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Biomedical subjects

P Casaer

Publications and source records attributed to P Casaer.

At least 19 recordsLinked to original sources

Neonatal neurological examination as a predictor of neuromotor outcome at 4 months in term low-Apgar-score babies in Zimbabwe.

The predictive value of the neonatal neurological examination (NNE) adapted from Prechtl, was investigated in 139 term Zimbabwean infants born with an Apgar score of five or less at 5 min. At 4 months, seven infants had died and 13 were lost to follow-up, leaving 119 infants to undergo the Infant Motor Screen (IMS). Eighty-eight infants were diagnosed as normal, six as suspect and 25 as abnormal at screening. The sensitivity, specificity, positive predictive value and negative predictive value of the NNE were 94%, 55%, 42% and 96%, respectively. Seventeen (14%) infants had developed microcephaly at 4 months and 13 (77%) of them scored abnormal on the IMS. Twenty-three of the 48 (48%) infants who had convulsions within 48 h of birth, were diagnosed as abnormal (P < 0.0001). The NNE proved to be very sensitive in detecting neurodevelopmental abnormalities in the neonatal period and the five abnormal syndromes derived from the NNE were able to correctly identify 94% of the abnormal infants.

Adolescent

Neurological status in severely jaundiced Zimbabwean neonates.

Neurological status was studied in 50 jaundiced infants with a total serum bilirubin of > 400 mumol/l (23.4 mg/dl). Infants were assessed in the neonatal period with the Neonatal Neurological Examination and 4 months of age with the Infant Motor Screen. Twenty-six (52 per cent) infants were premature. Analysis of variance did not show a significant difference between gestational age, birth weight, and maximum total serum bilirubin or between gestational age, birth weight, and neurological optimality score. Based on the presence of abnormal neurological syndromes the infants were classified as normal (n = 27), suspect (n = 11), or abnormal (n = 12). Serum bilirubin levels were higher (p < 0.0001) and the neonatal neurological examination scores lower (p < 0.0001) in the seven (14 per cent) infants who received an exchange transfusion. In the transfused group four out of seven infants and in the non-transfused group seven out of 43 infants were classified as abnormal (p < 0.03). The Neonatal Neurological Examination was shown to be sensitive in detecting neurodevelopmental abnormalities in the neonatal period, with a sensitivity of 83 per cent, specificity of 88 per cent, positive predictive value of 62 per cent, and negative predictive value of 96 per cent.

Analysis of Variance

Epidermoid tumours associated with lumbar punctures performed in early neonatal life.

Three children (ages 5, 7, and 12 years) with epidermoid tumours in the spinal canal, all of whom had a lumbar puncture during the early neonatal period, are reported. A considerable delay occurred from the first symptoms until the diagnosis was made. MRI of the lumbar spine was the method of choice in the diagnostic work-up. All three cases were successfully surgically treated. The link between lumbar punctures and epidermoid tumours and the possible risk factors involved are explored. Because of the variable clinical presentation, the possibility of the existence of these tumours should be considered in the differential diagnosis.

Age Factors

Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release.

OBJECTIVE: To investigate the influence of neurosurgical intervention on the appearance of upper motor neuron (UMN) signs in newborns diagnosed with occult spinal dysraphism and tethered cord (TC) during the first month of life. METHODS: A prospective study (1990 to 1996) of 22 consecutive newborns with occult spinal dysraphism monitored for the appearance of UMN signs. Untethering was performed when neurologic or urodynamic investigation indicated the presence of UMN dysfunction. RESULTS: Of 22 patients, 10 remained free of UMN symptoms during follow-up (mean, 67+/-22 months). Untethering was performed in 12 of 22 patients because of the presence of UMN symptoms. In 7 of these 12 patients, there was a documented asymptomatic period of 13+/-11 months before the onset of UMN symptoms. Untethering at a mean age of 18+/-17 months restored normal neurologic and urinary function in all patients (mean postoperative follow-up, 25+/-16 months). Of the 12 children, 5 presented with UMN signs at birth. In these children, untethering was performed at a mean age of 9+/-5 months. In two of these five patients, UMN symptoms did not resolve after surgery, and ongoing conservative bladder treatment was required (mean follow-up, 37+/-14 months). In none of the 12 operated children did signs of retethering occur. CONCLUSIONS: A significant number (10/22) of children born with occult spinal dysraphism and TC did not develop UMN symptoms during follow-up; neurosurgical correction after the appearance of an UMN sign restored normal neurologic and urinary function in all children; and untethering in children presenting at birth with UMN symptoms resulted in poorer outcome.

