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Biomedical subjects

L Dubowitz

Publications and source records attributed to L Dubowitz.

At least 37 records · Page 2Linked to original sources

Visual function in term infants with hypoxic-ischaemic insults: correlation with neurodevelopment at 2 years of age.

AIMS: To determine if there is any association between the findings of visual assessment performed at the age of 5 months and neurodevelopmental outcome at the age of 2 years in children who have sustained hypoxic-ischaemic insults. METHODS: Twenty nine term infants with hypoxic-ischaemic encephalopathy and/or brain lesions on neonatal magnetic resonance imaging (MRI) were prospectively evaluated. At 5 months of age all the infants had their visual function assessed using the Atkinson Battery of Child Development for Examining Functional Vision, which includes the assessments of optokinetic nystagmus (OKN), acuity, visual fields, fixation shift and phase and orientation reversal visual evoked potentials. At 2 years of age the children had a structured neurological evaluation and a Griffiths developmental assessment. RESULTS: There was good correlation between the extent of the early detected visual impairment and both neuromotor and global development. Children with more than three out of five abnormal visual tests at 5 months of age tended to have abnormal neurological examination results and abnormal developmental quotients. Children with three or fewer abnormalities tended to have developmental quotients in the normal range; the level of their performance, however, was still related to the number of visual tests passed. CONCLUSIONS: Individual visual tests can provide important prognostic information. While abnormal OKN and acuity were always associated with abnormal outcome, normal results on visual evoked potentials and fixation shift tended to be associated with normal outcome.

Brain↗

Early prognostic indicators of outcome in infants with neonatal cerebral infarction: a clinical, electroencephalogram, and magnetic resonance imaging study.

OBJECTIVE: The aim of this study was to identify prognostic factors in newborns with cerebral infarction. DESIGN: Antenatal and perinatal factors and early clinical, electroencephalogram (EEG), and magnetic resonance imaging (MRI) findings were compared with neurodevelopmental outcome in 24 children with evidence of cerebral infarction on neonatal MRI. RESULTS: Out of 24 infants, 19 had an infarction in the territory of a major cerebral vessel and 5 in the borderzone between cerebral arteries. Neuromotor outcome was normal in 17 and abnormal in 7 infants. Of these 7 infants, 5 infants showed a definite hemiplegia, whereas the other 2 showed some asymmetry of tone or function but no definite hemiplegia. None of the adverse antenatal or perinatal factors was significantly associated with abnormal outcome. Neonatal clinical examination was also not always predictive of the outcome. The extent of the lesion on MRI was a better predictor. In particular, it was the concomitant involvement of hemisphere, internal capsule and basal ganglia that was always associated with an abnormal outcome whereas the involvement of only one or two of the three tended to be associated with a normal outcome. EEG was also very helpful. Abnormal background activity either unilateral or bilateral was found in 6 infants and 5 out of 6 developed hemiplegia. In contrast, the presence of seizure activity in presence of a normal background was not related to abnormal outcome. CONCLUSIONS: Early MRI and EEG can help to identify the infants with cerebral infarction who are likely to develop hemiplegia.

Cerebral Infarction↗

An optimality score for the neurologic examination of the term newborn.

We describe the application of a revised version of the Dubowitz neurologic examination of the newborn in 224 low-risk, term newborn infants. The method has been updated by eliminating less useful items and including new items evaluating general movements and patterns of distribution of tone. An optimality score is included to make the evaluation more quantitative and for comparison with sequential examinations with neurophysiologic and imaging findings. The score is based on the distribution of the scores for each item in the population of low-risk term infants. We defined not only the most common pattern for each item but also the variability of the findings by using 10th and 5th centiles. Because most of the items assessing tone and the Moro reflex varied with gestational age between 37 and 42 weeks, the changes were incorporated in the scoring system. The total optimality score was the sum of the optimality scores of individual items. Although the association of 4 or more deviant scores was found in less than 10% of our infants, deviant results on 1 or 2 single items could be observed in a third of this normal population, suggesting that isolated deviant signs have little diagnostic value. In contrast, an abnormal distribution of tone patterns, which we have commonly observed in infants with brain lesions, was not found in this cohort.

