THE CHANGING PANORAMA OF CEREBRAL PALSY IN SWEDEN 1954—1970 III.

Abstract. From record data derived from an unselected series of 560 Swedish cases of cerebral palsy, born in 1954-70, the significance of prenatal factors associated with materno-foetal vascular insufficiency causing foetal deprivation of supply (FDS) was investigated. Cerebral palsied children were found to have, on an average, retarded intrauterine growth when compared with a normal population. The incidence of bleeding during pregnancy, maternal toxaemia and multiple pregnancies were significantly increased (p<0.0001). The incidence of the prenatal factors in question had remained unchanged through the years 1954-70. An additive interplay between factors of FDS and asphyxia/cerebral haemorrhage was revealed. As a whole the findings support the conclusion that FDS plays a major role in the pathogenesis of cerebral palsy today, particularly among the multihandkapped.     

IDENTICAL SYNDROMES OF CEREBRAL PALSY IN THE SAME FAMILY

Familial cases of cerebral palsy were traced all over Sweden. Fortythree families were collected, in 30 of which the patients were siblings. The families were divided into three groups: (1) 16 families with cases of identical syndromes and a history of normal pregnancy, delivery and perinatal period; (2) 3 families with cases of identical syndromes but an abnormal perinatal period; (3) 24 families with non-identical syndromes. Within the first group, which is of main genetic importance, 10 families were found with 2-3 siblings affected with congenital non-progressive ataxia and mental retardation, the mode of inheritance with all probability being autosomal recessive. Three families showed ataxic diplegia, two of them only in siblings, the third with affected members of both sexes represented in three generations. Surprisingly enough, pure spastic diplegia was only revealed in one family, viz. a grandfather and his grandson. Spastic tetraplegia was found in two mentally retarded siblings in an otherwise healthy sibship of 11 members. True microcephaly combined with a dystonic tetraplegic cerebral palsy was seen in one family and was thought to have an autosomal recessive inheritance as in similar cases reported in the literature. Chromosome studies and laboratory screening tests revealed no abnormalities indicating particular aetiological mechanisms.     

DYSKINETIC CEREBRAL PALSY

ABSTRACT. An unselected series of 116 dyskinetic cerebral palsy cases born 1959-70 was delineated and subgrouped according to neurological criteria into 35 hyperkinetic (30%) and 81 dystonic (70%) cases. Of the hyperkinetics, 80% had a mild motor disability, while 90% of the dystonics had moderate or severe disability. Subordinated spastic signs were found in 9% of the hyperkinetic and in 44% of the dystonic cases. IQ was >90 in 69% of the hyperkinetics and 25% of the dystonics and <50 in 11% and 41%, respectively. Two or more additional neurological abnormalities (spasticity, mental retardation, epilepsy, anarthria, dysarthria, hearing deficit) were detected in 46% hyperkinetic and 81% dystonic cases. A heavy multihandicap complex was present predominantly in the dystonic subgroup. The 5-19 year mortality rate was 6 % and was confined to the most severely handicapped dystonic cases. The twin rate was 5%. A total of 9% had birth weights ≤ 1500 g, and 31% had ≤ 2500 g. The weight- and age-specific incidences increased rapidly with decreasing birth weights and gestational ages. The crude incidence of hyperkinetic cerebral palsy was 0.07 and dystonic cerebral palsy 0.17 per thousand live births.     

PREVENTION OF CEREBRAL PALSY IN MOTOR RISK INFANTS BY TREATMENT AD MODUM VOJTA: A Controlled Study

Abstract. Brandt, S., Lønstrup, H., Marner, T., Rump, K. J., Selmar, P., Schack, L. K. (Clinic for Cerebral Palsy and Child Neurology, Orthopedic Hospital, Rigshospitalet, Copenhagen, Denmark) and d'Avignon, M., Norén, L. and Årman, T. (The Paediatric Clinics of St. Göran's and Danderyd Hospitals, Stockholm, Sweden). Prevention of cerebral palsy in motor risk infants by treatment ad modum Vojta. Acta Paediatr Scand, 69: 283, 1980.—The proposal by V. Vojta in 1974 to prevent development of cerebral palsy in 'motor risk' infants by special treatment has been investigated in 11 Danish and 10 Swedish babies and compared with 30 control infants with a similar risk, who were not given Vojta treatment. We found a tendency for 'uncomplicated' cerebral palsy cases to accumulate in the control group, although the difference was non-significant on a 5% level. Further con-trolled studies must be completed before it is possible to accept the prophylactive treatment of cerebral palsy recommended by Vojta.     

