Intrathecal Baclofen therapy in Germany: Proceedings of the IAB-Interdisciplinary Working Group for Movement Disorders Consensus Meeting.

Continuous intrathecal Baclofen application (ITB) through an intracorporeal pump system is widely used in adults and children with spasticity of spinal and supraspinal origin. Currently, about 1200 new ITB pump systems are implanted in Germany each year. ITB is based on an interdisciplinary approach with neurologists, rehabilitation specialists, paediatricians and neurosurgeons. We are presenting the proceedings of a consensus meeting organised by IAB-Interdisciplinary Working Group for Movement Disorders. The ITB pump system consists of the implantable pump with its drug reservoir, the refill port, an additional side port and a flexible catheter. Non-programmable pumps drive the Baclofen flow by the reservoir pressure. Programmable pumps additionally contain a radiofrequency control unit, an electrical pump and a battery. They have major advantages during the dose-finding phase. ITB doses vary widely between 10 and 2000 µg/day. For spinal spasticity, they are typically in the order of 100-300 µg/day. Hereditary spastic paraplegia seems to require particularly low doses, while dystonia and brain injury require particularly high ones. Best effects are documented for tonic paraspasticity of spinal origin and the least effects for phasic muscle hyperactivity disorders of supraspinal origin. Oral antispastics are mainly effective in mild spasticity. Botulinum toxin is most effective in focal spasticity. Myotomies and denervation operations are restricted to selected cases of focal spasticity. Due to its wide-spread distribution within the cerebrospinal fluid, ITB can tackle wide-spread and severe spasticity.

Fetal growth restriction is not associated with a reduced risk for bilateral spastic cerebral palsy in very-low-birthweight infants.

OBJECTIVE: To evaluate the influence of confounding and sampling bias on the relationship between fetal growth restriction in a very-low-birthweight-defined cohort (VLBW, < or =1500 g) and bilateral spastic cerebral palsy (BSCP) at early school-age. METHODS: Three hundred twenty-four of 407 long-term survivors of a regional cohort of VLBW newborns were followed until age 6 years. We categorized as small for gestational age (SGA) all infants whose birthweight Z-score was below -2 relative to published reference values. Uni- and multivariable logistic regression models were fit to estimate the risk of BSCP associated with SGA in the total sample, in subsamples defined by gestational age, and in a gestational age-matched case-control sample. RESULTS: In the total sample, no child below 28 weeks was SGA, and no child above 32 weeks had an appropriate birthweight for gestational age (AGA). The prevalence of BSCP was 14% in AGA and 2% in SGA infants. In both uni- and multivariable logistic regression analyses of the total sample, SGA was associated with a prominently reduced risk of BSCP (odds ratios range from 0.1 to 0.2, all 95% confidence limits exclude 1.0). However, analyses performed in samples defined by different gestational age cutoffs (24--31 weeks, 28--31 weeks) and in a sample using three gestational age-matched controls per BSCP-case did not show a protection by growth restriction (odds ratios range from 0.8 to 2.2, all 95% confidence limits include 1.0). CONCLUSIONS: In VLBW-defined samples, the apparent protective effect of SGA for BSCP can be explained, at least in part, by the highly skewed distribution of SGA over the available gestational age range. From this follows that study cohorts should be defined by gestational age and not by birthweight. In distorted samples like this one, even controlling for gestational age does not reduce the illusion of a reduced cerebral palsy risk for growth restricted infants. Only restriction of the sample by gestational age and/or matching for gestational age reveals the absence of this apparent protective effect.

Improved neurological outcome following early anatomical correction of transposition of the great arteries.