Arm↗

Orientation-reversal and phase-reversal visual evoked potentials in full-term infants with brain lesions: a longitudinal study.

The onset and maturation of visual cortical mechanisms can be recorded by using steady-state visual evoked potentials. The aim of this study was to evaluate and compare orientation-reversal (OR) and phase-reversal (PH) VEP as indicators of the maturation of cortical function in a population of fullterm infants with brain lesions on neonatal MRI. Forty-six infants with brain lesions on neonatal MRI were tested on both PH and OR VEP at 8 reversals/second at the age of 5 months and, if the responses were not significant, at a lower temporal frequency (4 reversals/second). Children whose VEPs were not significant at 5 months were tested longitudinally at 6, 9, 12 and 18 months. The results showed that 23 of the 46 infants (50%) did not show significant responses at 5 months and that while in 7 of the 23 (14% of the whole cohort) the responses became significant between 5 and 12 months, in the other 16 infants (34%) the VEP responses were persistently abnormal. Children with focal lesions, such as focal infarction or haemorrhages, tended to show normal or only mildly delayed VEP while more generalised lesions, such as the ones seen in infants with hypoxic-ischaemic encephalopathy grade 2 and 3, tended to be associated with abnormal VEP responses. The involvement of the optic radiations and occipital cortex was not always associated with abnormal VEP responses but the concomitant involvement of the basal ganglia was always associated with abnormal VEP. We were also able to demonstrate that VEP can be also used as a prognostic indicator: while normal OR VEP are reliably associated with a normal visual and neurodevelopmental outcome, abnormal 4 OR or 8 PH at 5 months are consistently associated with abnormal outcome.

Brain Diseases↗

Incidence of cranial ultrasound abnormalities in apparently well neonates on a postnatal ward: correlation with antenatal and perinatal factors and neurological status.

AIM: To evaluate cranial ultrasonography and neurological examination in a cohort of infants regarded as normal; and to determine the prevalence of ultrasound abnormalities and any potential association with antenatal or perinatal factors or deviant neurological signs. METHODS: Cranial ultrasound findings and neurological status were evaluated in 177 newborns (gestational age 36.3 to 42 weeks), admitted to a postnatal ward directly after birth and regarded as normal by obstetric and paediatric staff. The age of the infants at the time of examination ranged between 6 and 48 hours. Ultrasound abnormalities were present in 35 of the 177 infants studied (20%). Ischaemic lesions, such as periventricular and thalamic densities were the most common finding (8%), followed by haemorrhagic lesions (6%). The possible sequelae of antenatal haemorrhages, such as focal ventricular dilatation or choroid cysts, were present in 6%. Abnormal ultrasound findings were not significantly associated with signs of perinatal distress, such as cardiotocographic abnormalities or passage of meconium. Abnormal ultrasound findings tended to be associated with antenatal problems, although this did not reach significance. Ultrasound abnormalities were strongly associated with deviant patterns on the neurological examination. CONCLUSIONS: These results suggest that ultrasound abnormalities are more common than has been reported up to now. Lesions that could be ischaemic, such as flare densities, are seen even in the absence of any antenatal or perinatal risk factor.

Brain Diseases↗

Visual function in full-term infants with hypoxic-ischaemic encephalopathy.

Thirty-one full term infants with hypoxic-ischaemic encephalopathy (HIE) were studied with a battery of tests designed to evaluate visual function in infancy and with serial MRI. Their age ranged between 5 and 31 months. The aim of the study was to evaluate whether the degree of HIE or the site and size of lesions on MRI could predict visual outcome. Twenty of the 31 infants studied showed abnormal results on at least one of the visual tests used. While visual function was generally normal in grade I HIE and severely impaired in grade III, visual outcome was variable in grade II. MRI findings were better predictors of visual impairment than the degree of HIE. Normal scans tended to be associated with normal visual function, irrespective of the severity of HIE. All but one of the infants with diffuse hemispheric involvement also showed multiple visual abnormalities. The concomitant involvement of basal ganglia was always associated with more severe visual outcome. These results suggest that infants with generalised lesions secondary to global insults are at high risk of severe visual impairment even in presence of normal acuity and require early assessment of various aspects of visual function.

