders (24%), and arteriopathies (21%) were the most common risk factors. Some risk factor categories were more likely to be identified in isolation (i.e. cardiac disorders, prothrombotic abnormalities and chronic head and neck disorders) than others. The number of risk factors (n = 0-4) per patient and risk factor categories did not differ by age.Although we could not identify common patterns of risk factor combinations, several risk factors occurred more likely in isolation than others. Further research should focus on the impact of isolated presumed childhood stroke risk factors like certain prothrombotic abnormalities, migraine or a patent foramen ovale. With regard to different age groups, stroke mechanisms in male adolescents require particular attention.

Zusammenfassung. Laut den aktuellen Studien zur Gesundheit in Deutschland (GEDA) haben knapp 20 % der schwangeren Frauen einen „moderaten Alkoholkonsum“ und knapp 8 % einen riskanten Alkoholkonsum. 12 % der Schwangeren geben ein Rauschtrinken (≥ 5 Getränke pro Gelegenheit) seltener als einmal pro Monat, knapp 4 % jeden Monat und 0.1 % mindestens jede Woche an. Zurückhaltende, strenge Schätzungen ergeben, dass ca. 1 % aller Kinder intrauterin durch Alkohol geschädigt werden. Extrapoliert aus dieser Schätzung bedeutet dies, dass in Deutschland ca. 0.8 Millionen Menschen, davon 130 000 Kinder, mit einer Fetalen Alkoholspektrumstörung (FASD) leben. Die Mehrzahl der betroffenen Kinder werden nicht oder erst spät richtig diagnostiziert. Professionelle Helfer im Gesundheits- und Sozialsystem sind bislang über die Symptome und die notwendige Diagnostik der FASD nur unzureichend informiert. Ziel dieses Übersichtsartikels ist die Erläuterung der ärztlichen und psychologischen diagnostischen Möglichkeiten und Notwendigkeiten bei Kindern und Jugendlichen mit FASD. Eine frühzeitige Diagnose und ein konstantes förderndes und gewaltfreies Umfeld sind als wichtigste protektive Faktoren für den Langzeit-Outcome von Menschen mit FASD identifiziert worden.

Purpose To assess the impact of vertigo on health-related quality of life (HrQoL) of children/adolescents and to assess if the impact on HrQoL varies by age group, gender, and type of vertigo diagnoses. Methods A retrospective analysis was performed on the clinical and HrQoL data of children and adolescents referred to the German Center of Vertigo and Balance Disorders (n = 32; male = 17; female = 15; age range: 8–18 years), using the KIDSCREEN-52 questionnaire. For each scale, means of the Z-scores with 95% confidence intervals of the study and norm sample were compared. By nonparametric Kruskal–Wallis statistics differences between diagnostic groups were assessed. To assess the gender- and age-specific impact of vertigo on quality of life, Wilcoxon signed-rank test was used. Results The means of the physical well-being, psychological well-being, autonomy scale, and the general HrQoL index of patients were considerably lower than the means of the norm sample. The physical well-being seemed to be most affected by vertigo. The reduction of HrQoL was not related to gender and vertigo types but seemed to be higher in children suffering from vertigo aged 12 to 18 years than children aged 8 to 11 years. Conclusion These are the first data to demonstrate impaired HrQoL in children with chronic vertigo.

International audience; Background: General Movement Assessment (GMA) is a powerful tool to predict Cerebral Palsy (CP). Yet, GMA requires substantial training challenging its broad implementation in clinical routine. This inspired a world-wide quest for automated GMA. Aim: To test whether a low-cost, marker-less system for three-dimensional motion capture from RGB depth sequences using a whole body infant model may serve as the basis for automated GMA.Study design: Clinical case study at an academic neurodevelopmental outpatient clinic.Subjects: Twenty-nine high risk infants were assessed at their clinical follow-up at 2-4 month corrected age (CA). Their neurodevelopmental outcome was assessed regularly up to 12-31 months CA.Outcome measures: GMA according to Hadders-Algra by a masked GMA-expert of conventional and computed 3D body model (“SMIL motion”) videos of the same GMs. Agreement between both GMAs was tested using dichotomous and graded scaling with Kappa and intraclass correlations, respectively. Sensitivity and specificity to predict CP at ≥12 months CA were assessed.Results: Agreement ofthe two GMA ratings was moderate-good for GM-complexity (κ=0.58; ICC=0.874 [95%CI 0.730;0.941]) and substantial-good for fidgety movements (FMs; Kappa=0.78, ICC=0.926[95%CI 0.843;0.965]). Five children were diagnosed with CP (four bilateral, one unilateral CP). The GMs of the child with unilateral CP were twice rated as mildly abnormal with FMs. GM-complexity and somewhat less FMs, of both conventional and SMIL motion videos predicted bilateral CP comparablyto published literature.Conclusions: Our computed infant 3D full body model is an attractive starting point for automated GMA in infants at risk of CP.