Child, Preschool

MR of cerebellar cortical dysplasia.

MR imaging findings are described in four patients with cerebellar cortical dysplasia. Typically, cerebellar disorganized folia were seen as an irregular bumpy gray-white matter interface. In addition, cystlike cortical abnormalities were observed in two patients and associated supratentorial developmental abnormalities were seen in three patients. To our knowledge, cerebellar cortical dysplasia without supratentorial abnormalities, as seen in one patient, has not been reported before. We suggest that cerebellar cortical dysplasia represents a spectrum of abnormalities ranging from mild to extensive in severity.

Adolescent

The predictive value of developmental testing of extremely jaundiced African infants.

The predictive value of the Neonatal Neurological Examination (NNE) adapted from Prechtl (1977) and the Infant Motor Screen (IMS) from Nickel (1989) at 4 months was studied in severely jaundiced infants in Zimbabwe. Fifty infants were examined with the NNE, 41 with the IMS and 43 with the Bayley Scales of Infant Development (BSID) (Bayley 1969). Five infants had choreoathetosis and six had a motor delay at age 1 year. The NNE and IMS proved to be sensitive instruments particularly when two infants who became malnourished after the neonatal period were excluded. Logistic regression was used to investigate the relation between the BSID and five selected predictors from the NNE. This resulted in a correct classification of 93%. By using only the predictors acoustic blink and traction response, 80% of the infants were correctly classified but the number of false negatives was reduced from three to one.

Black People

The X-linked infantile spasms syndrome (MIM 308350) maps to Xp11.4-Xpter in two pedigrees.

We report 2 families with X-linked infantile spasms syndrome (X-linked West syndrome). Data from clinical examination, biochemical analysis, neuroimaging, and neuropathology are discussed. In these families, genetic linkage analysis was able to locate the disease gene to the distal part of the short arm of the X chromosome, between Xpter and Xp11.4. This is the first report of linkage with genetic markers in this disorder. Although most cases are sporadic, further unraveling of the genetic background of the familial cases might greatly improve our understanding of infantile spasms.

Child, Preschool

Use of external drainage for posthemorrhagic hydrocephalus in very low birth weight premature infants.

To treat progressive posthemorrhagic hydrocephalus we used early external ventricular drainage (EVD) in 14 premature infants. We think it is important that the catheters in these critically ill infants be inserted in the neonatal intensive care unit, allowing us to keep the infants in an extremely stable environment. Only after prolonged external ventricular drainage (on average 38.4 days) is a ventriculoperitoneal shunt considered, preferably when the child has reached a body weight of 2000 g. There were no infections or other severe drainage-related problems. We report mean daily EVD volumes (which are related to body weight) and EVD duration. The 14 patients included 9 who required permanent shunting. Comparing the mortality, morbidity and follow-up data to at least 3 months of age in this group with similar data for an earlier cohort treated with lumbar punctures and late permanent shunting, we demonstrate the safety of the policy we have recently adopted.

Cerebral Hemorrhage

Empty sella, growth hormone deficiency and pseudotumour cerebri: effect of initiation, withdrawal and resumption of growth hormone therapy.

UNLABELLED: An 11-year-old boy with normal visus and eye fundus, but with empty sella, growth hormone (GH) deficiency and central diabetes insipidus was found to have intracranial hypertension with papilloedema after 6 months of catch-up growth under recombinant human GH (rhGH) replacement therapy. Withdrawal of rhGH therapy was associated with normalisation of intracranial pressure within 1 week. Three months later, resumption of rhGH therapy at a lower dose was again followed by pronounced growth acceleration, but now without papilloedema. CONCLUSION: Children with empty sella and GH deficiency may be prone to rhGH-induced pseudotumour cerebri which appears to be rapidly reversible and dose-dependent.

Child

Extreme hyperbilirubinaemia in Zimbabwean neonates: neurodevelopmental outcome at 4 months.

