Laminaire distributie van de cellen van oorsprong van descenderende vezels uit de motorische cortex in de kat en de aap

De cortex cerebri omvat een zeer grate populatie neuronen. Een deel van deze corticale neuronen distribueert vezels naar subcorticale structuren. De corticale gebieden waaruit deze vezels afkomstig zijn, het verloop en de eindigingsgebieden van deze corticate vezels werden aanvankelijk met anterograde degeneratie technieken en later oak met anterograde autoradiografische technieken bestudeerd. Met behulp van deze technieken werd aangetoond, dat descenderende vezels uit motorische corticale gebieden in de aap en de kat in het striatum in de thalamus in structuren in de hersenstam en in het ruggemerg eindigen. Tot voor kart was weinig bekend over de anatomische kenmerken van de cellen van oorsprong van de descenderende corticale vezels. De in het laatste decennium ontwikkelde retrograde transport technieken maakten het echter mogelijk om deze neuronen door middel van retrograde neuronale labeling te identificeren. In de experimenten die in de hoofdstukken V en VI van dit proefschrift zullen worden beschreven, werden de cellen van oorsprong van descenderende vezels uit motorische corticale gebieden van de kat (hoofdstuk V) en van de aap (hoofd- stuk VI) met behulp van de retrograde HRP transport techniek geidentificeerd. Vervolgens werd in beide diersoorten de areale en laminaire distributie van de cellen van oorsprong van deze vezels bestudeerd. Volgens electrofysiologische onderzoekingen bestaat een deel van de corticale vezels naar hersenstam kernen en thalamus in werkelijkheid uit collateralen van corticospinale-o£ pyramidebaanvezels. In hoofdstuk VII van dit proefschrift zullen experimenten worden beschreven, waarin in de kat en de rat het bestaan van dergelijke corticospinale axon collateralen naar het mesencephalon en de laterale thalamus anatomisch werd onderzocht door middel van de retrograde fluorescente neuronale dubbel labeling techniek. Om de bevindingen in deze te beschrijven experimenten in hun juiste perspectief te zien, zullen in hoofdstu

Preoperative prediction of postoperative cerebellar mutism syndrome. Validation of existing MRI models and proposal of the new Rotterdam pCMS prediction model

Purpose Postoperative cerebellar mutism syndrome (pCMS) is a complication that may occur after pediatric fossa posterior tumor surgery. Liu et al. developed an MRI-based prediction model to estimate pCMS risk preoperatively. The goal of this study was to validate the model of Liu et al. and if validation was not as sensitive in our group as previously described to develop an easy to use, reliable, and sensitive preoperative risk prediction model for pCMS. Methods In this study, 121children with a fossa posterior tumor who underwent surgery at ErasmusMC/Sophia Children’s Hospital, the Netherlands between 2004 and 2018 could be included. Twenty-six percent of them developed pCMS. Preoperative MRI were scored using the Liu et al. model. Results The Liu et al. model reached an accuracy of 78%, a sensitivity of 58%, and a specificity of 84% in our cohort. In a new risk model some of the variables of Liu et al. were included as well as some of the recently described preoperative MRI characteristics in pCMS patients by Zhang et al. The new model reached an accuracy of 87%, a sensitivity of 97%, and a specificity of 84% in our patient group. Conclusion Because the Liu et al. model did not provide an as accurate risk prediction in our cohort as was expected, we created a new risk prediction model that reached high model accuracy in our cohort that could assist neurosurgeons in determining their surgical tactics and help prepare high risk patients and their parents for this severe complication

Ketogenic diet treatment in recurrent diffuse intrinsic pontine glioma in children: A safety and feasibility study

Background: The mean overall survival rate of children with diffuse intrinsic pontine glioma (DIPG) is 9–11 months, with current standard treatment with fractionated radiotherapy and adjuvant chemotherapy. So far, novel therapeutic strategies have not yet resulted in significantly better survival. The main source of energy for glioblastoma cells is glucose. Therefore, metabolic alterations induced by the use of the extremely carbohydrate-restricted ketogenic diet (KD) as adjuvant therapy are subject of interest in cancer research. Procedure: This study explores the safety and feasibility of the KD in children with recurrent DIPG and no remaining treatment options. Safety was defined as the number of adverse effects. Feasibility was defined as the number of patients who were able to use the KD for three months. Coping of patients and parents was measured with questionnaires. Results: Three of 14 children referred to our hospital between 2010 and 2015 were included. Two patients completed the study, and one died before the end of the study. Hospitalizations were needed for placing a nasogastric tube (n = 1) and epileptic seizures (n = 1). Adverse effects related to the diet were mild and transient. Parents were highly motivated during the study. Conclusion: Use of KD is safe and feasible, but the effect on survival has to be proven in a larger cohort of children who start the KD earlier after diagnosis, preferably as adjuvant therapy to fractionated radiotherapy