Objective The aim of the current study is to measure long‐term executive function, motor outcome, and QoL in children, adolescents, and young adults after VAD and Htx. Methods Patients were examined during routine follow‐up. Investigation tools were used as follows: Examination for MND of motor outcomes, Epitrack® for attention and executive functioning, and Kidscreen‐52 and EQ‐5D‐5L questionnaires for QoL. Additional data were retrospectively obtained by an analysis of patient medical records. Results Out of 145 heart transplant recipients at the department of pediatric cardiology of the University Hospital Munich, 39 were implanted with a VAD between 1992 and 2016. Seventeen (43.6%) patients died before or after Htx; 22 (56.4%) patients were included in our study. Mean age at transplant was 9.52 years (range: 0.58‐24.39 years, median 9), and the mean follow‐up time after Htx was 6.18 years (range: 0.05‐14.60 years, median 5.82). MND examination could be performed in 13 patients (normal MND: n = 11, simple MND: n = 1, complex MND: n = 1). Executive functioning was tested in 15 patients. Two (13.3%) patients had good results, six (40%) average results, three (20%) borderline results, and four (26.7%) impaired results. QoL (Kidscreen n = 7, EQ‐5D‐5L n = 8) was similar to a healthy German population. Conclusion Motor outcome, executive functioning and QoL in survivors of VAD bridging therapy and Htx can be good, though underlying diseases and therapies are associated with a high risk of cerebral ischemic or hemorrhagic complications.

Intrauterine Alkoholexposition kann zu einer toxischen Schadigung des kindlichen Gehirns und damit zur Fetalen Alkoholspektrumstorung („fetal alcohol spectrum disorder“, FASD) fuhren. Deren geschatzte Pravalenz betragt in Deutschland ca. 1 %. Die FASD persistiert lebenslang. Mit dem Krankheitsbild FASD werden neben Kinder-/Jugendarzten und Kinder-/Jugendpsychiatern auch Allgemeinarzte, Neurologen, Psychiater und Psychotherapeuten sowie Gynakologen (und weite Bereiche der Padagogik und Sozialarbeit) konfrontiert. Ausgehend von der systematisch recherchierten, methodisch bewerteten Literatur (2001–2015) konsentierte die multidisziplinare Leitliniengruppe Empfehlungen fur die Diagnose der FASD. In dieser Publikation werden die differenzierenden diagnostischen Kriterien fur das partielle Fetale Alkoholsyndrom (pFAS), die alkoholbedingte entwicklungsneurologische Storung („alcohol-related neurodevelopmental disorder“, ARND) und die alkoholbedingten angeborenen Malformationen („alcohol-related birth defects“, ARBD) dargelegt. Die Literaturrecherche zu pFAS, ARND und ARBD ergab 365 Treffer. Es wurden 58 Publikationen in die methodische Bewertung aufgenommen. Um das pFAS zu diagnostizieren, mussen 2 von 3 fazialen Auffalligkeiten (kurze Lidspalten, schmale Oberlippe und verstrichenes Philtrum), mindestens 3 Auffalligkeiten des Zentralnervensystems (ZNS) und ein wahrscheinlicher mutterlicher Alkoholkonsum vorhanden sein. Zur Diagnose der ARND werden mindestens 3 ZNS-Auffalligkeiten und eine gesicherte intrauterine Alkoholexposition gefordert. Alkoholbedingte angeborene Malformation wird als separate Diagnose verworfen. Evidenzbasierte und praktisch anwendbare Empfehlungen fur die Diagnose der FASD wurden bestimmt. Hinsichtlich der Versorgung und Sekundarpravention von Folgeerkrankungen besteht Forschungsbedarf.

