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Biomedical subjects

B Hagberg

Publications and source records attributed to B Hagberg.

At least 181 records · Page 10Linked to original sources
PubMed1978

Severe mental retardation in Swedish children born 1959-1970: epidemiological panorama and causative factors.

The findings from two recent Swedish retrospective studies on severely mentally retarded children born 1959-70 are surveyed. One study concerned an average county--Uppsala--in the middle of Sweden, the other a vast northern county, Västerbotten, with a sparse population. Figures for incidence and prevalence are given, as well as the percentage distribution of aetiological/pathogenic groups and subgroups, and the frequency of multihandicaps. Prenatal causes were apparent in about 70% of affected children, Down syndrome being the main condition (32%). A background of mutant genes in severe mental retardation was found significantly more often in Västerbotten than in Uppsala (17% and 7% respectively). Perinatal causes were revealed in 10 and 8% (9% of the two series pooled together) and postnatal causes in only 3 and 1% (2%). Associated CNS handicaps were present in about half of the children (42% in Uppsala and 52% in Västerbotten), with epilepsy in 30 and 36%, cerebral palsy in 18% and 19%, and a combination of epilepsy and cerebral palsy in 8 and 16%, respectively.

Adolescent↗
PubMed1978

Neurovisceral storage disorder simulating Niemann-Pick disease. A new form of oligosaccharidosis?

Two sisters are described with demonstrable splenomegaly already from infancy and, after the age of 2--4 years, signs of slowly progressive encephalophy, vacuolated lymphocytes in the peripheral blood, and peculiar foam cells in the bone marrow aspirates. The died at 7 3/4 and 6 1/2 years. Widely spread in the brain, the nerve cell bodies were found to show extensive ballooning. It was most striking in the brain stem and spinal cord, while cerebellar structures were remarkably well preserved. The cytoplasm of the ballooned nerve cells was filled with finely granular storage material stainable as a readily soluble glycolipid. The spleen, liver and intestinal wall contained numerous foamy PAS-positive macrophages. Chemical assays showed a ten-fold increase of lactosylceramide and a modest one of minor gangliosides brain cortex. No accumulation sphingomyelin could be revealed, and the sphingomyelinase activity was found to be normal. The ganglioside GM1 beta-galactosidase activity of leucocytes was reduced to 20--25% of normal, which indicated a disturbance of the glycosaminoglycan metabolism. The tissue content of glycosaminoglycans was, however, normal, but an accumulation of lactose was demonstrated in the spleen. It is postulated that the primary enzymic defect is a disturbance of a lysosomal beta-galactosidase with a substrate specificity for lactose and other oligosaccharides with a terminal beta-galactosidic linkage.

Bone Marrow↗
PubMed1978

Recovery in hydrocephalic dementia after shunt operation.

Twenty-three patients with hydrocephalic dementia were studied before and after shunt operation, and improvement was found in 12. Before operation, the improved cases showed more symptoms of confabulation, gait disturbance, urinary incontinence, lack of insight, and constructional apraxia. The improvement was also most marked in these symptoms. The significance of general versus specific symptoms, duration, and aetiology is discussed from a differential diagnostic standpoint, and we conclude that the adequate and early diagnosis of hydrocephalic dementia is essential for good outcome after shunt operation, and that psychiatric and psychometric evaluation enable such a diagnosis to be made.

Adult↗
PubMed1977

Severe mental retardation in a Swedish county. II. Etiologic and pathogenetic aspects of children born 1959--1970.

In an unselected series of 122 children born in 1959--1970 with severe mental retardation (SMR), the magnitude and distribution of the different etiologic and pathogenetic factors were analysed. Prenatal causes were considered relevant in 73 per cent, perinatal in 10 and postnatal in 3. In 12 per cent no traceable cause whatsoever was found. Infantile primary psychoses constituted 2 per cent. Within the prenatal group, no less than 43 per cent presented firm evidence favouring a genetic etiology, chromosomal syndromes together constituting 36 per cent and mutant gene disorders 7 per cent. Etiologically unclassifiable cases with prenatal stigmata and congenital defects amounted to 18 per cent. A condition of fetal deprivation of supply was considered to have been of major importance in 8 per cent. Almost half of the cases had one or more associated CNS handicaps. Cerebral palsy syndromes were evident in 18 per cent and epilepsies in 30 per cent. The relative risk of having cerebral palsy in addition to SMR was strongly correlated to cases classified as prenatally or perinatally acquired CNS syndromes. It is concluded that prenatal prevention is of special importance for reducing the occurrence of SMR. As chromosomal aberrations are the cause in more than 1/3 of cases with SMR, the desirability of developing effective cytogenetic screening methods and programs applicable to all pregnant women is particularly emphasized.

Adolescent↗
PubMed1977

Cerebral dysfunction in alcoholism and presenile dementia. A comparison of two groups of patients with similar reduction of the cerebral blood flow.

Fifteen chronic alcoholics and 15 presenile patients were matched in pairs with regard to their mean hemisphere cerebral blood parameters-an index of brain metabolism. The distribution of the regional cerebral blood flow was similar in the two groups. The alcoholics performed significantly better in all psychometric tests than the presenile patients and more alcoholics than presenile patients were still employed and working. Nine presenile patients and one alcoholic showed symptoms indicating brain stem dysfunction. The measurements of the cerebral blood blow were made at rest while the psychometric testings and the evaluation of the social performance gave information about the brain at work. It is suggested that cerebral dysfunction in alcoholics to some extent can be partly overcome by brain activation procedures. In contrast, the defects of higher mental functions in presenile dementia cannot be compensated for.

Alcoholism↗
PubMed1977

Severe mental retardation in a Swedish county. I. Epidemiology, gestational age, birth weight and associated CNS handicaps in children born 1959--70.

In a unselected series of children born in 1959--70 with severe mental retardation (MR)---defined as IQ less than 50---in a Swedish county, the incidence, prevalence, gestational length, birth weight and associated CNS handicaps were analysed. The cumulative incidence at 1-16 years of age was calculated at 3.3% and the prevalence at 11-16 years at 2.8%. These figures were lower than in most other previous studies. In the great majority of cases the pathogenesis was of prenatal origin. The mean gestational lengths and birth weights were decreased compared with those of an average Swedish population. Severe MR affected large and small for date babies more often than could be expected. On the other hand, babies, with a low birth weight, appropriate for gestational age, and with an uncomplicated history, were found not to run a special risk of severe MR. Among the 122 children, 42% had one or more associated CNS handicaps---epilepsy (30%) and cerebral palsy (18%) being the most common.

Adolescent↗
PubMed1976

The changing panorama of cerebral palsy in Sweden 1954-1970. III. The importance of foetal deprivation of supply.

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 less than 0.001). 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 multihandicapped.

Birth Weight↗
PubMed1976

[Pre-, peri- and and postnatal prevention of major neuropediatric handicaps in Sweden (author's transl)].

The actual distribution and panorama changes of neuropediatric handicaps in Sweden as well as modern aspects on prevention are presented. The following conclusions are drawn. Preventive programs directed towards brain damaging factors occurring after the neonatal period are more or less completed. Further gains, however moderate, should be attainable perinatally. The largest number of unsolved problems and today the most challenging, refer to secondary fetal brain dysfunction during the prenatal period, particularly the 3rd trimester.

Cerebral Palsy↗