Basal Ganglia↗

Minor neurological signs and perceptual-motor difficulties in prematurely born children.

AIM: To examine the spectrum of neurological dysfunction and perceptual-motor difficulties at school age in a cohort of prematurely born children, and the relation of these measures to neonatal brain lesions, intelligence quotient, and behavioural adjustment. METHOD: One hundred and eighty three children were tested at the age of 6 years using Touwen's Examination of the Child with Minor Neurological Dysfunction, the Movement Assessment Battery for Children (Movement ABC), the Developmental Test of Visual-Motor Integration (VMI), British Ability Scales, and Rutter Scales. RESULTS: Twenty six children had definite cerebral palsy and one was blind. Of the remaining 156, the proportions falling below the 15th centile point were 31% on Touwen's Examination, 44% on the Movement ABC, and 17% on the VMI. Forty two passed all three tests. No child with a normal ultrasound scan developed cerebral palsy, whereas nearly all those with major lesions did. Minor lesions, however, were not generally predictive of later outcome. Correlations between the tests were generally low. CONCLUSIONS: These findings stress the need to assess neurological and perceptual motor functioning separately at school age and to monitor relationships with other aspects of development.

Analysis of Variance↗

Basal ganglia damage and impaired visual function in the newborn infant.

AIM: To examine the effects of early lesions in the visual pathway on visual function; and to identify early prognostic indicators of visual abnormalities. METHODS: The visual function of 37 infants with perinatal brain lesions on magnetic resonance imaging was assessed using behavioural and electrophysiological variables. RESULTS: Normal visual behaviour was observed in most infants with large bilateral occipital lesions, but all the infants with associated basal ganglia involvement had abnormal visual function. Visual abnormalities were also present in six infants with isolated basal ganglia lesions. CONCLUSIONS: These observations suggest that basal ganglia may have an integral role in human visual development and that their presence on neonatal MRI could be an early marker of abnormal visual function.

Basal Ganglia↗

Visual function of prematurely born children with and without perceptual-motor difficulties.

The relationship between visual and perceptual-motor abilities at 6 years of age was investigated in a cohort of 141 prematurely born children without cerebral palsy. Visual acuity was assessed using the Sonksen-Silver Acuity System and stereopsis with the Titmus stereo test. Perceptual motor abilities were evaluated using the Movement Assessment Battery for Children and the Developmental Test of Visual-Motor Integration. The results showed a higher incidence of abnormalities both of linear acuity and stereopsis in the study group when compared to a group of reference children. Whereas abnormalities of linear acuity were not associated with perceptual-motor difficulties, abnormal stereopsis was significantly associated with poor performance on both perceptual-motor tests. Our results suggest that infants born preterm, even in the absence of other major neurological signs, are at risk for abnormal visual function and perceptual-motor difficulties. As these could interfere with everyday life and school performance, a longitudinal assessment of both areas of competencies is recommended so that diagnosis and possible intervention can take place as early as possible.

Brain Diseases↗

Sequential study of central and peripheral nervous system involvement in an infant with merosin-deficient congenital muscular dystrophy.

Diffuse white matter changes on brain imaging and peripheral neuropathy are associated features of merosin-deficient congenital muscular dystrophy (CMD). In this report we describe the early manifestation and evolution of brain changes, and the involvement of the peripheral nervous system in a female infant with merosin-deficient CMD diagnosed in the neonatal period who had sequential clinical, neurophysiological and magnetic resonance imaging (MRI) assessment. Both MRI and nerve conduction velocity in the first week of life failed to demonstrate any abnormality. By 6 months of age both nerve conduction and MRI were abnormal. White matter changes became more evident on a further scan at 1 yr of age and this pattern remained unchanged on the following scan performed at 17 months of age. Our findings suggest a failure in the physiological maturation process of myelination of both the central and peripheral nervous system.

Central Nervous System Diseases↗

Evaluation of the corpus callosum in clumsy children born prematurely: a functional and morphological study.