Der Schlaganfall im Kindesalter ist ein Notfall mit hoher Mortalitat und Morbiditat. Er hat – in Abhangigkeit von der zugrunde liegenden Atiologie – ein klinisch bedeutsames Rezidivrisiko. Die Atiologie ist vielfaltig. Vaskulopathien, Infektionen, kardiale Probleme und Koagulopathien spielen pathophysiologisch eine wesentliche Rolle. Die atiologische Abklarung muss umfassend sein – der kindliche Schlaganfall ist eine „Multiple-Risk-Erkrankung“. Klassische Leitsymptome sind die akute Hemiparese, die faziale Parese und die Sprachstorung: Sie bestehen bei ca. 75 % der betroffenen Kinder. Aufgrund der mannigfaltigen, teils unspezifischen Symptome, der breiten Differentialdiagnosen und des fehlenden Bewusstseins fur den kindlichen Schlaganfall wird die Diagnose mittels Bildgebung oft erst nach einer erheblichen Zeitverzogerung gestellt (im Mittel nach 24 h). Die in speziellen Fallen moglichen Therapien wie die Thrombolyse oder die mechanische Thrombektomie konnen dabei grundsatzlich nur innerhalb weniger Stunden nach dem Ereignis eingesetzt werden – sie sind spezialisierten Zentren vorbehalten. Als neurologisches Residualsymptom steht die Hemiparese im Vordergrund, je nach betroffenem Infarktareal treten cerebrale Krampfanfalle, Sprachstorungen etc. auf. Die Auswirkungen auf Kognition und Verhalten werden in der Versorgungspraxis oft ubersehen und unterschatzt.

Aim For children with medical complexity, interdisciplinary treatment approaches are required to address the various aspects defined within the biopsychosocial model. Methods The present study identifies dimensions of the biopsychosocial model to generate a standardized visualized severity score for chronic neurological diseases in children. We demonstrate the score's applicability and usefulness in clinical practice among clinicians with and without pediatric board certification with the aid of illustrative patient cases. The results are compared by Spearman correlation coefficient. Results Nine dimensions were identified as the basis for the development of the score, which consists of five grades of severity for each of the selected neuropediatric subsections. All board-certified pediatricians would recommend the application of the severity score in clinical routine. Furthermore, a good correlation was revealed between direct and indirect (severity score) assessment. Interpretation The severity score developed in this study takes into account biopsychosocial aspects of chronic diseases while being comprehensible and easily applicable in clinical routine—a biopsychosocial signature serving as an excellent, striking communication basis within the interdisciplinary team. However, upcoming studies including more patient cases are needed for further refinement.

BackgroundIn recent years, there has been increasing research interest in improving diagnostic and management protocols in childhood arterial ischaemic stroke (AIS). However, childhood stroke comprises, in approximately equal parts, both arterial ischaemic and haemorrhagic stroke (HS).ObjectiveThe aim of this study was to focus on the aetiology, clinical presentation, treatment and short-term outcome of children with spontaneous intracranial bleeding in a university hospital and elucidate differences to childhood AIS.DesignWe performed a retrospective analysis of electronic medical records of children (28 days–18 years) diagnosed with HS between 2010 and 2016.ResultsWe included 25 children (male child, n=11) with a median age of 8 years 1 month. The most common clinical presentations were vomiting (48%), headache (40%) and altered level of consciousness (32%). In more than half of the patients, HS was caused by vascular malformations. Other risk factors were brain tumour, coagulopathy and miscellaneous severe underlying diseases. Aetiology remained unclear in one child. Therapy was neurosurgical in most children (68%). Two patients died, 5 patients needed further (rehabilitation) treatment and 18 children could be discharged home.ConclusionsHS differs from AIS in aetiology (vascular malformations as number one risk factor), number of risk factors (‘mono-risk’ disease), clinical presentation (vomiting, headache and altered level of consciousness) and (emergency) therapy.