UNLABELLED: As part of a prospective study of severely jaundiced Zimbabwean infants, the relationship between maximum total serum bilirubin (TSB) concentration in the neonatal period and neurodevelopmental outcome at the corrected age of 4 months was studied. Fifty infants with a TSB of > 400 micromol/l (23.4 mg/dl) were enrolled and screened with a neonatal neurological examination (NNE). The cause of jaundice was low birth weight in 22 (44%), ABO incompatability in 8 (16%), sepsis in 8 (16%) and congenital syphilis (6%) in 3 infants. In 9 infants a cause could not be determined. At 4 months, 2 infants had died and 3 were lost to follow up, leaving 45 infants for the infant motor screen (IMS) at 4 months of age. Mean TSB in the neonatal period was 485 micromol/l (28.2 mg/dl), and 7 infants received an exchange transfusion. Mean TSB of the infants with an exchange transfusion was 637 micromol/l (37.2 mg/dl) (range 429-865 micromol/l (25-50.3 mg/dl)) and of the infants without transfusion 459 micromol/l (26.8 mg/dl) (range 400 740 micromol/l (23.4-43 mg/dl)) (P < 0.0001). The TSB was not associated with birth weight, gestational age, gender or head circumference of the baby. On the IMS, 6 of 45 (13.3%) infants scored abnormal, 6 (13.3%) suspect and 33 (73%) scored normal. Three of the six (50%) remaining infants who received an exchange transfusion scored abnormal on the IMS while only 3 of the 39 (8%) infants without exchange transfusion were abnormal. CONCLUSION: More than 25% of infants with a TSB of > 400 micromol/l (23.4 mg/dl) scored abnormal or suspect at 4 months of age and half of these infants already showed irreversible neurological symptoms. All infants who scored abnormal or suspect on the IMS with bilirubin levels between 400 and 500 micromol/l (23.4 and 29.2 mg/dl) had haemolytic disease or were premature.

Bilirubin

Neurological findings in neonates with low Apgar in Zimbabwe.

OBJECTIVES: Document neurological condition of African neonates with a low apgar score. SETTING: Mpilo Hospital, Bulawayo, Zimbabwe. SUBJECTS: 165 babies with an Apgar score of 5 or less at 5 min. METHODS: Neurological examination at term age according to Prechtl. Babies were classified as normal, suspect or abnormal and compared with two reference groups, one from Groningen, the Netherlands and one from Grenada in the Caribbean. RESULTS: A higher number of Zimbabwean babies were delivered by Caesarean section compared to the Groningen group (P < 0.001). Babies delivered by vacuum extraction scored significantly lower compared to babies delivered by Caesarean section (P < 0.003). Twenty abnormal signs derived from the neonatal neurological examination proved to be predictive on the total optimality score (P < 0.001). The number of infants who were classified as abnormal was higher in the Zimbabwean population (P < 0.01). CONCLUSION: The selected abnormal signs derived from the neonatal neurological examination proved to be highly predictive on the neurological condition. The neonatal morbidity in Zimbabwean neonates with a low Apgar score was higher when compared with two reference groups from Groningen and Grenada.

Adolescent

Stepwise decrease in VEP latencies and the process of myelination in the human visual pathway.

To assess the progress in myelination in the developing human brain, a prospective longitudinal study of flash visual evoked potentials (VEPs) was performed in 22 healthy preterm infants with the same gestational age at birth (between 30 weeks 0 day and 31 weeks 0 day). The individual curves of the changes in the N1a peak latency (the early peak of the N1 wave) decrease not linearly but in a stepwise pattern in the preterm period. Twenty-one infants out of the 22 have one or more 'acceleration week(s)' in which the latency decreases at a rate of more than 6 ms per week. These stepwise decreases in the latency may reflect a synchronized progress in myelination in several parts of the visual pathway. A detailed analysis of the 'acceleration weeks' in relation to postmenstrual age (PMA) indicates that they most prominently occur at 37 weeks PMA. At 37 weeks an initiation of myelination in the optic radiation has been demonstrated in post-mortem studies. We propose that a longitudinal follow-up study of VEPs can be accepted as a functional in vivo evaluation of myelination in the developing human brain.

Child Development

The effect of behavioural states on cerebral oxygenation during endotracheal suctioning of preterm babies.