The incidence of neurological residuals following anatomical correction of transposition of the great arteries (d-TGA) has not been described so far. Clinical examination, EEG recordings, and computed tomography (CT) scans were carried out in a consecutive series of 38 children with d-TGA surviving anatomic corrective surgery. The patients were classified into one of three groups according to the type of operation: 15 patients after two-stage approach (TSA) (Stage 1: pulmonary artery banding+aortopulmonary shunt; Stage 2: anatomic correction); 12 patients with primary anatomic correction within the first 2 weeks of life (early switch, ES); 11 patients with primary anatomic correction later in infancy (later switch, LS). In 26 patients (68%) we found no abnormalities on neurologic examination, CT scan, or EEG. Four patients suffered from spastic hemiplegia, 3 of these had cortical brain damage visible on CT scan, and 3 had focal epilepsy as well. In 2 otherwise clinical normal patients cortical infarction could be seen on a CT scan. Thus, in 5 cases (13% of 38 patients) cerebral infarcts were diagnosed by CT scan. The cortical vascular infarction was seen in 4 patients after TSA and in 1 after LS. In 6 patients we found other neurological abnormalities. Early anatomic correction in patients with d-TGA reduces the risk of cortical vascular infarction.

Successful vascular decompression in an 11-year-old patient with trigeminal neuralgia.

CASE REPORT: We present the case of an 11-year-old boy who was suffering distinct trigeminal neuralgia. At the age of 3 years, the patient had contracted a severe Epstein-Barr virus infection and developed mild meningoencephalitis. Magnetic resonance imaging scans showed a slight enhancement in the pontomesencephalic cistern as well as a neurovascular conflict at the right trigeminal nerve. Intraoperatively, thickened fibrous tissue was found that was attached to both the trigeminal nerve and the superior cerebellar artery. Microvascular decompression using Gore Tex as tissue implant brought immediate relief. DISCUSSION: Trigeminal neuralgia in pediatric patients is very rare. We present a case of typical trigeminal neuralgia in a child, demonstrating the pathogenesis of the neurovascular conflict due to subarachnoidal adhesions after meningoencephalitis.

[Sudden infant death syndrome in Hamburg. An epidemiologic analysis of 62 cases]. / Sudden Infant Death Syndrome in Hamburg. Eine epidemiologische Analyse von 62 Fällen.

During a two years period (1978/79) all 62 victims of sudden and unexplained death in Hamburg have been analysed retrospectively. For statistical analysis a control group of 297 healthy life births was used. The overall incidence of SIDS amounted to 2.4 per 1,000 life births. In the VLBW children the figure raised up to 9.1%. SGA children were highly overrepresented. Amongst Turkish mothers there were only two cases (3.2%) significantly less than with other groups. (9.1% of all life births in 1979 were Turkish). An ethnological factor can be discussed. In the lowest social class more SIDS cases were encountered. The analysis of the circumstances around death confirmed previous results. A consecutive and longitudinal analysis of SIDS cases seems mandatory for evaluating the effect of preventive methods.

Development of very low birth weight infants: a regional study of 371 survivors.

We re-examined 371 infants with birth weights less than 1501 g at a corrected age of 18-20 months. This sample amounted to 91% of such infants admitted to one of the six neonatal intensive care units in Hamburg between July 1983 and 1986. The neurological examination and a developmental evaluation using the Griffith Developmental Scale revealed higher rates of abnormalities than in most other studies. Fifty-five children (14.8%) suffered from cerebral palsy, classified in 45 as spastic diplegia, in 5 as spastic tetraplegia, in 1 as spastic hemiplegia and in 4 as dystonia. Of the children, 41 (11%) showed minor neurological deviations (hyperactivity, clumsiness, intention tremor). The development of 30 children (8%) without neurological abnormalities was moderately retarded (DQ 80-89, corrected for gestational age [GA]). Nineteen children (5%) were severely retarded (DQ less than 80, corrected for GA) and four children (1.5%) were blind due to retrolental fibroplasia. An isolated delay of speech development was found in 5 children. Seventy children (18.9%) had a major and 87 children (23.5%) a minor handicap.

Development and perinatal risk factors of very low-birth-weight infants. Small versus appropriate for gestational age.