Hintergrund Die zeitliche Verzögerung zwischen Symptombeginn und Diagnose ist eine Herausforderung in der Behandlung von Kindern mit arteriell ischämischem Schlaganfall. Frühere Studien zur klinischen Präsentation beschäftigten sich v. a. mit kumulativen Symptomen. Zielsetzung Ziel dieser Studie ist es, mögliche Symptommuster aufzuzeigen. Methoden In einer aktiven Beobachtungsstudie zwischen 01/2015 und 12/2016 (ESPED-Studie) wurden Kinder mit Erstdiagnose eines arteriell ischämischen Schlaganfalls eingeschlossen. Isoliert auftretende Erstsymptome wurden verschiedenen Symptomkombinationen gegenübergestellt. Zudem wurde untersucht, inwieweit ein als „akut“ oder „progredient“ klassifiziertes Auftreten der Symptome Rückschlüsse auf die zugrundeliegende Ätiologie erlaubt. Ergebnisse Es wurden 99 Kinder in die Studie eingeschlossen. Unabhängig vom Alter traten überwiegend fokale Symptome auf (86%). Krampfanfälle als Initialsymptom wurden insbesondere bei Säuglingen beschrieben (67%), wohin-gegen diffuse, unspezifische Symptome vor allem bei Vorschulkindern (38%) und älteren Kindern (59%) auftraten. Isoliert traten fokale Symptome bei 37 Kindern auf, 48 Kinder zeigten zusätzlich unspezifische Symptome, darunter auch 9 Kinder mit Krampfanfällen. Isolierte unspezifische Symptome zeigten sich lediglich bei 7 Kindern, 2 Kinder wurden nur mit Krampfanfällen symptomatisch. Die Akuität des Symptombeginns wurde bei 53/78 als „akut“ und bei “25/78 Fällen als „progredient“ klassifiziert, lieferte jedoch keinen Hinweis auf die zugrundeliegende Ätiologie. Schlussfolgerung Jedes neue fokal neurologische Defizit sollte unabhängig vom Auftreten (isoliert oder kombiniert, akut oder progredient) an einen kindlichen Schlaganfall denken lassen. Background Time delay between onset of clinical symptoms and diagnosis is a challenge in childhood arterial ischemic stroke. Most previous studies reported cumulative symptoms. Objective We attempted to identify typical symptom patterns and assessed their emergence in childhood stroke. Methods Prospective active surveillance in ESPED, a hospital based Pediatric Surveillance Unit for rare diseases in Germany, between January 2015 and December 2016. Case definition: first diagnosis of a radiologically confirmed arterial ischemic stroke. Symptom patterns were identified as occurring in isolation or in combination. We distinguished acute vs. progressive onset. We ascertained risk factors to identify the possible etiology. Results 99 children with childhood arterial ischemic stroke were reported. Focal symptoms were the predominant presenting feature (86%), independent of age. Seizures were more often seen in infants < 1 year (67%), whereas diffuse symptoms were more present in pre-school children (38%) and older children (59%). 37 children had focal features alone and 48 additional non-specific features, including 9 with seizures. Isolated non-specific features accounted for 7 cases, and 2 children had (focal) seizures as the only symptom. In 77% of all cases at least one risk factor was identified. The emergence of symptoms was acute in 53/78 cases and progressive in 25/78 cases. The pattern of emergence was unrelated to the underlying etiology. Conclusions Any new focal neurological deficit in isolation, or associated with seizures or further non-specific symptoms should alert to childhood stroke.

Several controlled studies have shown that in children with cerebral palsy, botulinum toxin A (BTX/A) can decrease muscular hyperactivity associated with spasticity and improve function. Studies have hitherto focused on the dimensions of impairment and functional limitations. In this pilot study with BTX/A in children with cerebral palsy, we used the Pediatric Evaluation of Disability Inventory (PEDI) to evaluate the effect of treatment. PEDI is a reliable and valid instrument that focuses on assessing disability in daily life. Patients with cerebral palsy (n = 17, median age 5.5 years, age range 2.5 to 16.5 years) were treated with BTX/A for pes equinus (n = 8) or adductor spasm (n = 9). PEDI assessment was carried out before and 1 month after the first treatment with BTX/A. Scaled scores were calculated according to the user’s manual for the Mobility domain with scores near „0” reflecting low capability and scores near „100” reflecting high capability. We found a significant improvement in the mobility domain-caregiver dimension from 52.3% ± 26.6% to 56.6% ± 26.7% (mean, standard deviation P < 0.05), as well as in the self care domain-functional skills from 63.6% ± 18.7% to 65.2% ± 19.6% (mean, standard deviation P < 0.05). Our data indicate that in young patients with cerebral palsy, BTX/ A therapy of the lower extremity can reduce the disability. For these patients PEDI is a valuable assessment instrument that reflects the effect of treatment with BTX/A on the disability. (J Pediatr Neurol 2003; 1(1): 29-34).

Active participation and the highest level of independence during daily living are primary goals in neurorehabilitation. Therefore, standing and walking are key factors in many rehabilitation programs. Despite inconclusive evidence considering the best application and efficacy of robotic tools in the field of pediatric neurorehabilitation, robotic technologies have been implemented to complement conventional therapies in recent years. A group of experienced therapists and physicians joined in an "expert panel." They compared their clinical application protocols, discussed recurring open questions, and developed experience-based recommendations for robot-assisted treadmill therapy (exemplified by the Lokomat, Hocoma, Volketswil, Switzerland) with a focus on children with cerebral palsy. Specific indications and therapeutic goals were defined considering the severity of motor impairments and the International Classification of Functioning, Disability and Health framework (ICF). After five meetings, consensus was found and recommendations for the implementation of robot-assisted treadmill therapy including postsurgery rehabilitation were proposed. This article aims to provide a comprehensive overview on therapeutical applications in a fast developing field of medicine, where scientific evidence is still scarce. These recommendations can help physicians and therapists to plan the child's individual therapy protocol of robot-assisted treadmill therapy.

