[Differentiation of cerebrospinal immunoglobulins in mumps meningoencephalitis]. / Die Differenzierung der liquoreigenen Immunglobuline bei der Mumps-Meningoencephalitis.

In 24 children with mumps-meningitis or -meningoencephalitis the process dynamics of the locally produced CSF immunoglobulins were quantified by end-point laser-nephelometry. In 8 cases there was a local production of all the immunoglobulins IgG, A and M. In 6 cases the humoral immune response was still detectable after six weeks, in one child after one year. In all cases of meningoencephalitis locally produced immunoglobulins became detectable and their levels were higher than in meningitis. The locally produced CSF immunoglobulins reached the maximum in the first disease stage. There was no shift from IgM to IgG production in the CSF compartment. At present time there is no explanation for the wide variation of the humoral immune response within the central nervous system.

[Physiological proteinuria in the newborn]. / Physiologische Proteinurie bei Neugeborenen.

The levels of the individual proteins albumin, caeruloplasmin, immunoglobulin G, and alpha 2-macroglobulin were determined by Laurell's electroimmunoassay in the serum and urine of 127 children. Their age ranged from 29 gestational weeks to 13 years. Highest levels of the proteins in the urine were found during the first days of life. The values did not depend on gestational age. The urine concentrations are influenced by glomerular filtration and the reabsorption of proteins and water. The steady state of these functions can be characterized by the serum/urine concentration ratios and the clearance of the proteins. In the first days after birth the permeability of the renal barrier is maximal, the concentration ratios are low and increase markedly up to the end of the first three months; after that, they continue to rise only slightly. The clearances of all the examined individual proteins decrease between term and the third month of life, thereafter increasing until late childhood. The selectivity of the glomerular filtration also shows its highest rate of development during the first days after term.

[Changes in the granulocyte membrane in mucoviscidosis]. / Veränderung der Granulozytenmembran bei Mucoviscidose.

Important polymorphonuclear leukocyte (PMN) functions are dependent on an intact cytoskeleton, consistent of the microtubulus and microfilament system. This can be assayed from the mobility of fluorescence labelled Concanavalin A (Con A) receptor complexes on the cell surface. Con A shows an uniform surface distribution on cells with an intact microtubulus system, whereas it's disruption causes a Con A cap formation. The alteration of the microtubulus and microfilament systems induces a patchy Con A distribution. Only 44% PMNs of 20 children with cystic fibrosis exhibited with a random Con A fluorescence distribution an intact cytoskeleton compared to 69% control PMNs. But 32% cystic fibrosis PMNs and 23% control PMNs showed a Con A capping phenomena, which is associated with a microtubulus alteration. In addition, 24% cystic fibrosis PMNs demonstrated a patchy fluorescence, representing a microtubulus and microfilament disruption, compared to 8% patched control PMNs. This study supports the idea, that the chronic bacterial infections cause in cystic fibrosis PMNs a cytoskeleton defect, which represents as a leukocyte-function defect an additional infection promoting factor.

[Clinical findings and treatment of paraquat poisoning in childhood]. / Klinik und Therapie der Paraquat-Intoxikation im Kindesalter.

Two of three children with Paraquat poisoning were treated with hemoperfusion. All patients survived. One patient had an acute renal failure after the intoxication. A percutaneous poisoning is discussed in one child with large skin necrosis after direct contact with Paraquat. An irreversible lung fibrosis did not set in. The prognosis for a patient with Paraquat intoxication depends on a speedy and consistent detoxication from both the bowel and from the blood by hemoperfusion. This is the only way to survive the acute intoxication and to avoid lung fibrosis, that would lead to death.

Development of the blood-CSF barrier.

The development of the blood-CSF barrier was investigated by establishing total protein, individual protein levels and cell-counts in the CSF and serum of 185 healthy children, with an age range from 27 fetal weeks to 16 years. Albumin, alpha 2-macroglobulin and immunoglobulin G were found in all CSF specimens. With the exception of IgG, their concentrations decreased continuously until the end of the first year of life. IgM was not present in the CSF and IgA was found in only a few cases. Serum:CSF ratios of individual proteins increased continuously from the fetal period to the first year and there was a decline in barrier selectivity during the same period. Maturation of the barrier was maximal during the third month of life, decreasing quickly thereafter. The barrier condition of preterm infants was closely related to conceptional age, but not to the time of birth.

Two cases of coxsackie B2 infection in neonates: clinical, virological, and epidemiological aspects.

Two cases of neonatal coxsackie virus B2 infection are described. One infant presented with meningitis and enteritis, the other with rhinitis, meningoencephalitis, and enteritis. Both infants made good recoveries. The virus infection could also be demonstrated in all nonimmune family members, most of whom gave a history of recent mild febrile disease (pharyngitis, diarrhea). Enterovirus infections may be suspected in cases of neonatal meningitis or myocarditis associated with gastrointestinal signs, especially 1. when it is during the hot season July-October, 2. when there has been febrile illness in other family members recently. For an effective and rapid isolation of the agent, rectal swabs or stool specimens not only from the patient, but also from household contacts should be sent to the virus laboratory on several consecutive days. Meningitic infection may be proved by an early c.s.f. sample. For serodiagnosis a first blood specimen should be drawn as soon as possible, a second one some days later. The importance of rapid virological diagnosis and of stringent hygienic measures to prevent spread of the infection is stressed.

Transient EEG patterns during sleep in healthy newborns.

