Functional imaging studies on responders and non-responders to therapeutic interventions in obese children are rare. We applied fMRI before and after a one-year sports therapy in 14 obese or overweight children aged 7-16 years. During scanning, participants observed a set of standardized pictures from food categories, sports, and pleasant and neutral images. We were interested in alterations of the cerebral activation to food images in association with changes in the BMI-standard deviation score (BMI-SDS) after therapy and therefore separated the observation group into two outcome subgroups. One with reduction of BMI-SDS >0.2 (responder group) and one without (non-responder group). Before therapy fMRI-activation between groups did not differ. After therapy we found the following results: in response to food images, obese children of the responder group showed increased activation in the left putamen when compared with the non-responder group. Pleasant images evoked increased insula activation in the responder group. Only the responder group showed enhanced activity within areas known to store trained motor patterns in response to sports images. Both the putamen and the insula are involved in the processing of emotional valence and were only active for the therapy responders during the observation of food or pleasant stimuli. Elevated activity in these regions might possibly be seen in the context of an increase of dopaminergic response to emotional positive stimuli during intervention. In addition, sport images activated motor representations only in those subjects who profited from the sports therapy. Overall, an altered response to rewarding and pleasant images and an increased recruitment of motor engrams during observations of sports pictures indicates a more normal cerebral processing in response to these stimuli after successful sports therapy in obese children. (C) 2014 Elsevier B.V. All rights reserved.

Case Report: Sturge-Weber syndrome is a rare disease (incidence of 1:20.000). Most likely caused by impaired proliferation of vessels while first trimester of pregnancy, its results a lepto-meningeal angiomatosis with naevus flammeus, glaucoma, and epilepsy. We report a case about a girl with Surge-Weber Syndrome, which was diagnosed at first year of life. While treatment epilepsy at our department, a growth velocity less than percentile 3 was noted. Growth hormone deficiency was confirmed by two GH-stimulations tests, magnetic resonance imaging did not show any malformation. Since human growth hormone (hGH) replacement therapy, an effective in reducing the deficit in height has been observed.

Key words acute disseminated ­enzephalomyelitis - neuropediatrics - immunoadsorption - plasmapheresis

Fragestellung: EEG-Veränderungen stellen einen wichtigen Parameter bei der Verlaufsbeurteilung von ZNS-Erkrankungen wie Meningitiden bzw. Enzephalitiden dar. Aufgrund möglicher Fluktuationen während einer Ableitung ist eine Beurteilung der Dynamik oft nur durch paralleles Sichten aufeinanderfolgender Registrierungen möglich. Ausgehend von der Theorie Pritchards und Dukes, dass EEG-Signale auch als chaotische Muster betrachtet werden können, ergibt sich mithilfe der Zeitreihenanalytik die Möglichkeit der mathematischen Berechnung von nicht-linearen Dynamiken.

Introduction: Stiff-persons syndrome (SPS) is a rare disorder characterized by progressive stiffness of trunk muscles, sudden occurring painful spasms, vegetative and psychiatric symptoms. SPS is strongly associated with autoimmune diseases and rarely observed in children. Here we present a case, the diagnostic strategy and successful treatment of SPS in a pediatric patient.

Introduction: Non-ketotic hyperglycinemia is a rare metabolic disorder. Postnatal patients show symptoms of respiratory failure, hypotonia, weakness of drinking and seizures. The further course is characterized by stato- and psychomotoric retardation, spasticity, blindness and epilepsy. The EEG shows a burst-suppression pattern to hypsarrhythmia. In the MRI of the brain abnormalities of the corpus callosum could be found.

Objective: Food cues yield different patterns of brain activation in obese compared with normal-weight adults in prefrontal and limbic/paralimbic areas. For children, no mapping studies comparing representation sites for food and other stimuli between obese and normal-weight subjects are available. Design: We used a cross-sectional design of two age-matched subject groups to investigate differences in brain activation in response to visually presented food, pleasant, and neutral pictures between obese/overweight and normal children. Subjects: 22 overweight/obese children were compared with 22 normal-weight children. Measurements: Functional magnetic resonance imaging (of the whole head during perception of visually presented stimuli), psychological testing, and psychophysical measures of heart rate deceleration were assessed. Results: Obese children showed higher activation of the dorsolateral prefrontal cortex (DLPFC) in response to food pictures. In addition, DLPFC activation was negatively correlated with self-esteem. In contrast, normal-weight children showed higher activation of the caudate and hippocampus specific to food pictures, and of the anterior cingulate cortex and thalamus to visual cues in general. In response to food stimuli, obese children showed a heart rate deceleration correlating positively with activation of the ventrolateral prefrontal cortex. Conclusion: Obese children react to food stimuli with increased prefrontal activation, which might be associated with increased inhibitory control.