In der Diskussion um den Nutzen der Heimmonitorversorgung insbesondere fur das Kollektiv fruhgeborener Kinder mit einem Apnoe-Bradykardie-Syndrom und/oder einer chronischen Lungenerkrankung spielt die Verkurzung des stationaren Aufenthaltes nicht nur unter soziookonomischen Gesichtspunkten sondern auch vor dem Hintergrund psychologischer und entwicklungsneurologischer Aspekte eine wichtige Rolle. Diesen Argumenten gegenuber stehen jedoch die Kosten dieser ambulanten Uberwachung sowie die fehlenden Standards fur die konsekutive Nachsorge dieser Kinder. Der folgende Artikel hat zum Ziel, die aktuelle Praxis der Heimmonitorverordnung und ihrer Nachbetreuung vor dem Hintergrund der Evidenzlage im Kontext der zu berucksichtigenden differenzialdiagnostischen Uberlegungen am Beispiel des etablierten Konzeptes der Neonatologie und des integrierten Sozialpadiatrischen Zentrums am Dr. von Haunerschen Kinderspital der Ludwig-Maximilians-Universitat in Munchen zu diskutieren.

Objective The objective of our study was to evaluate the knowledge about fetal alcohol spectrum disorders (FASD) and the implementation of the German guideline for FASD among different professionals in the health and social system and among parents with children with FASD. Methods A questionnaire about FASD, containing 20 items, was sent by post to all children's hospitals (n = 287), all hospitals for child and adolescent psychiatry (n = 173), all social paediatric centres (n = 162), all neuropaediatricians (n = 129) and all youth welfare offices (n = 672) in Germany. Furthermore a link to the questionnaire as online version was put in the member's newsletter by 14 relevant professional societies. Besides, the questionnaire was distributed personally to the attendees of the annual national FASD conference (n = 363). Results Altogether 428 persons took part in the survey. 273 participants were professionals and 155 parents of children with FASD. More than 95% of the professionals and parents knew that alcohol consumption during pregnancy constitutes a risk for the child. The prevalence of maternal alcohol consumption and of FASD was underestimated. Although approx. 70% of the professionals knew which disorders belong to FASD just a few could tell their specific deficits. Questions regarding effective intervention for children with FASD and the long-term outcome were only partially answered correctly. Discussion Professionals in the German health and social system are aware of FASD but underestimate the level of damage and the impact on every day functioning of the affected people.

Mindestens 14 % der deutschen Frauen trinken Alkohol in der Schwangerschaft. Schatzungsweise 4/1000 Kinder zeigen das Vollbild eines fetalen Alkoholsyndroms (FAS). Mogliche Risikofaktoren fur mutterlichen Alkoholkonsum und fur die Entwicklung eines FAS sollen bestimmt werden. Eine systematische Literaturrecherche von 2001 bis 2013 wurde zur Evaluation der durch europaische Studien belegten Risikofaktoren fur mutterlichen Alkoholkonsum durchgefuhrt. Diese Recherche wurde auf europaische Studien beschrankt, da die darin beschriebenen gesellschaftlichen Rahmenbedingungen und Entwicklungen am ehesten mit denen der deutschen Gesellschaft vergleichbar sind. Eine weitere systematische Literaturrecherche wurde zur Evaluation der durch internationale Studien belegten Risikofaktoren fur die Entstehung eines FAS durchgefuhrt. Die sehr geringe Anzahl europaischer Studien hatte hier zu keinem aussagekraftigen Ergebnis gefuhrt; deswegen wurde diese Recherche auf amerikanische und kanadische Studien ausgeweitet. Vor allem gut ausgebildete und gut verdienende, altere Frauen konsumieren Alkohol in der Schwangerschaft. Allerdings trinken auch vermehrt Frauen Alkohol, in deren Umfeld Alkohol oder Drogen konsumiert werden oder die (andere) Drogen nehmen oder rauchen. Frauen, die unter einer psychiatrischen Krankheit leiden, haben ebenfalls ein hoheres Risiko, Alkohol in der Schwangerschaft zu trinken. Haufig liegt eine Kombination verschiedener Risikofaktoren fur Alkoholkonsum in der Schwangerschaft vor. Ob eine alkoholkonsumierende schwangere Frau tatsachlich ein Kind mit fetalem Alkoholsyndrom (FAS) oder fetaler Alkoholspektrumstorung (FASD) zur Welt bringt, scheint neben Zeitpunkt, Dauer, Haufigkeit und Menge des Alkoholkonsums von weiteren Faktoren wie Ernahrung, Alter, Ethnizitat und genetischer Disposition abhangig zu sein. Die Kenntnis von Risikofaktoren kann sowohl zur Primarpravention des FAS durch Aufklarung und Unterstutzung der betroffenen Mutter und ihrer Familien als auch zur fruhzeitigen Diagnose des FAS bei betroffenen Kindern beitragen.