371 long-term surviving very low-birth-weight (VLBW) infants born between July 1983 and June 1986 with a birthweight under 1501 g were followed-up. This sample amounted to 91% of such infants of six neonatal intensive care units in Hamburg (Germany). A neurological examination and a developmental evaluation using the Griffith Developmental Scale were carried out at the age of 18 to 20 months, corrected for gestational age. Ninety-six of the 371 infants were small for gestational age (SGA), 275 appropriate for gestational age (AGA). Striking differences between these two groups were found concerning perinatal risk factors and neurological outcome. Maternal risk factors associated with intrauterine growth retardation such as maternal toxemia and signs of fetal stress were found in a high percentage of the mothers of SGA-children, factors associated with premature labor and chorioamnionitis in mothers of AGA-children. Cerebral palsy was detected in only 7% of the SGA-children but 17.5% of the AGA-children. The difference in the development of cerebral palsy was attributed mainly to different postconceptual ages of the SGA- and AGA-children. In general, minor neurological abnormalities were detected in as many as 30% of SGA- and only 15.3% of AGA-children. None of the SGA-children over 33 weeks of gestational age developed cerebral palsy, but 25% minor neurological abnormalities. As to cerebral palsy the prognosis of SGA-infants with a very low birthweight is not different from AGA-infants with a similar gestational age. Regarding the development of minor neurological abnormalities, however, intrauterine growth retardation seems to be a risk factor independent from gestational age.

Etiological classification of cerebral palsy in very low birthweight infants.

As part of a longitudinal follow-up program a single case analysis of the history of 53 children with a birthweight of less than 1501 g, who had developed cerebral palsy, was performed. In 17 children (32%) a definite explanation for the neurological impairment and in another 17 (32%) at least a possible explanation could be found. In 19 of the 53 children (36%) the history was uneventful and did not offer a causal event. The common documentation in obstetric and pediatric case files left the cause of cerebral palsy undetected in one third of the cases.

Diplegic cerebral palsy in Swedish term and preterm children. Differences in reduced optimality, relations to neurology and pathogenetic factors.

An unselected series of 93 Swedish diplegic children born in 1969-1976 and subgrouped into 49 term (TDC) and 44 preterm (PDC) cases were analyzed for differences in reduced optimality in pre- and perinatal conditions, these also being related to degree of handicap, associated neurology and conventional pathogenetic grouping. Comparisons of the reduced optimality with those of a dyskinetic and a control series were also made. TDC were shown to have more severe handicaps and more additional neurologic abnormalities than PDC. The profile of reduced optimality was weighted in TDC to items pointing to fetal maladjustment/deprivation and birth asphyxia and in PDC to items accompanying preterm birth and to postpartal items. The optimality of diplegics was in general more reduced than in controls and less than in dyskinetics. This was especially true for TDC. Differences in the background mechanisms of the diplegia between TDC and PDC were indicated from dissimilarities in the combined patterns of reduced optimality and conventional pathogenetic grouping. Postpartal complications predominated among PDC. A prepartal factor as the only cause of the diplegia was likely in 41% of TDC, and as a contributory cause in another 24%. Birth asphyxia, present in 31% of the TDC, was never the only risk factor among infants born at term.

[Intracranial metastasis in neuroblastoma]. / Intrakranielle Metastasierung bei Neuroblastom.

Computed tomography is the most useful diagnostic method to demonstrate the pathophysiological consequences of secondary craniocerebral neuroblastoma. Hyperostotic metastases with subperiostal epidural tumour plaques as well as tumour deposits displacing and compressing adjacent venous sinuses show up well. Moreover, post-contrast computed tomography is a sensitive method for controlling patients under cytostatic or radiation therapy. In general, cranial computed tomography should be a part of basic diagnostics in neuroblastoma.

Increased risk of spastic diplegia among very low birth weight children after preterm labor or prelabor rupture of membranes.