The aim of this study was to evaluate the incidence of functional and neuroradiological abnormalities of the corpus callosum in a group of 21 prematurely born children (GA < 34 weeks) who were found to be "clumsy" on the Movement Assessment Battery for Children at 6 years of age. All children underwent functional and morphological assessment of the corpus callosum. The functional assessment included tests of somesthesis, diadochokinesis and repetitive finger tapping. The morphology of the corpus callosum was evaluated on midsagittal MRI. Thirteen of the 21 clumsy children showed morphological abnormalities which were significantly associated with functional abnormalities. Morphological changes of the corpus callosum were also significantly associated with lesions on both neonatal ultrasound and late MRI. Our results support the view that morphological abnormalities of the corpus callosum are frequent in children born prematurely. The association between these abnormalities and lesions on US or MRI suggests that they are likely to be secondary to pre- or perinatal lesions.

Agenesis of Corpus Callosum↗

How local is the impact of a specific learning difficulty on premature children's evaluation of their own competence?

The aim of this study was to determine whether children's perceptions of their own competence levels reflected their actual strengths and weaknesses (Specificity Hypothesis) or transcended these (Generality Hypothesis). Harter and Pike's measure of self-perception was administered to 163 prematurely born 6-year-olds with or without motor co-ordination and/or reading problems. Associations between children's self-perceptions and their scores on standardised tests of motor co-ordination and reading were assessed in three distinct ways. These analyses produced converging results: self-perceptions of physical competence were associated specifically with performance on the Movement ABC Test, and self-perceptions of cognitive competence were associated specifically with performance on the BAS Word Reading Scale. Our results support the Specificity Hypothesis.

Achievement↗

Visual function and perinatal focal cerebral infarction.

AIMS: To evaluate the visual function of infants with perinatal cerebral infarction in whom the site and size of the lesion has been determined using magnetic resonance imaging (MRI). METHODS: Twelve infants with cerebral infarction on MRI were studied with a battery of tests specifically designed to evaluate visual function in infancy. This included tests: for visual attention (fixation shifts); of cerebral asymmetry (optokinetic nystagmus, visual fields); for assessment of acuity (forced choice preferential looking); and neurophysiological measures of vision (phase reversal and orientation reversal visual evoked potential). RESULTS: A considerable incidence of abnormalities on at least one of the tests for visual function used was observed. The presence or severity of visual abnormalities could not always be predicted by the site and extent of the lesion seen on imaging. CONCLUSIONS: Early focal lesions affecting the visual pathway can, to some extent, be compensated for by the immature developing brain. These data suggest that all the infants presenting with focal lesions need to be investigated with a detailed assessment of various aspects of vision.

Cerebral Infarction↗

Hypoxic-ischaemic encephalopathy: early and late magnetic resonance imaging findings in relation to outcome.

Sixteen infants with hypoxic-ischaemic encephalopathy (HIE) were studied using serial magnetic resonance imaging (MRI) up to the age of 2 years. The infants had regular neurological and developmental assessments. An nuclear magnetic resonance (NMR) score was devised to quantify the early and late MRI findings and a neurological optimality score was used to quantify abnormal neurological signs at the time of the final examination. The follow up MRI score was compared with the neonatal MRI score and the outcome of the child. There was a strong positive correlation between the neonatal and follow up MRI scores and between MRI scores and optimality score. All infants with a normal outcome had patchy white matter abnormalities. All infants with an abnormal outcome had extensive white matter abnormalities. The outcome was most severe in those infants with additional basal ganglia atrophy with or without cyst formation. Infants with mild HIE who are developmentally normal at the age of 2 years do not have normal MRI scans and may be at risk of minor neurological problems by school age. Bilateral basal ganglia abnormalities are associated with severe developmental delay, but infants with mainly white matter and cortical abnormalities have less severe problems despite extensive tissue loss.

Basal Ganglia↗

Minor neurological and perceptuo-motor deficits in children with congenital muscular dystrophy: correlation with brain MRI changes.

