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Biomedical subjects

B Hagberg

Publications and source records attributed to B Hagberg.

At least 109 records · Page 6Linked to original sources
PubMed1990

Delayed-onset dyskinetic 'cerebral palsy--a late effect of perinatal asphyxia?

A 12-year-old girl, born full term after severe pre- and peripartal asphyxia, with transient abnormal newborn neurology and slight motor development deviations but no cerebral palsy syndrome, is described. At the age of 5, she developed a slowly progressive dyskinetic hemisyndrome, initially uncoordinated hyperkinetic movements in her left arm, dystonic posturing then supervened. Since the age of 11, her right side had also slowly become involved. As extensive examinations excluded other causes, it is concluded that this case represents a delayed onset dyskinetic 'CP-syndrome' subsequent to a postanoxic non-progressive insult to the basal ganglia, but with a well compensated functional integrity before the age of 5.

Asphyxia Neonatorum↗
PubMed1990

Ocular pathology in disialotransferrin developmental deficiency syndrome.

Disialotransferrin developmental deficiency (DDD) syndrome is a recently described disease consisting of hepatopathy, mental retardation and neuropathy. The biochemical findings indicate a defect in the assembly of the carbohydrate moiety that is common to the secretory glucoproteins. It is believed to be of autosomal recessive inheritance. An ophthalmological examination of ten children suffering from this syndrome showed that all had ocular involvement. Esotropia (and deficient abduction) was found in all ten patients. Seven children had retinitis pigmentosa which was verified by an ERG in three. One patient had retinal signs suggestive of retinitis pigmentosa. The high incidence of ocular findings in the DDD syndrome, which are reported for the first time, indicate that an ophthalmological examination is a helpful diagnostic tool in this disease.

Adolescent↗
PubMed1990

Rett syndrome and the autistic disorders.

Rett syndrome is a disorder noted to date only in females and characterized by a pervasive developmental disability following apparently normal early infancy. In addition to gait difficulties, stereotypic hand movements, and loss of communication and purposeful hand skills, autistic-like behavior is an early sign that often results in misdiagnosis. Despite these significant clinical abnormalities, neuropathologic features are modest, and no consistent laboratory abnormality or diagnostic marker has been identified. The current status of research in RS is considered within the context of autism and other disorders in which autistic features may occur, such as the fragile X syndrome. The concept of autism as neurobiologically based behavior is developed. As such, autism is regarded as an umbrella category containing an ever-expanding list of specific disorders.

Autistic Disorder↗
PubMed1989

EEG development in Rett syndrome. A study of 30 cases.

EEG development through advancing clinical stages was studied in 30 cases of Rett syndrome, a progressive encephalopathy seen so far only in girls. Besides slowing of background activity and epileptic patterns, which are found in many types of 'degenerative' brain diseases, we observed in advanced stages of Rett syndrome unusual EEG patterns resembling those seen in slow virus encephalitis. On the other hand, the EEG was normal in one case of advanced Rett syndrome. The typical steps of EEG development were: initially normal EEG; as the first abnormality rolandic spikes, often followed by other epileptic patterns; and, in more advanced stages, a pseudoperiodic delta pattern and occasionally generalized periodic spike activity. The results motivate a discussion concerning other possible origins of Rett syndrome in addition to the supposed genetic one. The question is raised whether Rett syndrome might be etiologically heterogeneous.

Adolescent↗
PubMed1989

Psychometric characteristics in patients with frontal lobe degeneration of non-Alzheimer type.

Twenty patients, selected from a longitudinal study of patients with presenile and senile dementia, underwent psychometric assessment including semi-structured observations of behaviour in the test situation and examination for dysphasia. The patients were chosen on the basis of post-mortem investigation and showed frontal lobe degeneration (FLD) of non-Alzheimer type in 16 cases and Pick's disease in four cases. The patients are described with reference to cognition, language function, behaviour and emotional reactions in the test situation. The test results were analyzed and compared with psychometric results from other groups of demented patients, above all with Alzheimer's disease. The cognitive dysfunction was less severe in patients with onset of dementia below the age of 56 years as compared to patients with later onset, i.e., 56 years or older. Dysfunction of expressive speech was the most consistent finding in the patients with frontal lobe degeneration, while receptive speech functions were relatively less frequently affected.

Adult↗
PubMed1989

The changing panorama of infantile hydrocephalus and cerebral palsy over forty years--a Swedish survey.

