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.

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.

Background and aims: To compare findings of cranial ultrasound (US) and magnetic resonance imaging (MRI) of the neonatal brain. Methods: A retrospective population based cohort study of premature infants with a very low birth weight (VLBW) of less then 1500 grams who were admitted to our neonatal unit on day 1 (period: 2007 to 2009). All infants underwent cranial US on day 1, 3, 7, 14, 28, 42 monthly thereafter and at term corrected age. Infants who were eligible for this study obtained a MRI at term (37 to 42 weeks gestational age) corrected age. Retrospectively, we compared the cranial US findings as a predictor of a wide spectrum of pathology on MRI. Results: Paired MRI and US studies were performed in (n=140) VLBW infants who were born at a median gestational age of 28 (range: 22+1 to 34+5) weeks and a median birth weight of 1020 (range: 335 to 1495) grams. US predicted some MRI findings accurately: germinal layer haemorrhage (GLH), cystic lesions, intraventricular haemorrhage (IVH) and severe white matter (WM) echogenicity on US for the presence of WM haemorrhagic parenchymal infarction on MRI. Other MRI changes were less well-predicted: delay in maturation and myelination, reduced cortical folding, congenital malformations, punctate lesions and mild or no WM echogenicity on US for the presence of normal (n=112) WM signal intensity on MRI. Conclusions: MRI of the neonatal brain might shed light on the origin of brain lesions causing long-term neurodevelopmental sequelae and might change our perinatal and neonatal management.

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:

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.

Gegenstand unserer Untersuchungen ist die Anwendung neuer Methoden der nichtlinearen Zeitreihenanalyse für die Analyse von Elektroenzephalographien im Kindes- und Jugendalter. Im Mittelpunkt des Interesses stehen Vorhersagbarkeit von Anfällen, Fokuslokalisation, Therapiebeurteilung und prognostische Aussagen vor Beendigung einer antikonvulsiven Behandlung. Das von uns entwickelte Verfahren transformiert die Zeitreihe des digitalisierten EEG in eine symbolische Zeitreihe durch Berechnung der Häufigkeiten von Permutationen (Symbolen) von 4 jeweils benachbarten Werten. Diese werden jeweils in einem Zeitintervall fixierter Länge (2 Sekunden) ermittelt, welches entlang der Zeitreihe verschoben wird. Die Entropien der Häufigkeiten werden schließlich kanalweise berechnet und graphisch dargestellt. Die Verarbeitung der Daten kann in kurzer Zeit erfolgen und erscheint relativ artefaktunabhängig. Um eine Anwendbarkeit dieses Verfahrens zu prüfen, untersuchten wir die EEG-Daten von Patienten mit fokalen und generalisierten Anfallsleiden. Bei den untersuchten Patienten lassen sich Veränderungen in der Signalkomplexität des iktualen bzw. interiktualen EEG-Verlaufes ausmachen. Kinder mit einer Rolando-Epilepsie zeigen sowohl vor wie auch kurz nach antikonvulsiver Einstellung im Bereich des Rolando-Fokus eine Entropieminderung unabhängig vom Auftreten hypersynchroner Aktivität. Diese könnte auf eine Funktionsstörung im betroffenen Areal hinweisen. Nach mehrmonatiger Behandlung sind diese Seitendifferenzen rückläufig. Bei generalisierten Epilepsien lassen sich am Beispiel der Absencen im Anfall bzw. bei Hyperventilation vor dem Anfall Entropieveränderungen feststellen. Im Gegensatz zu vielen anderen Methoden der nichtlinearen Zeitreihenanalyse bietet unsere Methode der Permutationsentropie eine schnelle Berechenbarkeit und gestattet die Bearbeitung auch längerer EEG-Registrierungen. Sie ermöglicht einen umfassenden Überblick und zeigt Langzeitveränderungen und Periodizitäten auf. Möglicherweise lassen sich durch die vorgestellte Methode auch funktionelle und prognostische Aussagen treffen. Dazu sind weitere Untersuchungen geplant.

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.

Enteroviruses are common with infections of the CNS, such as encephalitis and myelitis, but they may cause various diseases in different organ systems, particulary with fatal outcome. Pleconaril is a new orally acting antiviral drug with broad anti-picornavirus activity, which provides to treat rhinoviral and enteroviral infections. To explain the importance for clinical use, we report a case of severe enteroviral infections of the CNS, treated by Pleconaril. A 14 year old girl presented with a severe polio- like myelitis including flaccid paraparesis and urinary incontinence due to Coxsackievirus-infection. Because of prolonged course and virus persistence we treated with Pleconaril, after treatment a remarkable improvement could be noticed, continency and the ability to walk without aid were regained within a few weeks. With the development of new antiviral substances we are now given more opportunities to treat infectious conditions of the central nervous system. We suggest to include enteroviruses in diagnostic procedures since there is an effective treatment with the new drug Pleconaril available.

Nystagm is found as a symptom in different conditions that can affect various organs of the body. It always requires sophisticated diagnostic procedures, including radiologic imaging, consultation of ENT and ophthalmologic specialists and electrophysiological measurements. Case report: The child presented with ocular flutter that parents noted from about the 9th week after birth, otherwise he had developed normally so far. MRI of the brain showed enlargement of internal and external cerebrospinal space, so that a neurodegenerative disorder was suspected. On examination cerebrospinal fluid had normal cell count and differenciation, protein and lactat content. The diagnosis was made by the experienced ophthalmologist, who discovered altered pigmentation in iris and retina, disclosing ocular albinism. Visual evoked potentials showed an asymmetry in cortical potentials that confirmed the diagnosis. Conclusion: Underlying disorder causing the nystagm in our patient is an ocular albinism. Albinism is caused by a disorder in biosynthesis and distribution of melanin, it affects about ½0,000 people. In the case of our patient the enlargement of cerebrospinal space and albinism seem to appear coincidentally, further ophthalmologic and neuropediatric follow-ups are definitely necessary.