24 healthy full-term newborns underwent polygraphic recordings of EEG, EMG, EOG, ECG, abdominal and thoracic respiration during day-time-sleep. Transient EEG patterns (rhythmic alpha and beta activity, spikes/sharp waves and frontal sharp transients) were visually evaluated and quantified. Rhythmic alpha activity is not very prominent. It is found in all states of sleep and appears only as scattered waves and interrupted sequences. Their duration varies from 1 to 5 sec. Rhythmic beta activity is a feature of quiet and active-REM sleep, repeatedly in spindle-shaped formations. "Typical" sleep spindle activity, however, is very rare. In a few cases rhythmic beta activity also appears in the form of the delta brush pattern. Spikes/sharp waves are sporadic in appearance, non-repetitive in nature and occur mostly multifocal with parietal predominance. They are more frequent in quiet sleep (1 per 2.3 min on average) than in active-REM sleep (1 per 18 min), and were rare in transitional states of sleep (1 per hour). "Frontal sharp transients" show great variations of configuration. They occur most frequently in quiet sleep. Often they appear only unilaterally. Their voltage maximum is frontal, fronto-temporal or praecentral. They are more frequent in REM sleep after wakefulness than after quiet sleep. The individual as well as the sleep-state related frequencies of spike/sharp waves and "frontal sharp transients" tend to differ widely with a range of a factor 10. In some of the newborns these graphoelements are not observed at all during certain sleep phases.

[Infectious gastroenteritis in childhood (author's transl)]. / Infektiöse Gastroenteritis bei Säuglingen und Kleinkindern.

Stool specimens from 126 children with acute gastroenteritis and from 42 controls were screened bacteriologically and virologically including electron-microscopy. Rota viruses were found in 36.5% of the children with diarrhoea, enteropathogenic E. coli in 13.5% and candida in 7.1%. These pathogens were not found in the controls. Enteroviruses were found in almost the same frequency in both groups (13.5% in children with gastroenteritis, 16.6% in the controls). The etiologic role of adenoviruses, which could be seen in 5.6% of the diarrhoea group, remains unexplained.

Late elevation of CSF proteins in three cases of mumps meningoencephalitis.

Three children suffered from mumps meningoencephalitis and pneumonia. The clinical course was uneventful. After 2 weeks antibiotic therapy for pneumonia a repeat lumbar puncture showed a massive increase of CSF protein levels. This phenomenon was transitory. It is explained by an obstruction in CSF pathway in the course of mumps meningoencephalitis.

[Neonatal ventriculitis. A case report (author's transl)]. / Ventrikulitis des Neugeborenen. Ein Fallbericht.

Meningitis of the newborn is often accompanied by ventriculitis. This may be one of the reasons for the still unfavourable prognosis of neonatal meningitis. In a few cases we achieved sterile ventricular fluid with additional intraventricular application of antibiotics. An examination of ventricular fluids should be performed when there is the slightest suspicion of ventriculitis. Early institution of additional antibiotic therapy intraventricularly as well as some of the newer antibiotics (p.e. Cefotaxime) seem to produce better results. We observed the following complications of meningoventriculitis: hydrocephalus, porencephaly, and multicystic encephalopathy.

Computed tomography in Pelizaeus-Merzbacher disease.

CT findings in two related males suffering from the classical X-linked recessive form of Pelizaeus-Merzbacher disease (PMD) are described. CT revealed marked cerebellar atrophy and focal areas of demyelination of cerebral white matter in a 25-year-old patient. This agrees with known neuropathological changes. However, CT was normal in the 14-year-old nephew, although his neurological symptoms were nearly as severe as his uncle's. Judging from this observation and from the scant information in the literature it seems that CT in classical PMD is normal in the first decade and is therefore not helpful in confirming the diagnosis of PMD at an early stage.

Immunocytological studies in a case of Bruton's disease.

Immunopathological findings in a case with infantile X-linked agamma globulinemia (Bruton's disease) are reported and compared to similar data from two cousins of the patient. Besides a severe decrease in B-lymphocytes in blood and lymph node, residual B-cells showed an almost total defect in Ig surface receptor expression. In addition, there was an unusual lymphocyte population which apparently exhibited E-rosette formation (i.e. T-cell characteristics) and phagocytosis. The data suggest that in Bruton's disease there may be a disturbance of the T-cell system besides the well known B-cell deficiency.

[Micromelic type of spondylo-meta-epiphyseal dysplasia (author's transl)]. / Mikromele Form der Spondylo-meta-epiphysären Dysplasie.

A 3 6/12 years old girl with micromelic type of spondylo-meta-epiphyseal dysplasia combined with hepatosplenomegaly and muscular hypotonia is described.

[Blood-cerebrospinal fluid barrier and the local immune response in the course of meningitis in childhood]. / Blut-Liquor-Schranke und lokale Immunantwort im Verlauf von Meningitiden im Kindesalter.

The condition of the blood-CSF barrier can be evaluated by the simultaneous quantitation of marker proteins in serum and cerebrospinal fluid. The concentration ratios of albumin and alpha 2-macroglobulin, plotted versus the hydrodynamic radii are used as permeability parameter. There are wide ranging barrier disturbances during the early stages of meningitis. In mumps meningitis only slight disturbances were found, meanwhile in bacterial meningitis the barrier permeability was strongly increased. In both disease groups one may detect secretory fractions of both immunglobulins G and A. In some cases of mumps meningitis a prolonged humoral immune reaction was found.