Diffuse white matter changes on magnetic resonance imaging (MRI) have been a consistent feature in some children with the "pure" form of congenital muscular dystrophy (CMD) in which there are no structural changes in the brain or severe mental retardation. The aim of this study was to assess fine motor and perceptuo-motor abilities in children with CMD with and without MRI changes. Twenty-two children with "pure" CMD were investigated with a standard neurological examination and a battery of tests (Manual dexterity from the Movement ABC, test of visual-motor integration, Zurich Neuromotor test) which have already been used to detect minor neurological signs related to white matter changes. The cohort was then divided in two groups for analysis depending on the presence or the absence of diffuse white matter changes. A significant difference was found for all the tests between the group of the CMD children with normal MRI and the group with diffuse white matter changes. The manual dexterity and the Zurich Neuromotor tests showed a greater sensitivity than the test of visual-motor integration, which had some false negatives. It is of interest that in the group with diffuse white matter changes the presence of contractures or weakness did not seem to affect the quality of the performance; all these children scored abnormally on the test, irrespective of the severity or the extent of contractures and weakness. In contrast, in children with normal MRI severe contractures and weakness did affect the performances. Our results demonstrate that perceptuo-motor difficulties and minor neurological soft signs are a consistent feature in CMD children with diffuse MRI changes but not with normal MRI.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Ischaemic and haemorrhagic brain lesions in newborns with seizures and normal Apgar scores.

Serial ultrasound scans and conventional and diffusion weighted magnetic resonance imaging (MRI) were performed on 16 neonates who presented with seizures. The Apgar scores were normal and subsequently no metabolic or infective cause could be found. The aim of the study was to evaluate the extent to which early sequential imaging can elucidate the cause of seizures in apparently neurologically normal infants. Fourteen of the infants had haemorrhagic or ischaemic lesions on MRI and these were detected by ultrasound scanning in 11. Early ultrasound scanning detected the haemorrhagic lesions but the ischaemic lesions were often not seen until the end of the first week of life. Early MRI, however, was able to detect all the ischaemic lesions. The evolution of the insult could be timed by using serial ultrasound scans and a combination of diffusion weighted and conventional MRI during the first week of life, confirming a perinatal insult even in the absence of fetal distress. Although the aetiology of these lesions remains obscure, serial ultrasound scans will detect the presence of cerebral lesions in neonates presenting with isolated seizures but additional MRI sequences will give better definition on type, site, and extent of the pathology.

Apgar Score↗

Neurological 'soft' signs may identify children with sickle cell disease who are at risk for stroke.

UNLABELLED: Stroke is one of the most frequent complications of sickle cell disease (HbSS), occurring in 7-17% of children. Recent studies recognized more minor lesions on MRI, not associated with clinical signs on standard neurological examination, which however have been found to be a risk factor for developing stroke later. The aim of this study was to evaluate whether minor lesions observed on imaging could be associated with 'soft' neurological signs not detectable on conventional neurological examination. Fourteen children with HbSS were assessed with MRI, standard neurological examination and evaluation of 'soft' signs (Zurich Neuromotor Test) and motor function (Movement ABC). Eight of the 14 children scanned showed lesions on MRI but only 3 of the full cohort were abnormal on standard neurological examination. However, all of the eight children with MRI lesions also showed abnormal signs on at least one of the two tests (Zurich and Movement ABC). All the children with normal MRI were normal on all the tests performed. The sensitivity of Zurich Neuromotor Test and Movement ABC in the group of children with MRI lesions is 0.88 and 0.75, respectively, and increases to 1 when the two tests are used together. The specificity of both tests is 1 even when the tests are used separately. CONCLUSION: Although the number of cases is small, 'soft' signs may reliably identify the presence of even minor MRI lesions, allow the evaluation of the global incidence of major and minor neurological signs and may also help to identify the population at risk for developing strokes. This population could then be closely monitored and benefit from early intervention.

Adolescent↗

Birthweight and gestation of village deliveries in Papua New Guinea.

This study investigates the birthweight, gestational age, and body proportions of home births in a malarious area of Papua New Guinea. A population based cohort of pregnant women was followed longitudinally through pregnancy. Within the first few days of birth anthropometric measures were taken and a gestational age assessment made. Of the 121 live singleton infants, forty per cent were low birthweight and the preterm rate was 4 per cent. Infants appeared to be proportionally growth retarded. Low birthweight was associated with short maternal stature, and preterm delivery maternal illness. Mean birthweight derived from hospital statistics of babies born to women from the same area was higher than the mean derived from this population based study.

Anthropometry↗