[Treatment of an uncommon case of a cardiogenic shock : Simultaneous use of a VA-ECMO and an Impella-CP®]. / Die Behandlung eines ungewöhnlichen kardiogenen Schocks : Simultaner Einsatz einer VA-ECMO und einer Impella-CP®.

We report the case of a 48-year old woman where probably the intramuscular administration of glucocorticoids by an orthopedist induced a pheochromocytoma crisis. The development of a cardiogenic shock with a cardiac arrest made the use of a venoarterial extra corporeal membrane oxygenation (VA-ECMO, Cardiohelp®, Maquet, Rastatt) necessary. To treat a pulmonary edema under VA-ECMO an Impella-CP® (Abiomed, Aachen) was implanted. A coronary angiography, endomyocardial biopsies and a computer tomography were performed. Finally a 7 cm × 5 cm tumor mass in the left adrenal medulla was detected. The blood values presented high levels of catecholamines. The total results of the examinations led us to the final diagnosis of a pheochromocytoma which was finally surgical removed.

The panorama of cerebral palsy in Sweden part XII shows that patterns changed in the birth years 2007-2010.

AIM: This was the 12th population-based study to explore the epidemiology of cerebral palsy (CP) in western Sweden. METHODS: From 2007 to 2010, there were 104 713 live births in the area. We analysed the birth characteristics, aetiology and neuroimaging findings, calculated the prevalence and compared the results with previous study cohorts. RESULTS: Cerebral palsy was found in 205 children, corresponding to a crude prevalence of 1.96 per 1000 live births. The gestational age-specific prevalence for <28 gestational weeks was 59.0 per 1000 live births, 45.7 for 28-31 weeks, 6.0 for 32-36 weeks and 1.2 for >36 weeks. Hemiplegia accounted for 44%, diplegia for 34%, tetraplegia for 5%, dyskinetic CP for 12% and ataxia for 3%. Neuroimaging showed maldevelopment in 12%, white matter lesions in 49%, cortical/subcortical lesions in 15% and basal ganglia lesions in 11%. The aetiology was considered prenatal in 38%, peri/neonatal in 38% and remained unclassified in 24%. CP due to term or near-term asphyxia had decreased. CONCLUSION: A nonsignificant decrease in CP prevalence was seen in term-born children. Hemiplegia was still the most prevalent CP type, while the prevalence of dyskinetic CP had decreased. One in two children had white matter lesions, indicating late second- or early third-trimester timing.

Non-infectious risk factors for different types of cerebral palsy in term-born babies: a population-based, case-control study.

OBJECTIVE: To identify non-infectious antenatal and perinatal risk factors for cerebral palsy (CP) and its subtypes in children born at term. DESIGN: A population-based, case-control study. SETTING: The western healthcare region of Sweden. POPULATION: A population-based series of children with CP born at term during 1983-94 (n=309) was matched with a control group (n=618). METHODS: A total of 62 variables, maternal characteristics, and prepartal, intrapartal and postpartal variables were retrieved from obstetric records. Both univariate and multivariate analyses were performed for spastic and dyskinetic CP, and for the total CP group. MAIN OUTCOME MEASURES: Cerebral palsy (CP) and subtypes. RESULTS: Univariate analysis resulted in 26 significant risk factors for CP. Birthweight (OR 0.54, 95% CI 0.39-0.74), not living with the baby's father (OR 2.58, 95% CI 1.11-5.97), admittance to a neonatal intensive care unit (NICU) (OR 4.43, 95% CI 3.03-6.47), maternal weight at 34 weeks of gestation (OR 1.02, 95% CI 1.00-1.03) and neonatal encephalopathy (OR 69.2, 95% CI 9.36-511.89) were found to be risk factors for CP in the total CP group in our multivariate analysis. Factors during the periods before, during and after delivery were all shown to increase the risk of spastic diplegia and tetraplegia, whereas mostly factors during the period before delivery increased the risk of spastic hemiplegia, and only factors during delivery increased the risk of dyskinetic CP. Admittance to an NICU was a risk factor for all CP subtypes. CONCLUSIONS: The risk factor pattern differed by CP subtype. The presented risk factors may be useful indicators for identifying children at risk of developing CP, and helpful for targeting individuals for early intervention programmes.

Speech problems affect more than one in two children with cerebral palsy: Swedish population-based study.