The time trends and background of infantile hydrocephalus (IH) and cerebral palsy (CP) are surveyed. The changes in live birth prevalence, disability patterns, associated neuroimpairments and distribution of etiologies are analysed. Both the risk of IH and that of CP sharply increase with decreasing birth weight and gestational age. It is concluded that the remarkably enhanced survival of particularly very preterm infants, those at the highest risk of long-term morbidity, implies an increasing number of impaired children as long as the outcome of survivors is not drastically improved. The data presented are thought to be of relevance as to reconsideration of the effectiveness of perinatal care for preterm babies.

Cerebral Palsy↗
PubMed1989

Neurophysiological findings in the Rett syndrome, I: EMG, conduction velocity, EEG and somatosensory-evoked potential studies.

Nine girls, aged 10 to 22 years, with confirmed Rett syndrome--eight as stage IV and one at stage III--were investigated neurophysiologically. EMG and neurography studies were performed, and somatosensory-evoked responses (SER) were recorded as well as EEG with topographic mapping. Even in advanced clinical stages, no major motor root involvement or demyelinating motor peripheral neuropathy was detected on EMG or neurography, but an axonopathy, possibly of secondary origin, was observed. EEG showed slowing and dominance of low frequency activity of subcortical origin, with or without epileptic discharges. SER findings suggested involvement of the spinal cord and the spinothalamic system. Neurophysiological investigations can be used by the clinician in the differential diagnosis of the Rett syndrome.

Adolescent↗
PubMed1989

Neurophysiological findings in the Rett syndrome, II: Visual and auditory brainstem, middle and late evoked responses.

Nine girls with the Rett syndrome (RS) were investigated neurophysiologically using evoked potentials techniques. Visual- (VER) and auditory-evoked responses, including the early (ABR), middle (MLR) and late components (ACR), were recorded. There was evidence of variable, multilevel impairment of the nervous system. While ABR and MLR indicated lesions at the brainstem and midbrain levels, the late responses and VER pointed to an intra-cerebral/cortical defect. It is suggested that the perceptual mechanisms still functioned and some discrimination properties remained.

Adolescent↗
PubMed1989

Epidemiology of spastic tetraplegic cerebral palsy in Sweden. II. Prevalence, birth data and origin.

The prevalence and origin of spastic tetraplegic cerebral palsy (TPL) was investigated in a population-based study from 15 Swedish counties and the city of Gothenburg. The series comprised 96 children and adolescents born in 1959-1978. The prevalence at the ages of 5-24 years was 8 per 100,000. The etiological analysis was based on the 91 TPL subjects, born in Sweden. The preterm rate was 7%. There were 46 males and 45 females. Excluding postnatal cases, the mean birth weight was 2949 g and the proportion of SGA 21%. An obvious prenatal origin was found in 21 (mainly microcephaly, other CNS maldevelopment, intrauterine CMV-infection), an obvious perinatal origin in nine (mainly cerebral hemorrhage), and an obvious postnatal origin in 16 (mainly CNS infection). A potential prenatal origin was considered in six, a combined pre- and perinatal in 15, a perinatal in 13 and in 11 the origin of TPL was untraceable. An optimality analysis showed that reduced optimality in the partum and postpartum periods discriminated between pre- and perinatal etiology of TPL. Cases with obvious perinatally derived TPL had a high load of complications in the partum and postpartum periods, whereas these periods had been fairly uncomplicated in cases with an obviously prenatal origin of TPL. - It was indicated that some 50-55% of TPL was prenatally, around 30% perinatally and some 15-20% postnatally derived. The low preterm rate as well as the proportion of perinatal brain damage in TPL cases has probably increased in recent birth year periods due to the appearance of very preterm, severely multi-impaired children in the CP panorama.

Adolescent↗
PubMed1989

The changing panorama of cerebral palsy in Sweden. V. The birth year period 1979-82.

The prevalence of cerebral palsy (CP) in Swedish infants born in the four-year period 1979-82 is reported and related to the prevalence in infants born during the twenty-year period 1959-78. In 1979-82 it was 2.17 per 1,000 livebirths, 1.23 for children born at term and 0.94 for preterms, which means that the rising trend since the beginning of the 1970s persisted. The most pronounced rise, from 0.18 per 1,000 livebirths in the period 1967-70 to 0.67 in 1979-82 was found in the subgroup of preterms with spastic/ataxic diplegia. The severity of motor disability and the relative frequency of mental retardation, infantile hydrocephalus and epilepsy among preterm CP children successively increased over the same period of time. The livebirth prevalence of CP in term infants increased slightly but non-significantly during the period 1967-82. The birthweight-specific prevalence of CP per 1,000 newborns surviving the first week of life increased in all birthweight groups during the period 1967-82, significantly for birthweights below 1,500 g and over 2,500 g. The rising prevalence of CP was concomitant with a parallel fall in perinatal mortality, especially in very preterm infants. In the 1980s, severely multi-handicapped, very preterm children, only exceptionally seen among CP children born in the 1960s and 1970s, has become a matter of concern.

