Historic review: a short history of neuropediatrics in Germany between 1850 and 1950.

Since the middle of the nineteenth century, there has been increasing knowledge about the diagnosis and therapy of diseases of the central and peripheral nervous system and muscle in children. The leading causes were cerebral palsy, epilepsy, inflammatory and degenerative diseases, and the innate reduction in intelligence. Because of the often lack of healing options, many pediatricians had little interest in treating these children and left their care to the pedagogues and psychologists. Pioneers of child neurology in the German-speaking countries were Ludwig Mauthner, Franz von Rinecker, Julius Zappert, and Georg Peritz. Especially with the beginning of the National Socialist terror regime, rigorous treatment methods were used, the care facilities for disabled children were closed, and these were either actively murdered or interned under inhumane conditions. In this time, some specialists in neuropediatrics had an ambivalent position between the care for their patients and the selection for their elimination. After 1950, new findings in diagnostics and therapy, especially from Anglo-American countries, played a significant role in the rise of neuropediatrics in Germany.

Historic review: the foundation-period and the first 15 years of the "Gesellschaft fuer Neuropaediatrie" (GNP) between 1950 and 1990.

After the end of Nazi terrorism with many crimes against neuropsychiatric and disabled people, from 1950 on detection and treatment of these diseases in children and adolescents grew continuously: in the 50s and 60s, several German books on neuropediatric topics were published, a journal was founded, and junior pediatricians established many scientific contacts with foreign neuropediatricians. In 1972, the team of the Kehl-Kork Epilepsy Clinic invited neuropediatric colleagues from Germany, Austria, and Switzerland for a first joint workshop. On March 4th, 1975, the Neuropaediatric Society of the German-speaking countries was founded in Heidelberg and its goals, as well as guidelines for membership, were formulated. The first annual meeting took place 1975 in Heidelberg as well. Between 1975 and 1990, the number of members of the GNP continued to increase from 140 to more than 230, despite strict admission criteria. The president was elected for a one-year term and was responsible for organizing the annual meeting, which took place alternately in Germany, Austria or Switzerland. The continuity of the society was ensured by secretary and treasurer as well as several longtime assessors. The meetings covered the entire spectrum of neurological and neuropsychological disorders in children. In addition to the age-dependent clinical investigation, the most important diagnostic methods were electrophysiology, in particular, the EEG and EMG, the new possibilities of cerebral imaging utilizing X-ray computed tomography and ultrasound, and biochemical analyzes for detection of metabolic diseases. Research projects were mostly carried out in university institutions and were only partially multi-center or financed with public funds. International contacts took place on many levels, e.g. through scholarships and personal exchanges with European and US scientific societies and institutions. Unfortunately, the opportunity to exchange ideas with colleagues from the German Democratic Republic (GDR) was limited. Several working groups addressed controversial issues of developmental neurology, epileptology, and alternative therapies. With the establishment of social pediatric centers in Germany from the late 1980s, there was an increasing demand for well-educated and experienced neuropediatricians.

Effects of Levetiracetam and Sulthiame on EEG in benign epilepsy with centrotemporal spikes: A randomized controlled trial.

PURPOSE: BECTS (benign childhood epilepsy with centrotemporal spikes) is associated with characteristic EEG findings. This study examines the influence of anti-convulsive treatment on the EEG. METHODS: In a randomized controlled trial including 43 children with BECTS, EEGs were performed prior to treatment with either Sulthiame or Levetiracetam as well as three times under treatment. Using the spike-wave-index, the degree of EEG pathology was quantified. The EEG before and after initiation of treatment was analyzed. Both treatment arms were compared and the EEG of the children that were to develop recurrent seizures was compared with those that were successfully treated. RESULTS: Regardless of the treatment agent, the spike-wave-index was reduced significantly under treatment. There were no differences between the two treatment groups. In an additional analysis, the EEG characteristics of the children with recurrent seizures differed statistically significant from those that did not have any further seizures. CONCLUSION: Both Sulthiame and Levetiracetam influence the EEG of children with BECTS. Persistent EEG pathologies are associated with treatment failures.

[How can child development disorders be recognized in early childhood?]. / Wie erkennt man Entwicklungsstörungen im frühen Kindesalter?

In the last 10 years beside the well known developmental criterias like turning from back to prone, crawling, standing up, free walking, speaking of the first words and the position-reactions, the exact observation of the spontaneous movements, of handling-planing and the speech development could be detected as more important factors for the examination of development. Certain parameters of explanation of developmental disorders are to differenciate from uncertain and only probably one. Of special interest are the frequent problems like excessive crying, feeding- and sleeping disorders.

Tyrosine hydroxylase deficiency causes progressive encephalopathy and dopa-nonresponsive dystonia.

Tyrosine hydroxylase (TH) is the key enzyme in the biosynthesis of the catecholamines dopamine, epinephrine, and norepinephrine. Recessively inherited deficiency of TH was recently identified and incorporated into recent concepts of genetic dystonias as the cause of recessive Dopa-responsive dystonia or Segawa's syndrome in analogy to dominantly inherited GTP cyclohydrolase I deficiency. We report four patients with TH deficiency and two with GTP cyclohydrolase I deficiency. Patients with TH deficiency suffer from progressive infantile encephalopathy dominated by motor retardation similar to a primary neuromuscular disorder, fluctuating extrapyramidal, and ocular and vegetative symptoms. Intellectual functions are mostly compromised. Prenatally disturbed brain development and postnatal growth failure were observed. Treatment with levodopa ameliorates but usually does not normalize symptoms. Compared with patients with dominantly inherited GTP cyclohydrolase I deficiency, catecholaminergic neurotransmission is severely and constantly impaired in TH deficiency. In most patients, this results not in predominating dystonia, a largely nondegenerative condition, but in a progressive often lethal neurometabolic disorder, which can be improved but not cured by L-dopa. Investigations of neurotransmitter defects by specific cerebrospinal fluid determinations should be included in the diagnostic evaluation of children with progressive infantile encephalopathy.