Our objective was to study the association of spastic diplegia (SDP; N = 29) with the initiator of preterm birth in a regional cohort of 312 6-year-old very low birth weight children (< or =1500 gm). We determined the prevalence of SDP among those children born after idiopathic preterm onset of labor (POOL) or prelabor rupture of membranes (PROM) (12% SDP), and among those born after pregnancy-induced hypertension or other medical indications for preterm delivery (4% SDP). Stratification showed that 83% of the children with diplegia were born after POOL or PROM. The threefold increased risk of SDP among those children born after POOL or PROM compared with the remainder of the cohort (crude odds ratio 3.2, 95% confidence interval 1.2 to 8.5) remained elevated after controlling for perinatal and neonatal variables (odds ratio 2.4 to 2.7) in logistic regression models. We conclude that birth after POOL or PROM increases the risk of SDP among very low birth weight children and speculate that this might be related to infectious processes leading to both POOL or PROM and SDP.

[The epidemiology of blastomyces infections with special reference to postoperative intensive care wards (author's transl)]. / Beitrag zur Epidemiologie von Sprosspilzen unter besonderer Berücksichtigung einer operativen Intensivstation.

230 strains of Blastomyces were isolated during a nine month's period in the course of routine laboratory examinations of faecal and other specimens. Candida albicans was the most frequently present (151), followed by Candida tropicalis (39) and Torulopsis glabrata (16). During an environmental study in a postoperative intensive care unit 426 swabs were taken. 88 strains of Blastomyces were cultured from 75 swabs. The observations indicate that Candida albicans as a free-living organism lacks favourable conditions for multiplication in sick rooms and wards, and that it does not play the same role in cross-infection as does, e.g., Psudeomonas aeruginosa or strains of Klebsiella. The same apparently also applies to Candida tropicalis and Torulopsis. Measures to prevent infection with yeast-like fungi in hospitals are reviewed.

Neonatal onset of rash in Still's disease.

We report a neonate with a rash that appeared on the second day of life in association with elevated body temperatures and increased C-reactive protein levels. The rash was evanescent and recurred on a daily basis during the first year of life. At 15 months, the infant developed swelling of the right knee joint. Early-onset systemic juvenile rheumatoid arthritis can mimic congenital infections and should be considered in the differential diagnosis of neonatal exanthemas.

Sudden infant death syndrome in Hamburg. An epidemiological analysis of 150 cases.

A retrospective analysis was carried out of 150 SIDS cases seen in the five year period between 1979 and 1983 in Hamburg, Germany. The overall incidence was 2.3 per 1,000 live births. 82% of all infants and 100% of the preterm infants (corrected according to gestational age) dying of SIDS, died within the first 6 months of life. Infants of mothers less than 18 years of age, infants with more than 2 siblings, infants with birthweight less than 1,500 g and SGA-preterm infants were found to be high-risk subgroups. A high percentage (20% of all cases, 43% of infants with mothers less than 18 years) were underweight at death (under the 3rd weight-percentile). The study emphasizes the complexity of internal and external factors in the etiology of SIDS.

Intra-atrial malpositions of Silastic catheters in newborns.

The intra-atrial position of Silastic catheters placed in the center of the right atrium was assessed by two-dimensional echocardiography in 25 newborns. In 32% of the infants, the catheter tip was located in the patent foramen ovale, and in another 40% of the infants, the catheter tip was located close to the patent foramen ovale. This placement increases the risk of systemic air and fat embolism. Consequently, the tip of Silastic catheters should be placed just behind the confluence of the superior-inferior vena cava and the right atrium.

Development of a regional cohort of very-low-birthweight children at six years: cognitive abilities are associated with neurological disability and social background.

The authors studied the intelligence, memory, visuomotor skills and la nguage of 298 six-year-old children with very low birthweight (VLBW) (less that 1500g). Of 591 VLBW childern born July 1983 to June 1986 within 50km of the centre of Hamburg, Germany, 330 were traceable at age six years and 298 of these were seen by a neuropaediatrician and a psychologist; the other 19 were too severely disabled for psychological assessment with the standardized tests used. The mean memory performance of VLBW children at age six years was below the standard mean in all diagnostic and socio-economic subgroups. As expected, visuomotor development was clearly influenced by neurological but not socio-economic status. Intelligence and language skills were much more closely related to socio-economic background that to neurological morbidity. However, VLBW children with hyperactivity, clumsiness or cerebral palsy differed significantly in intelligence and visuomotor performance from those without neurological symptoms.