Near infrared spectroscopy (NIRS) was used to investigate the effect of behavioural states on changes of oxygenated (O2Hb), deoxygenated haemoglobin (HHb) and total haemoglobin (tHb), during endotracheal suctioning. In an open prospective design, NIRS measurements have been done during 20 suctioning episodes in 13 preterm neonates. Heart rate, arterial oxygen saturation, and carbon dioxide tension were monitored continuously. Behavioural state (BS) observations were made and documented as well. The statistical analysis showed that in patients who were active, with crying periods during suctioning (behavioural states 4-5), changes of oxygenated (p < 0.005) and deoxygenated haemoglobin (p < 0.05), as well as of arterial oxygen saturation (p < 0.05) and heart rate (p < 0.05) were significantly greater than in patients who were quiet with predominant behavioural state 1, 2 and 3. These results underline the influence of behavioural states on the physiological answers to endotracheal suctioning. NIRS proved to be a valuable tool to evaluate possible harmful effects of different suctioning techniques.

Cerebrovascular Circulation

Neurodevelopmental outcome in babies with a low Apgar score from Zimbabwe.

The early identification of neurological dysfunction in the neonatal period, the predictive value of single items of the neonatal neurological examination (NNE) adapted from Prechtl and the developmental outcome at 1 year of age in infants with a low Apgar score in Zimbabwe were studied. One hundred and sixty-five infants were examined with the NNE and 142 with the Bayley Scale of Infant Development (BSID) at 1 year of age. Twenty-three infants had cerebral palsy, ten had a motor delay or developmental delay, and four were mentally retarded. The NNE proved to be a sensitive instrument for detecting neurodevelopmental abnormality. Logistic regression analysis was used to investigate the relationship between the BSID and nine selected predictors from the NNE. This resulted in a correct classification of 94%. However, the number of false negatives was high. By using only the variability of movements and fixation as predictors the number of false negatives was reduced to one.

Apgar Score

Normal brain perfusion pattern of technetium-99m-ethylcysteinate dimer in children.

UNLABELLED: The purpose of this study was to assess the normal perfusion pattern of the pediatric brain with 99mTc-ethylcysteinate dimer (99mTc-ECD). METHODS: Tomographic imaging was performed with a dedicated system with high sensitivity and resolution. Sixteen children, referred for brain imaging in the workup of seizure disorder, were included since they turned out negative after a 1-yr follow-up. A standardized brain presentation was obtained after reslicing and reorienting of the three-dimensional volumetric dataset. RESULTS: Quantitative analysis did not reveal significant left-right uptake differences per patient. Three age clusters were investigated that showed differences in regional uptake, mainly a relatively increased uptake in basal ganglia, visual and motor cortex. An uptake ratio or perfusion index was calculated after normalization. Normal limits were established for the children in the three groups. CONCLUSION: Technetium-99m-ECD is a safe agent for children and should be the radiopharmaceutical of choice for brain perfusion studies because of favorable radiation dosimetry and stability. The age dependence of perfusion necessitates a database comparison before concluding that the observed perfusion pattern is normal.

Adolescent

Use of a ventriculosubgaleal shunt in the management of hydrocephalus in children with posterior fossa tumors.

We present our experience with the use of ventriculosubgaleal shunting (VSGS) for the management of hydrocephalus associated with posterior fossa tumors in a consecutive series of 63 children. In 42 children, a temporary VSGS was inserted. There were no complications associated with the procedure, and it gave a period of several days in which to improve the general condition of the child, and to perform the necessary imaging studies, without an encumbering external drainage device. In most cases the VSGS could be removed after the posterior fossa surgery, without new general anesthesia. Other advantages and drawbacks of this technique are discussed.

Brain Neoplasms

Add-on therapy of fenfluramine in intractable self-induced epilepsy.

Eleven institutionalized children (7 girls, 4 boys) with refractory epilepsy received fenfluramine as add-on therapy. Besides therapy resistance, the patients manifested self-induction of seizures. Eight patients were photosensitive and/or pattern sensitive. All antiepileptic drugs of doubtful efficacy were withdrawn. During withdrawal, patients did neither improve nor show an increase in seizure frequency. Fenfluramine was then added and administered once to three times daily at dosages between 0.5 and 1 mg/kg.day. Side effects were mild and transient. Of the 11 patients, 7 became seizure-free and in 4 patients a more than 75% decrease of all seizures was obtained.

Adolescent