Objective To show non-inferiority of levetiracetam to sulthiame with respect to efficacy, tolerability and safety in benign epilepsy with centrotemporal spikes in a prospective, double-blinded randomized controlled trial. Methods A sample size of 60 subjects (treatment group) was calculated to show reliable statistical results for non-inferiority. A total of 44 patients could be randomly allocated to either (LEV or STM) treatment group. Explorative data analysis was performed to investigate differences in the number of treatment failure events (occurrence of a seizure during the observation period of 6 months) and total dropouts. In addition, information of the occurrence of adverse events was collected. Results 43 patients were analyzed. One patient had to be excluded due to protocol violation. Treatment failure events occurred in four patients (19.0%) in the LEV treatment group and in two patients (9.1%) in the STM treatment group, respectively, (p = 0.412). The number of dropouts due to adverse reactions was five in the LEV treatment group and one in STM treatment group (23.8% vs. 4.5%, respectively, p = 0.095). Severe adverse events occurred in patients treated with LEV (n = 2, 9.5%). The total number of dropouts due to either seizure recurrence or adverse events was significantly higher in the LEV group (n = 9, 42.9%) compared to the STM group (n = 3, 13.6%, p = 0.03). Interpretation The study results concerning non-inferiority were not conclusive, as the calculated sample size was not reached to support sufficient statistical power due to limited recruitment in a 26 months period. The rates of seizure free patients were [relatively] high in both groups. However, the results indicate that termination of drug treatment due to seizure recurrence or adverse events occurred more frequently in the LEV group compared to STM. Behavioral disturbances were the most common adverse event causing study termination.

Complementary and alternative medicine (CAM) is widely used by both physicians and patients with primary headache syndromes. Despite a considerable number of articles addressing CAM in primary headache syndromes, the overall evidence for CAM is still poor. The aim of this review was to give an overview of the current evidence of the main alternative therapies used in the treatment of primary headache syndromes of childhood. MEDLINE and Cochrane Library were systematically searched for articles dealing with complementary and alternative treatment or prophylaxis of headache and migraine published within the past 20 years.

Background The identified preventable risk factors for primary headache in adolescents are smoking; consumption of coffee or alcoholic mixed drinks; physical inactivity; muscle pain in the head, neck, or shoulder region; and chronic stress. Objective To investigate the interrelation of headache with other health complaints and the specificity of the above-mentioned risk factors for headache in adolescents. Methods A total of 1,260 students (grades 10 and 11) filled in questionnaires on headache, dietary, and lifestyle factors. The type of headache and health complaints such as dizziness, abdominal pain, musculoskeletal pains, symptoms of possible fatigue syndrome, and psychic complaints were assessed. Results Isolated headache was found in 18% of the headache sufferers; most frequently isolated tension-type headache (78.2%). Only among adolescents with a combination of headache (mainly migraine) and other health complaints, significant associations for almost all analyzed risk factors were found. The strength of the associations with the considered risk factors was very similar in all three analyzed strata except for considerably lower odds ratios for isolated headache. Conclusion All analyzed risk factors are nonspecific for headache in adolescents because they also increase the risk for other health complaints. Interventions, therefore, should consider a holistic approach focusing not only on headache but also on a broader spectrum of health complaints.

Introduction Several risk factors for headache have been identified, some of which are potentially amenable to interventions. The potential effect of such interventions can be predicted by the population-attributable risk fraction (PARF). We assessed PARFs of the the following risk factors: neck muscle pain, chronic stress, alcohol consumption, smoking, coffee consumption, and physical inactivity. We studied the maximal possible effect achievable by avoidance of these risk factors. Methods Two approaches to estimate PARFs are compared, which assess their cumulative and individual impact of risk factors by age: the Levin formula and the average attributable fraction. Results The overall impact for removal of all six risk factors amounts to 19.7% for the average attributable fraction. Neck tension and consumption of alcohol ranked as the strongest population-attributable risk factor for any headache. The potential impact for migraine was considerably higher (43.8%). With increasing age, the overall impact of risk factors on headache increases by 18.9%. Conclusion Based on the estimations of the most appropriate approach, up to 20% of headaches in general and up to 43% of migraine in adolescents might be preventable by removing risk factors amenable to intervention, with increasing proportions by age.