Familial hemiplegic migraine (FHM) and alternating hemiplegia of childhood (AHC) are severe neurological disorders that share clinical features. Therefore, FHM genes are candidates for AHC. We performed mutation analysis in the CACNA1A gene in a monozygotic twin pair with clinical features overlapping with both AHC and FHM and identified a novel de novo CACNA1A mutation. We provide the first evidence that a CACNA1A mutation can cause atypical AHC, indicating an overlap of molecular mechanisms causing AHC and FHM. These results also suggest that CACNA1A mutation scanning is indicated in patients with a severe neurological phenotype that includes paroxysmal (alternating) hemiplegia.

Objective: Obesity is one of the world's greatest health problems. It becomes increasingly important in pediatrics. Searching for individual factors of obesity genesis we performed a study of normal and overweight children and adolescents. We used neurophysiological examinations like event related potentials (ERP), functional MRI (fMRI) and peripheral-physiological parameters such as skin conductance and heart rate variability and we collected psychological examination data.

Background: Viewing pictures activates the human brain in a complex manner. Stimulated brain areas can be identified by electrophysiological methods even in children.Subjects and method: Event related potentials (ERP) were recorded with EEG-surface electrodes in children and adolescents who were shown pictures of increasing complexity. Pictures were categorized into morphologic features such as brightness, color and pattern as well as semantic content.Results: Presentation of comics and unstructured pictures, similar in color and brightness, revealed ERP with nearly identical early components (P 100). However, substantial differences were found in frontocentral brain areas. A negative wave at approximately 400 ms was recorded only when comics were shown.Conclusions: Semantic scenes provoked significant different ERP, which indicate an early involvement of complex neuronal networks. These results could be applied in the research of pediatric cognitive disturbances.

Hintergrund: Patienten und Methode: Ergebnisse: Schlussfolgerung: Background: Subjects and method: Conclusions:

Alpers-Huttenlocher syndrome (AHS) is a progressive neurologic disorder characterized by the clinical triad of psychomotor regression, refractory seizures (often with a focal component) and liver disease after a normal development over the first weeks to years of life. Recently it has been shown that autosomal recessive mutations of the mitochondrial (mt) polymerase gamma (POLG) result in mtDNA depletion and cause AHS.

Objective: Searching for the neurophysiological background of the motivation of eating behavior we currently conduct a clinical trial with 45 obese children and adolescents and 20 age-related controls. Before and after multimodal therapy we acquired psychological data, event related potentials (ERP), functional MRI and peripheral physiological parameters. First results of ERP and peripheral physiology will be presented.

Background: Hereditary deficiency in polymerase gamma (POLG) leads to reduction of functional mitochondrial DNA. Several diseases may result, for instance Alpers syndrome, juvenile spinocerebellar ataxia with epilepsy as well as progressive external ophthalmoplegia.

An 8-year old girl from Kazakhstan after unremarkable pregnancy and perinatal period, presented at our hospital with progressive loss of mental functions and gait ataxia since the age of six. Neurologic examination revealed spastic tetraplegia with preference of the lower limbs, mild ataxia, dysarthria and bilateral large and unresponsive pupils.

Background: Cognitive Evoked Potentials (CEP) reflect the processing of visual, acoustic, somatosensory or olfactory information. Frontal cortex is attached great importance in controlling food intake. Within an obesity-study we analysed CEP after presentation of different pictures to draw conclusions on semantic picture processing.

The Chiari-syndrome Type I being a malformation of the posterior cranial fossa often leads to syringomyelia. The disease becomes apparent in adolescence with kyphoscoliosis, headache, vertigo, ataxy, hearing loss, partial paralysis and other neurological disorders. The onset is typically characterized by dissociated anesthesia, due to the frequent localization of the syrinx in the neighborhood of the posterior column of the spinal cord. It is reported a case of an 15-years-old-girl suffering from intensive headache, recurrent left-sided paresthesias and progredient scoliosis. Somatosensory evoked potentials of left ulnar and tibial nerves revealed a complete loss of cortical response. Diagnostic imaging showed an Chiari-malformation I with herniation of cerebellar tonsils and secondary syringomyelia of the cervical spinal cord. After surgical treatment with posterior fossa decompression, C1 laminectomy and partial excision of cerebellar tonsils the patient had a clear improvement in symptoms. Postsurgical the width of cervical syrinx decreases. Now somatosensory evoked potentials of the left ulnar and tibial nerves show amplitude-reduced cortical responses with a normal latency.