AIM: To describe speech ability in a population-based study of children with cerebral palsy (CP), in relation to CP subtype, motor function, cognitive level and neuroimaging findings. METHODS: A retrospective chart review of 129 children (66 girls, 63 boys) with CP, born in 1999-2002, was carried out. Speech ability and background information, such as type of CP, motor function, cognitive level and neuroimaging data, were collected and analysed. RESULTS: Speech disorders were found in 21% of the children and were present in all types of CP. Forty-one per cent of the children with speech disorders also had mental retardation, and 42% were able to walk independently. A further 32% of the children were nonverbal, and maldevelopment and basal ganglia lesions were most common in this group. The remaining 47% had no speech disorders, and this group was most likely to display white matter lesions of immaturity. CONCLUSION: More than half of the children in this CP cohort had a speech disorder (21%) or were nonverbal (32%). Speech ability was related to the type of CP, gross motor function, the presence of mental retardation and the localization of brain maldevelopment and lesions. Neuroimaging results differed between the three speech ability groups.

The changing panorama of cerebral palsy in Sweden. X. Prevalence and origin in the birth-year period 1999-2002.

AIM: The aim of the study was to describe the prevalence and origin of cerebral palsy (CP), which is the tenth report from the western Swedish study. METHODS: A population-based study covering 85,737 live births in the area in 1999-2002. Birth characteristics and neuroimaging findings were recorded, prevalence of CP was calculated and aetiology was analysed. RESULTS: CP was found in 186 children. The crude prevalence was 2.18 per 1000 live births. The gestational age-specific prevalence for <28 gestational weeks was 55.6 per 1000 live births, whereas it was 43.7 for 28-31 weeks, 6.1 for 32-36 weeks and 1.43 per 1000 for >36 weeks. There was a female majority among children born at term and a male predominance in children born preterm. Hemiplegia accounted for 38%, diplegia for 32%, tetraplegia for 7%, whereas 17% had dyskinetic CP and 5% ataxia. Neuroimaging showed white-matter lesions in 31% and cortical/subcortical lesions in 29%. The aetiology was considered to be prenatal in 36%, peri/neonatal in 42%, whereas it remained unclassified in 21%. CONCLUSION: The decrease in CP prevalence observed since the 1980s had ceased. An increase in children born at term and in dyskinetic CP was found. In children born before 28 weeks of gestation, the prevalence decreased significantly. White-matter and cortical/subcortical lesions dominated on neuroimaging.

Synchronized communication between people with dementia and their volunteer caregivers. A video-based explorative study on temporal aspects of interaction and the transfer to education.

Processes of demographic change are leading to decreasing human resources in professional as well as lay care; this decrease necessitates new concepts of care, especially for the growing number of people with dementia (p.w.d.). Since the amendment to the German Care Insurance Law (2002), family carers have been entitled to regular weekly relief, provided by volunteers who have been given a thirty-hour-training. As difficulties in information processing in p.w.d. form an important part of the symptoms in dementia sufferers--with a high impact on communication as well as competent functioning in activities associated with daily life, we wanted to establish how much awareness and sensitivity voluntary attendants show in "tuning in" to the p.w.d. and her/his individual capacity to interact. In an exploratory study the authors analyzed videotaped interactions between volunteer caregivers and dementia-sufferers which were recorded in everyday situations during the process of ongoing care. Using methods of Video Interaction Analysis and Grounded Theory, we developed categories which describe how in tune the helpers are with the timing skills--or lack of them--of p.w.d.. We think that understanding the different ways in which p.w.d. structure their time can improve their communication and interaction. The categories --"speed and adjustment of speed", "mutuality" and "time control"--seemed crucial in understanding the subsequent course of the interactions. In a second step, these categories have recently been used by students and staff of the Lausitz University of Applied Sciences to provide training that sensitizes volunteer attendants to the topic and to learn about volunteers; judgement on the importance of continuing education in this field.

Dyskinetic cerebral palsy in Europe: trends in prevalence and severity.

OBJECTIVE: To describe the trends for and severity of dyskinetic cerebral palsy in a European collaborative study between cerebral palsy registers, the Surveillance of Cerebral Palsy in Europe (SCPE). METHODS: The prevalence of dyskinetic cerebral palsy was calculated in children born in 1976-1996. Walking ability, accompanying impairments and perinatal adverse events were analysed. RESULTS: 578 children had dyskinetic cerebral palsy, of whom 70% were born at term. The prevalence per 1000 live births increased from 0.08 in the 1970s to 0.14 in the 1990s. For the 386 children (70%) with a birth weight of > or =2500 g, the increase was significant (0.05 to 0.12). There was a concurrent decrease in neonatal mortality among children with a birth weight of > or =2500 g. Overall, 16% of the children walked without aids, 24% with aids and 59% needed a wheelchair. Severe learning disability was present in 52%, epilepsy in 51% and severe visual and hearing impairment in 19% and 6%, respectively. Accompanying impairments increased with motor severity. In children born in 1991-1996, perinatal adverse events, that is an Apgar score of <5 at 5 min and convulsions before 72 h, had occurred more frequently compared with children with bilateral spastic cerebral palsy (BSCP, n = 4746). Children with dyskinetic cerebral palsy had more severe cognitive and motor impairments than children with BSCP. CONCLUSIONS: The prevalence of dyskinetic cerebral palsy appears to have increased in children with a normal birth weight. They have frequently experienced perinatal adverse events. Most children have a severe motor impairment and several accompanying impairments.