A sensitive and specific triage of patients with primary or secondary headache is a major concern in evaluating pediatric headache patients. History and physical examination are the major tools for differentiating primary headache disorders from symptomatic headaches caused by defined pathologies. If the criteria of the International Headache Society for a primary headache disorder are met, no further investigations are necessary. However, physicians should be familiar with subtle signs in history and physical examination that raise suspicion of intracranial pathology. These features, also named "red flags" and "relatively red flags," are outlined in detail in this review. Any red flag should prompt neuroimaging. In case of relatively red flags, a more restrained approach can be appropriate depending on the individual setting. Excessive concerns of patients and parents regarding an underlying pathology can constitute an indication for neuroimaging. Offering neuroimaging implicates the important issues of incidental findings and of "false reassurance." These risks should be discussed with patients and parents before the investigation. In any pediatric headache patient, regular clinical reevaluations should be warranted, even if neuroimaging is normal. The value of clinical follow-up examinations for a reasonable and reliable assessment of the patients cannot be overestimated.

Background Idiopathic intracranial hypertension (IIH) in children is a rare condition of unknown etiology and various clinical presentations. The primary aim of this study was to evaluate if our pediatric IIH study group fulfilled the revised diagnostic criteria for IIH published in 2013, particularly with regard to clinical presentation and threshold value of an elevated lumbar puncture opening pressure. Additionally we investigated the potential utilization of MR-based and fundoscopic methods of estimating intracranial pressure for improved diagnosis. Patients and methods Clinical data were collected retrospectively from twelve pediatric patients diagnosed with IIH between 2008 and 2012 and revised diagnostic criteria were applied. Comparison with non-invasive methods for measuring intracranial pressure, MRI-based measurement (MR-ICP) and venous ophthalmodynamometry was performed. Results Only four of the twelve children (33%) fulfilled the revised diagnostic criteria for a definite diagnosis of IIH. Regarding noninvasive methods, MR-ICP (n = 6) showed a significantly higher mean of intracranial pressure compared to a healthy age- and sex-matched control group (p = 0.0043). Venous ophthalmodynamometry (n = 4) showed comparable results to invasive lumbar puncture. Conclusion The revised diagnostic criteria for IIH may be too strict especially in children without papilledema. MR-ICP and venous ophthalmodynamometry are promising complementary procedures for monitoring disease progression and response to treatment.

Introduction: Acute ischemic stroke (AIS) is a rare event in infancy. Besides vasculopathy, thrombophilia, or cardiac disorders, cancer and chemotherapy are known predisposing factors for AIS. Leukemia can be associated with different abnormal coagulation parameters, but severe bleeding or thrombosis occurs rarely. Clinical Course: We report the case of a 2-year-old boy who was presented to our emergency ward after a prolonged seizure with right sided postictal hemiparesis. Cranial computed tomography scan revealed a large infarction and edema due to thrombosis of the left carotid artery, the middle cerebral artery, and the anterior cerebral artery. Laboratory workup showed 196 g/L leukocytes with 75% myeloid blast cells. Immediate exchange transfusion, hydration, and chemotherapy with cytarabine were started. During the hospital course intracranial pressure increased and the patient developed a unilateral dilated pupil unresponsive to light. Cranial computed tomography scan revealed a new infarction in the right middle cerebral artery territory. Refractory increased intracranial pressure and brain stem herniation developed, and the child died 3 days after admission to hospital. Conclusion: Seizures with postictal hemiparesis due to cerebral infarction can be a rare manifestation of acute myeloid leukemia. Leukocytosis and cancer-induced coagulopathy are main reasons for thrombosis and/or hemorrhage. High leukocyte counts need immediate interventions with hydration, careful chemotherapy, and perhaps exchange transfusion or leukapharesis. In the presence of thrombosis, anticoagulation must be discussed despite the risk of bleeding due to hyperfibrinolysis and low platelet counts. Mortality may be reduced by awareness of this rare presentation of leukemia and prompt institution of leucoreductive treatment.

