[Perinatal brain damage. Conclusions concerning etiology].
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Biomedical subjects
Publications and source records attributed to F J Schulte.
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A considerable number of high risk brain tumours require an interdisciplinary approach with surgery, radiation and chemotherapy rather than one single therapeutic regimen. Children with a medulloblastoma must be subdivided into four different risk groups depending on the size of the tumour, on the age of the child and on cerebral spinal fluid cytology findings. The treatment protocol for ependymomas depends on the location of the tumour and on histological grading. Pineal region tumours can be successfully removed; further therapy depends on the type of the tumour. Children with hemispheric high grade astrocytomas gain substantially from local radiation therapy. The GPO (Gesellschaft für Pädiatrische Onkologie) has most recently taken the initiative to found a pediatric neuro-oncology working group in order to establish and to evaluate different treatment modalities.
In Germany, the relative frequency of pineal region tumours seems to be much higher than hitherto assumed. At the University Hospital Hamburg, from 1980-1985 17 children with pineal region tumours were encountered amongst 102 children with CNS tumours. Two-cell-type germinoma is the most frequent pineal region tumour. Cerebrospinal fluid cytology is highly successful in identifying this germ cell tumour. Surgical removal has become a reasonably safe procedure in the treatment of pineal region tumours and was successful in all 10 cases so treated. In addition, our patients with two-cell-type germinomas received craniospinal axis radiation. All children, treated by both surgical removal and craniospinal axis radiation are so far relapse-free and are functioning on a pretreatment level.
This is a report of a 1 1/2-year-old male with megaloblastic anaemia and a progressive neurological disorder clinically resembling leucodystrophy. The child was exclusively breast fed and his mother had been a strict vegetarian for more than 4 years before the child was born. After Vitamin B12 therapy the anaemia disappeared and the neurological condition dramatically improved.
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Since 1981, 96 infants considered at increased risk of SIDS underwent home monitoring for prolonged sleep apnea: 23 infants after a near miss for SIDS event, 28 siblings of a SIDS victim and 45 infants with a variety of perinatal risk factors. For a total of 65 infants the course of home monitor surveillance was completed by September 1984 with a duration ranging from 6 to 15 month: 26% (4/15) of the near miss for SIDS group, 23% (3/13) of the SIDS siblings and 13% (5/37) of the perinatal risk cases developed more than one prolonged apneic episode with additional symptoms requiring vigorous intervention by parents. Two infants of the perinatal risk group became SIDS victims: despite an apnea alarm after 15 seconds the parents were unable to resuscitate their infant in one case, the other died from SIDS about 4 month after monitoring was discontinued because of an uneventful course and normal polygraphic sleep recordings The large number of prolonged apneas requiring intervention and the two SIDS cases (3% of the total study group) indicate a considerably increased risk of prolonged life-threatening sleep apnea and SIDS in the population monitored.
By means of polygraphic sleep recording, the sleep apnea profile with respect to the number and duration of inactive, obstructive and mixed apneic episodes as well as periodic breathing has been investigated in infants born preterm at 40, 52 and 64 weeks conceptional age and compared to that of term infants. At 40 weeks preterm infants showed significantly more apnea and periodic breathing compared to term infants. The difference was essentially due to obstructive and mixed apnea in non-REM sleep. There was a sharp decrease in all apneic variables--inactive, obstructive and mixed apnea as well as of periodic breathing--at 52 weeks conceptional age in infants that were previously preterm. Both groups exhibited a rather identical sleep apnea profile at 64 weeks. Two prospectively studied infants in the preterm group later became SIDS victims. One of them might have been identified as being at risk on the basis of his apnea profile compared to the normative data now available.
Polygraphic recordings were made on 10 preterm infants recovering from respiratory distress syndrome and 12 healthy preterm control infants at 40, 52, and 64 weeks' conceptual age to study the influence of respiratory distress syndrome on the development of the sleep apnoea profile. Two significant differences were found: infants with respiratory distress syndrome not only had a lower incidence of non-obstructive apnoea and periodic breathing at 40 weeks but also a persistently higher incidence of obstructive and mixed apnoea at 52 and 64 weeks' conceptual age; the latter finding being related to non-rapid eye movement sleep only. While the lower incidence of both types of apnoea at 40 weeks suggests an advanced maturation of respiratory drive, the persistence of obstructive and mixed apnoea related to non-rapid eye movement sleep may reflect the impact of respiratory distress syndrome on airway structures.
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The clinical course of the early onset gangliosidoses can be explained on the basis of the developmental time course of different brain structures and functional systems. In particular, the maturation of the four basic motor control systems--spinal cord, brain stem and cerebellum, basal ganglia, cerebral cortex--determines the appearance of certain motor deficits. In late onset GM2 gangliosidoses, however, regional preference of the storage process has to be assumed in order to explain certain characteristic features of the disorder. An attempt is made to explain cellular dysfunction in GM2 storage disorders on the basis of developmental defects and/or destruction of the endoplasmatic reticulum and Golgi apparatus distortion of the neuronal geometry. Hyperirritability and epilepsy are possibly due to a dysequilibrium between excitatory and inhibitory postsynaptic influences caused by the distortion of the synaptic geometry on the nerve cell surface. Again, the clinical appearance of hyperirritability and the type of epilepsy are dependent upon the developmental age of the affected nervous system.
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We retrospectively investigated the influence of gestational age, perinatal risk, and the duration of incubator care periods in 193 surviving preterm infants with a gestational age between 28 and 36 weeks raised in our intensive care nursery incubators from 1965--1967. 24 (12.4%) of the children showed definite neurosensory hearing defects, which were particularly encountered in the high frequencies. No correlation could be substantiated between hearing difficulties and hyperbilirubinemia, streptomycin application and gestational age. This study does not support the assumption that the duration of noise exposure in currently used incubators is a major determinant for the development of deafness in otherwise healthy preterm infants. Our study did show, however, a strong correlation between the sum of all perinatal risk factors and neurosensory hearing loss.
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The extra- vs intrauterine development of both visual and auditory cortical evoked response patterns was compared at 33, 37 and 40 weeks conceptional age. The maturation of visual cortical evoked responses is retarded in infants at 37 and 40 weeks conceptional age when born with a gestational age of less than 32 weeks, which thus implies a long extrauterine life span. The maturation of the auditory cortical evoked responses is not influenced by premature exposure to the extrauterine environment. The results are explained on the basis of the particular central nervous system growth spurt periods and a thus defined vulnerable period of different brain structures.
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