PERINATAL MORTALITY: CHANGES IN THE DIAGNOSTIC PANORAMA 1974–1980

ABSTRACT. During the period 1974–1980, all late abortions (>19 completed gestational weeks) (LA), late fetal deaths (LFD) and early neonatal deaths (END) were surveyed in a cotninuous material of 17 813 births with an ascertained gestational age established by early ultrasound fetometry. There was no maternal death during the period. The total perinatal mortality (PM) was 0.98 % with an END rate of 0.51 %. In about 45 % of LFD no diagnosis was found. Intrapartum death was extremely rare as was END caused by asphyxia or infection. In END, death from immaturity constituted the major group in the beginning of the period, while after 1977 lethal malformations was the dominating diagnosis. There was a continuous decrease in Idiopathic Respiratory Distress Syndrome (IRDS) as a cause of END. The porportion of END in extremely premature children showed a constant decrease in the beginning of the period. No difference in sex was found in END except for lethal malformations where there was a significant male preponderance. As a consequence of a more active obstetrical care, some fetuses who would previously have been classified as LA were probably delivered liveborn, extremely premature and appeared as END. The question of where to set the limits for what should be included in PM is thus highly relevant.     

EVALUATION OF PROGNOSIS AND TREATMENT IN BELL'S PALSY IN CHILDREN

Nerve-excitability measurements performed early in the course of the disease are of a good prognostic value. The results show that when excitability is maintained unimpaired after 72 hours of the onset, there is a chance of 100% spontaneous cure. When the excitability is diminished by less than 50%, 90% recovery may occur, but when it is markedly diminished or absent the prognosis is bad. The schedule of treatment to be followed should be based on the result of nerve excitability measurement early in the course of the disease. Systemic vasodilators, as nicotinic acid early in the course of the disease has excellent effect as it cuts the vicious circle of arteriolar spasm without increasing pressure within the facial canal. It is advisable that early short wave therapy should be avoided as it may increase nerve compression through local vascular congestion in the vicinity of the nerve. Incidence of physiological block is higher in children with Bell's palsy than in corresponding adult patients who, as a result have a higher incidence of nerve degeneration and worse prognosis. The severity of paralysis does not indicate the degree of nerve lesion.     

THE INCIDENCE OF DIABETES MELLITUS IN SWEDISH CHILDREN 1970–1975

ABSTRACT. report a retrospective study of diabetic children, 0–14 years of age, from seven Swedish departments of paediatrics. There were 359 new cases in the years 1970–1975. Notification suggested that there was a mean yearly incidence of 19.6 cases per 100000 with a year to year variation of 10.0–26.4 per 100000. Consequently about 330 new cases of childhood diabetes would be expected in Sweden every year. Incidence varied considerably between different geographical areas. The age distribution was bimodal with a main peak at about 12 years and another peak at about 7 years. There was some evidence for clustering of new cases in January and the autumn. The mean prevalence of childhood diabetes in the seven districts was 1.3 per 1000.     

SEASONAL INCIDENCE OF SOME CONGENITAL MALFORMATIONS IN THE CENTRAL NERVOUS SYSTEM IN SWEDEN, 1965–1972

Abstract Seasonal trends of some congenital CNS malformations in infants born in Sweden during the years 1965 to 1972 were investigated. The study is based on reports to the Swedish Register of Congenital Malformations, statistics from the National Board of Health, and records from Malmö General Hospital and the Hospital of Lund. The malformations analysed in this study are anencephaly, spina bifida aperta (with or without hydrocephalus) and isolated hydrocephalus. Variations in incidence of months of birth and last menstrual period (LMP) were tested statistically in three different ways: standard χ²-test for heterogeneity between recorded numbers of infants each calender month, Edwards' method, and a squared sinus function, all with or without correction for variations in genera) monthly birth rates. Statistical significant seasonal variations were found for anencephaly with an LMP maximum in March and for spina bifida aperta with a maximum in July. Factors which can explain such seasonality are briefly discussed.     