To investigate frequency and phenotype of TNFRSF1A and MEFV mutations in childhood-onset multiple sclerosis (MS). Twenty-nine clinically well characterized patients were investigated for mutations in exons 2, 3, 4, and 6 of the TNFRSF1A gene and in exons 2, 3, 9, 10 of the MEFV gene. Standardized morbidity ratio (SMR) was used to assess whether the number of observed mutations was higher than expected. Eleven out of 29 patients tested positive for mutations. Heterozygosity for the TNFRSF1A R92Q (rs4149584) variant was found in 6/11 mutation-positive patients. The SMR for R92Q in our pediatric MS population was 4.6 (95% CI 1.7–10.0), 7.0 (95% CI 2.6–15.2), and 13.6 (95% CI 5.0–29.7), depending on reference population. Six patients carried at least one heterozygous MEFV mutation with SMRs of 21.4 (95% CI 7.9–46.6) and 14.6 (95% CI 5.4–31.9). Clinical characteristics of childhood MS patients with or without mutations did not differ significantly. Conclusion One third of our childhood MS patients had a heterozygous mutation in the TNFRSF1A and/or MEFV gene. This proportion by far exceeds the number of mutations expected and was higher than in adult MS patients, suggesting that these mutations might contribute to the pathogenesis of childhood MS.

Introduction Benign epilepsy with centrotemporal spikes (BECTS) is a common epilepsy syndrome in childhood. Besides the occurrence of seizures, mild cognitive impairments and behavioral problems affecting language skills, spatial perception, memory, executive function, and academic achievement might be present. There is no international consensus about the decision whether or not to treat affected children. The influence of treatment on cognitive functions is debated. Methods Patients diagnosed with BECTS were assessed in short term auditory memory, long-term verbal memory, intelligence and behavior using the “number recall” test from the Kaufman assessment battery for children, the “verbal learning memory test”, the “culture free intelligence test” and the “child behavior checklist” prior to a randomized controlled antiepileptic therapy and after a treatment period of 6 months with either sulthiame or levetiracetam. Results 43 of 44 randomized patients were analyzed. One patient had to be excluded due to protocol violation. Patients who completed the study showed a non-significant improvement in parent-reported behavioral problems under therapy. Cognitive skills were not affected. Conclusion The present data suggest that antiepileptic drug treatment of children with BECTS with either sulthiame or levetiracetam does not affect cognitive performance. Behavior improved in a subset of patients though not reaching statistical significance.

Introduction A total of 83% of children report headache during a 6-month period. The estimated 1-year prevalence of chronic daily headache (CDH) in children is at least 1 to 2%. Muscle pain is associated with headache severity and chronicity. Muscle pain can be associated with active muscular trigger points, a functional concept still remaining a controversy. An integrated approach including bio-behavioral management is accepted as standard treatment but does not provide sufficient pain relief in all patients. Objective We report the individual clinical course of five adolescents with treatment-refractory CDH associated with focal muscle pain. We describe a concept of short-term integrative intervention including botulinum toxin (StiBo) in a personalized “follow the referred pain pattern” injection regimen with the focus on long-term follow-up. Results StiBo showed short-term efficacy on headache frequency and severity. In the long-term follow-up, CDH was not existent in any of the patients. Conclusion The treatment may have enabled the patients to draw attention away from a repeated circle of muscle-triggered pain and withdrawal of daily activities toward self-driven activities, thereby potentially preventing the development of further chronification. To prove this hypothesis, a prospective, placebo-controlled study in young adolescents with CDH should be initiated including objective outcome parameters on muscular level.

Epilepsiezentren werden als uberregionale Kompetenzzentren definiert, die uber spezielle Expertise und eine besondere Ausstattung zur ambulanten und zur stationaren Versorgung von Patienten mit Epilepsien und verwandten Erkrankungen verfugen. Die Kernaufgaben umfassen neben der Diagnostik und Therapie auch Aspekte der Weiterbildung beteiligter Berufsgruppen sowie der Schulung von Patienten und Laien. Spezielle Anforderungen beziehen sich auf institutionelle, personelle und apparative Ebenen. Epilepsiezentren konnen sich Schwerpunkte geben, die z. B. die Epilepsiechirurgie oder die Betreuung mehrfach behinderter Menschen betreffen. Ferner kann die Spezialisierung auf Ebene verschiedener Altersgruppen erfolgen (Kinder, Jugendliche und Erwachsene). In dem vorliegenden Beitrag werden die besonderen Anforderungen an Epilepsiezentren fur Kinder und Jugendliche v. a. mit dem Schwerpunkt der Epilepsiechirurgie diskutiert.