Late cerebral graft versus host reaction in a bone marrow transplanted girl with Hurler (MPS I) disease.

A girl with Hurler disease (MPS IH) underwent allogeneic stem cell transplantation at 13 months of age with her one HLA-B antigen mismatch mother as donor. The procedure was complicated by cerebral hemorrhage and a ventricular-peritoneal shunt device was inserted. Mild GVH reactions were rapidly reversed. One year after transplantation ventriculitis was suspected and the shunt was replaced by a ventricular drainage catheter. Antibiotics had no effect and graft-versus-host disease (GVHD) was diagnosed. All symptoms were reversed by prednisolone and cyclosporine. Increased albumin and pleocytosis in the cerebrospinal fluid (CSF) normalized concomitantly. Electron microscopy of the CSF sediment showed debris consisting of numerous complex aggregates of thin lamellae and electron dense fragments with a tight lamellar texture. Biochemical analysis of the CSF sediment proved that the debris contained galactosylceramide and sulfatide. The electron microscopic and biochemical findings were interpreted to represent stripping of central myelin as a result of subacute GVHD in the central nervous system and its desquamation from the brain parenchyma into the ventricular CSF through the post-hemorrhage defect. From reversal of the GVHD at 2 years of age until follow-up at 10 years of age the clinical condition remained stable with no recurrence or deterioration.

Dyskinetic cerebral palsy: a population-based study of children born between 1991 and 1998.

The aim of this study was to describe the epidemiology, aetiology, and clinical findings in dyskinetic cerebral palsy (CP)in a population-based follow-up study of children born between 1991 and 1998. Age range at ascertainment was 4 to 8 years and prevalence was 0.27 per 1000 live-births. Forty-eight children were examined (27 males, 21 females; mean age 9y, range 5-13y). Thirty-nine had dystonic CP and nine a choreo-athetotic subtype. Primitive reflexes were present in 43 children and spasticity in 33. Gross Motor Function Classification System levels were: Level IV, n= 10 and Level V, n= 28. The rate of learning disability (n= 35) and epilepsy (n= 30) increased with the severity of the motor disability. Thirty-eight children had anarthria. Peri- or neonatal adverse events had been present in 34 of 42 children born at >or=34 weeks' gestation. Motor impairment was most severe in this group. Placental abruption or uterine rupture had occurred in 8 participants and 19 of the 42 near-term/term children required assisted ventilation, compared with 1% and 12% respectively in other CP types. Neuroimaging in 39 children born at >or=34 weeks revealed isolated, late third trimester lesions in 24 and a combination of early and late third trimester lesions in seven. Dyskinetic CP is the dominant type of CP found in term-born, appropriate-for-gestational-age children with severe impairments who have frequently experienced adverse perinatal events.

Gross and fine motor function and accompanying impairments in cerebral palsy.

The aim of this study was to describe and analyze gross and fine motor function and accompanying neurological impairments in children with cerebral palsy (CP) born between 1991 and 1998 in western Sweden. A population-based study comprised 411 children with a diagnosis of CP ascertained at 4 to 8 years of age. Gross Motor Function Classification System (GMFCS) levels were documented in 367 children (205 males, 162 females). Bimanual Fine Motor Function (BFMF) classification levels of 345 of the children and information on learning disability, epilepsy, visual and hearing impairments, and hydrocephalus from 353 children were obtained. For spastic CP, a new classification according to the Surveillance of Cerebral Palsy in Europe of uni- and bilateral spastic CP was applied. GMFCS was distributed at Level I in 32%, Level II in 29%, Level III in 8%, Level IV in 15%, and Level V in 16%. The corresponding percentages for BFMF were 30.7%, 31.6%, 12.2%, 11.9%, and 13.6% respectively. Learning disability was present in 40%, epilepsy in 33%, and severe visual impairment in 19% of the children. Motor function differed between CP types. More severe GMFCS levels correlated with larger proportions of accompanying impairments and, in children born at term, to the presence of adverse peri/neonatal events in the form of intracranial haemorrhage/stroke, cerebral infection, and hypoxic-ischaemic encephalopathy. GMFCS Level I correlated positively to increasing gestational age. We conclude that the classification of CP should be based on CP type and motor function, as the two combine to produce an indicator of total impairment load.