OMPHALOCELE and GASTROSCHISIS IN SWEDEN 1965–1976

Abstract. Lindham. S. (Department of Paediatric Surgery, Karolinska Hospital, Stockholm, Sweden). Omphalocele and gastroschisis in Sweden 1965–1976. Acta Paediatr Scand, 70:55, 1981.–The present investigation supports the view that omphalocele and gastroschisis are two different congenital malformations. They differ not only anatomically but also regarding the incidence of concomitant malformations, sex ratio and maternal age. There has been a significant increase in the incidence of gastroschisis in Sweden during the period 1965–1976 but no corresponding increase in the incidence of omphalocele. The incidence of abdominal wall defects in stillborn is twenty times higher than in liveborn children.     

CHILDHOOD CANCER IN SWEDEN, 1958–1974

ABSTRACT. All cases of tumours and tumour-like conditions in children 0–14 years reported to the Swedish cancer registry during the period 1958–74 have been studied. The material consists of 3797 individuals on file in this registry. The most common cancer diagnoses in children 0–14 years are leukemia and tumours of the central nervous system (together constituting approximately 58% of all cases). Almost half of the cancers affect children below five years of age. The lowest incidence occurs in the ages 7–8 years, and the highest during the first year of life. The types of tumours below one year of age show a different distribution than in other age groups. A significant increase in the incidence of childhood cancer occurred, while the mortality rates showed a slight decrease during the period studied. A remarkable increase in the incidence figures was noted concerning tumours of the nervous system, especially in boys. The decrease in the mortality rates was most obvious regarding Wilms' tumour, and leukemia in children 0–4 years of age.     

AN APPRAISAL OF TECHNIQUES FOR STUDYING CEREBRAL CIRCULATION IN THE NEWBORN

ABSTRACT. The study of cerebral circulation in ill pre-term infants is important both to improve our understanding of the physiological aspects of cerebral blood flow control, and to provide further insight into the aetiology of intracranial intra-ventricular haemorrhage. Progress in this field has been hampered by the absence of measurement techniques which must be safe and clinically applicable whilst at the same time providing results with sufficient quality for the confident development of hypotheses.
The symposium was held in order to evaluate critically the measurement techniques which might be considered for use in the sick, human newborn infant.     

EVALUATION OF A NATION-WIDE NEONATAL METABOLIC SCREENING PROGRAMME IN SWEDEN 1965–1979

ABSTRACT. Aim, J. and Larsson. A. (Department of Paediatrics, Karolinska Institute, St. Goran's Children's hospital, and the PKU Section, Department of Bacteriology, National Bacteriological Laboratory, Stockholm, Sweden). Evaluation of a nation-wide neonatal metabolic screening programme in Sweden 1965–1979. Acta Paediatr Scand, 70:601,.–In Sweden, neonatal screening for phenylketonuria was started in 1965 and a total of 1326000 infants were studied up to 1979. During various periods of time, screening was also carried out for galactosaemia, hereditary tyrosinaemia, histidinaemia, and homocystinuria. In screening for phenylketonuria and galactosaemia no false-negative results were obtained and the incidences were 1/30850 and 1/81100, respectively. In screening for hereditary tyrosinaemia only 1 out of 6 patients was identified by screening and the incidence was 1/106 710. Two cases of histidinaemia were detected, which corresponds to an incidence of 1/36 840. Both children developed normally without any treatment. No child with homocystinuria was detected in the screened population of more than 300 000 newborn infants. A screening programme involving phenylketonuria and galactosaemia was considered to be optimal among the tested disorders.     

A NEURO-DEVELOPMENTAL APPROACH TO THE TREATMENT OF CEREBRAL PALSY A PRELIMINARY REPORT*

An attempt has been made to assess the value of a neuro-developmental approach in the treatment of children with cerebral palsy. Some‘facilitation’techniques used to encourage automatic postural reactions are mentioned and it is emphasized that these patterns are more easily established in the very young before the increased tone or fluctuation of tone has become well established.