Diagnosis of Guillain–Barré syndrome in children and validation of the Brighton criteria

To describe the key diagnostic features of pediatric Guillain–Barré syndrome (GBS) and validate the Brighton criteria. Retrospective cohort study of all children (<18 years) diagnosed with GBS between 1987 and 2013 at Sophia Children’s Hospital, Erasmus MC, Rotterdam. Clinical information was collected and the sensitivity of the Brighton criteria was calculated. 67 children (35 boys) were included, with a median age of 5.0 years [interquartile range (IQR) 3.0–10.0 years]. Bilateral limb weakness was present at hospital admission in 93% of children, and at nadir in all patients. Children presented with tetraparesis in 70% or with paraparesis in 23%. Reduced reflexes in paretic limbs were observed at hospital admission in 82% and during follow-up in all children. The progressive phase lasted median 6 days (IQR 3–8 days) and less than 4 weeks in all children. A monophasic disease course was seen in 97%, including 5 children with a treatment-related fluctuation. Two children had a later relapse at 9 weeks and 19 weeks after onset. 77% of the children showed an elevated protein level in CSF. Nerve conduction studies showed evidence for a poly(radiculo)neuropathy in 91% of the children. 46 children had a complete data set, the sensitivity of the Brighton criteria level 1 was 72% (95% CI 57–84) and 96% (95% CI 85–99) for level 2 and 98% (95% CI 88–100) for level 3. The majority of the pediatric GBS patients presented in this cohort fulfilled the current diagnostic criteria

Activities and participation of children and adolescents after mild traumatic brain injury and the effectiveness of an early intervention (Brains Ahead!): Study protocol for a cohort study with a nested randomised controlled trial

Background: Approximately 20% of children and adolescents who have sustained mild traumatic brain injuries ma

Fatigue and physical functioning in children with multiple sclerosis and acute disseminated encephalomyelitis

Background and Objective: Fatigue and physical impairments are a major concern in children with multiple sclerosis (MS) and after acute disseminated encephalomyelitis (post-ADEM). We here aimed to evaluate the interaction between fatigue, exercise capacity, motor performance, neurological status, and quality of life (HRQoL). Methods: In this cross-sectional study, data of 38 children (MS n = 22, post-ADEM n = 16), aged 4–17 years attending our national pediatric MS center, were studied. Fatigue was measured with the Pediatric Quality of Life Multidimensional Fatigue Scale, exercise capacity with the Bruce Protocol, motor performance with the Movement Assessment Battery for Children second edition, HRQoL with the Pediatric Quality of Life Questionnaire, and extent of disability with the Expanded Disability Status Scale (EDSS). Results: Children with MS and post-ADEM experienced more fatigue (p < 0.001), reduced exercise capacity (p < 0.001), and impaired motor performance (p < 0.001), despite low scores on the EDSS. Fatigue, but not the other parameters, was significantly correlated with HRQoL. Fatigue was not correlated with exercise capacity. Conclusion: We confirm the major impact of fatigue on quality of life in children with MS and post-ADEM. Fatigue was not explained by reduced exercise capacity or impaired motor performance. An important finding for clinical practice is that the low EDSS score did not reflect the poor physical functioning

Evaluation of management and guideline adherence in children with mild traumatic brain injury

Aim: To evaluate the management and guideline adherence in children with mild traumatic brain injury (MTBI) in emergency departments (ED) in the Netherlands. Methods: A multicentre cohort study was conducted, including children younger than 18 years with MTBI who presented within 24 hours after trauma in the ED of hospitals in the southwest region of the Netherlands, in 2014. Primary outcome measures for management were percentages of performed computed tomography (CT) scans and hospital admissions. Guideline adherence was defined as percentages of correctly following the guideline. Secondary outcome measures were differences in management and guideline adherence between hospitals. Results: About 563 patients were analysed. Hospital admission was the most frequently performed management type (49.2% hospital admission vs. 30.9% CT). In only 49.7% of patients, the guideline was followed correctly. A substantial overuse of hospital admission (35%) and underuse of CT (40.1%) were found. Percentages of hospital admission and CT varied between 39.4–55.6% and 23.3–44.1%, respectively, across hospitals. Percentages of correctly following the guideline varied between 39.2–64.9% across hospitals. Conclusion: These findings suggest that physicians in the participating hospitals prefer hospital admission of children with MTBI instead of CT despite the current recommendations of the national MTBI guideline in the Netherlands

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 dis