Cerebral Palsy↗
PubMed1989

Disialotransferrin developmental deficiency syndrome.

Seven mentally deficient children and adolescents (three pairs of siblings and one singleton) were studied. A peculiar external appearance, a characteristic neurohepatosubcutaneous tissue impairment syndrome and, as a biological marker, an abnormal sialic acid transferrin pattern were characteristic features. All seven seemed odd from birth and prone to acute cerebral dysfunction during catabolic states. Abnormal lower neurone, cerebellar, and retinal functions dominated from later childhood. The disialotransferrin pattern found in serum and cerebrospinal fluid is thought to be the biological marker of a newly discovered inborn error of glycoprotein metabolism with autosomal recessive inheritance.

Adolescent↗
PubMed1988

Epidemiology of infantile hydrocephalus in Sweden: a clinical follow-up study in children born at term.

The long-term outcome of infantile hydrocephalus (IH) in children born at term during a period of active shunt treatment was studied in a population-based survey. The series consisted of 68 children greater than or equal to 6 years old and born in 1967-78 in the south-western Swedish health care region. The clinical follow-up included neuro-paediatric assessment, Stott's test of motor impairment, the WISC test, CT and EEG analyses. Nineteen of the 68 children (28%) had cerebral palsy, 17 (25%) minor motor dysfunction and 32 (47%) no motor dysfunction; mental retardation was present in 26 (38%), 16 with an IQ 50-70 and 10 with IQ less than 50; 42 children (62%) had normal intelligence and epilepsy was found in 15 (22%). Compared with a non-shunted IH series from the 1950s, the survival of IH children had considerably increased. Of constituents characterizing the IH syndrome from the time prior to shunting, ataxia, divergent squint and the special "Cocktail-party behaviour" had significantly decreased, all of which conditions are highly related to chronic expansion of the ventricular system. The frequencies of other impairments such as mental retardation and epilepsy were fairly similar, reflecting the present increased survival of IH children with primarily non-IH-dependent brain damage. IH children with associated brain parenchymal defects had the poorest outcome, and those without had in general a much more favourable one. Thus the single most important factor for the outcome of IH was found to be the presence or absence of associated primary brain damage or maldevelopment.

Cerebral Palsy↗
PubMed1988

Epidemiology of infantile hydrocephalus in Sweden. Current aspects of the outcome in preterm infants.

The outcome in a population-based series of 61 Swedish preterm infants born in 1967-82 with infantile hydrocephalus (IH) was investigated. Sixteen (26%) died before the age of two years. The available information was updated when the 45 surviving children were at least four years and six months old. A structured follow-up examination was performed in the 13 children who had passed the age of six years. Among the 45 survivors, 47% had cerebral palsy, 51% mental retardation and 33% epilepsy. The overall outcome for preterm infants with IH was found to be poorer than that for fullterm ones. Prognostic factors correlating to a poor outcome were an obvious origin of IH (pre- or perinatal) and a gestational age of less than 28 weeks. It is concluded that handicapped IH children born very or extremely prematurely constitute a new, and to a large extent severely brain-damaged group that has entered the Swedish IH panorama since the end of the 1970s.

Female↗
PubMed1988

The basic care needs of profoundly mentally retarded children with multiple handicaps.

The ability profiles, aetiological patterns and basic care needs for a small minority of the most seriously multi-impaired and profoundly mentally handicapped children were analysed. The analyses were based on (1) a care-load study (1980-81) of a population-based series of 53 children and adolescents born in 1966-76, representing the most profoundly retarded third of those with and IQ less than 50 in the city of Gothenburg, and (2) 10 years clinical experience of a paediatric multi-impairment unit, including a cross-sectional consensus evaluation of the children being cared for in August 1986. It is concluded that these children represent a long-term paediatric care-group in need of continuous and life-long baby-care, with access to medical support and service. When it is not possible to care for these children in their own homes, it cannot be realistically expected that they can be included in a normalisation scheme, implying that they can become integrated residentially and socially in society and participate in traditional training-school programmes.

Abnormalities, Multiple↗