The changing panorama of cerebral palsy in Sweden. IX. Prevalence and origin in the birth-year period 1995-1998.

AIM: This is the ninth report from the western-Swedish study of the prevalence and origin of cerebral palsy. METHODS: A population-based study covering the 88 371 live births in the area in 1995-1998. Birth characteristics, neuroimaging findings and risk factors in children with cerebral palsy were recorded, prevalence was calculated, and aetiology was analysed. RESULTS: The study comprised 170 children with cerebral palsy, i.e. a prevalence of 1.92 per 1000 live births. Excluding eight post-neonatally derived cases, the gestational age-specific prevalences were 77 per 1000 for children born before 28 wk of gestation, 40 for children born at 28-31 wk, 7 for children born at 32-36 wk and 1.1 for children born after 36 wk of gestation. Spastic hemiplegia, diplegia and tetraplegia accounted for 38%, 35% and 6%, respectively, dyskinetic cerebral palsy for 15%, and ataxia for 6%. For the first time, hemiplegia was now most common, due to the decline in preterm diplegia. There was a further increase in full-term dyskinetic cerebral palsy. The origin of cerebral palsy in children born at term was considered to be prenatal in 38%, peri/neonatal in 35% and unclassifiable in 27%, while in children born preterm it was 17%, 49% and 33%, respectively. CONCLUSION: The decreasing trend from the period 1991-1994 continued, both in children born at term and especially in those born preterm. However, the increase in dyskinetic cerebral palsy in children born at term was a matter of concern. In this group, a perinatal hypoxic ischaemic encephalopathy had been present in 71%.

Glucose and insulin levels in young subjects with different maternal histories of hypertension: the Hypertension in Pregnancy Offspring Study.

OBJECTIVE: To analyse whether hypertension during pregnancy is associated with early signs of impaired glucose metabolism in the offspring. DESIGN: Longitudinal study with a 5-year follow-up. SETTING: University Hospital, Göteborg, Sweden. MAIN OUTCOME MEASURES: Fasting levels of glucose, insulin and C-peptide. SUBJECTS: Thirty-six children were born to mothers with hypertension in pregnancy. The children were divided into two groups according to their mothers' blood pressure at follow-up 7-12 years after pregnancy. Nineteen children had hypertensive mothers (HT), while 17 children had normotensive mothers at follow-up (NT). A control group (C) comprised 16 children, who were born after normotensive pregnancies to mothers who remained normotensive. RESULTS: Fasting plasma glucose was significantly higher in HT than in NT (5.2 vs. 4.9 mmol L-1; P < 0.05). In C fasting glucose was 5.1 mmol L-1. The same trend was seen for fasting insulin in HT. NT and C, respectively (6.7 vs. 4.7 vs. 5.3 microU mL-1). The C-peptide level was 1.61, 155 and 1.64 ng mL-1, respectively. Calculated insulin resistance was 1.5 in HT. 1.0 in NT and 1.2 in C. CONCLUSIONS: It is suggested that hypertension during pregnancy may be associated with impaired glucose metabolism and elevated fasting glucose levels in the offspring during adolescence.

Hypertension in pregnancy and size at birth.

Low birth weight and high placental weight are associated with increased cardiovascular morbidity and mortality. Children born after hypertensive pregnancies have higher blood pressure than children born after normotensive pregnancies. Hypertension in pregnancy is considered a major risk factor for intrauterine growth retardation. The present study describes size at birth and perinatal characteristics in children born after hypertensive pregnancies. During five consecutive years 17,000 deliveries took place in the well defined geographic area of eastern Göteborg. Hypertension in pregnancy complicated 261 pregnancies. For comparison 260 normotensive pregnancies, matched for maternal age and time of delivery, were used. Standard deviation score for birth weight and length were calculated according to reference standards for birth weight (BWSDS) and length (BLSDS) based on all Swedish births 1977-1981 (n = 475,588). Children born after hypertensive pregnancies had lower birth weight, were shorter and had a shorter gestational period as compared with children born after normotensive pregnancies. Head circumference and placental weight did not differ and there were no significant differences in BWSDS. It is concluded that hypertensive pregnancies are characterized by lower birth weight and shorter gestational period. However, intrauterine growth retardation is not a general characteristic of hypertension in pregnancy.