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Biomedical subjects

David Neubauer

Publications and source records attributed to David Neubauer.

12 recordsLinked to original sources
PubMed2006

Head growth in infants with infantile spasms may be temporarily reduced.

Epileptic activity, as a component of epileptic encephalopathies, can interfere with brain growth and development. Infantile spasms as a syndrome represent such epileptic activity during the period of spasms and hypsarrhythmia. The rate of head growth in infants with infantile spasms during the period of spasms has not been studied previously. A retrospective study of head growth in 38 infants with infantile spasms and no other cause of abnormal head growth is presented. Mental outcome was assessed at follow-up. The mean head circumference of infants with infantile spasms was not significantly smaller than in the normal population, but the proportion of head circumference below the tenth percentile in the infantile spasms group was higher (27%). Head circumference below the tenth percentile in the fourth and fifth month after the onset of infantile spasms was significantly associated with later mental retardation (P = 0.004). There was no correlation with specific treatment of infantile spasms. Transiently diminished head growth in infants with infantile spasms coincides temporally with the onset of infantile spasms and "catches up" during remission of infantile spasms in favorable cases. This pattern can reflect the negative influence of epileptic activity on brain growth during the sensitive period. Head circumference can provide a reliable predictive value of mental outcome in children with infantile spasms.

Case-Control Studies↗
PubMed2006

The risk of lower mental outcome in infantile spasms increases after three weeks of hypsarrhythmia duration.

To assess the correlation between hypsarrhythmia duration and mental outcome in infantile spasms (IS) the medical records of 48 infants with IS were reviewed retrospectively and psychological assessments undertaken at follow-up at the age of 3 to 13 years. We found 18 (38%) cryptogenic IS cases with typical hypsarrhythmia and 30 symptomatic with modified hypsarrhythmia-further classified into 15 cases as multifocal, 10 as pseudoperiodic and 5 as unilateral hypsarrhythmia. A short treatment lag (one to two weeks) occurred in 25, three to four weeks in 10 cases. Spasms ceased within one month after treatment in 23 infants. At follow-up 15 children had normal mental outcome (borderline included). A correlation between hypsarrhythmia duration longer than three weeks and lower mental outcome was found using the logistic regression model. The duration of hypsarrhythmia represents a sensitive prognostic parameter in IS; the risk of mental retardation increases after three weeks of hypsarrhythmia.

Age of Onset↗
PubMed2006

Amplitude-integrated electroencephalography in full-term newborns without severe hypoxic-ischemic encephalopathy: case series.

AIM: To assess the diagnostic value of amplitude-integrated electroencephalography (EEG) in comparison to standard EEG in newborns without severe hypoxic-ischemic encephalopathy who were at risk for seizures. METHODS: The study included a consecutive series of 18 term newborns without severe hypoxic-ischemic encephalopathy, but with clinical signs suspicious of epileptic seizures, history of loss of social contact, disturbance of muscle tone, hyperirritability, and/or jitteriness. Amplitude-integrated and standard EEG tracings were assessed for background pattern, epileptiform activity, and sleep-wake cycling. RESULTS: Amplitude-integrated EEG and standard EEG recordings of 15 newborns were suitable for analysis. Only two different background patterns were seen on amplitude-integrated EEG and standard EEG, with the absence of severely abnormal background patterns. Of 15 newborns, epileptiform discharges were present on amplitude-integrated EEG in 3 newborns, and on standard EEG in 6 newborns. Sensitivity of seizures discharges on amplitude-integrated EEG to correspond with epileptiform discharges on standard EEG was 50%; specificity 100%,positive predictive value 100%, and negative predictive value 75%. Of 4 newborns suspected of having sleep myoclonus, amplitude-integrated EEG correctly identified the newborn who had epileptiform activity on standard EEG. CONCLUSION: The diagnostic value of amplitude-integrated EEG monitoring of term newborns without severe hypoxic-ischemic encephalopathy is limited, but could have a role in evaluating presence or absence of epileptiform activity and in differentiating non-epileptic movement from seizures.

Electroencephalography↗
PubMed2005

Comparison of two methods of neurologic assessment in infants.

Information provided by the neonatal neurologic assessment is important for identifying infants with neurologic abnormalities at a very early age. The aim of this study was to compare two distinct approaches to the neurologic assessment of newborns: the Amiel-Tison neurologic assessment, and Prechtl's qualitative assessment of general movements. The results of both assessments were studied in a group of 45 preterm infants with different risk factors for brain damage that were compared at term age and at a corrected age of 3 months. The predictive power of the two methods regarding the neurologic and developmental outcome at a corrected age of 12-15 months was analyzed. The agreement of the two methods was excellent at term (kappa = 0.87) and good at 3 months (kappa = 0.54). The sensitivity of both methods for detecting children with neurologic abnormalities was high both at term and at 3 months (0.92, 1.0 for the Amiel-Tison assessment and 0.96, 1.0 for general movements). The specificity of both techniques was low at both ages (0.45, 0.75 for the Amiel-Tison assessment and 0.40, 0.35 for general movements). The agreement of the neurologic and developmental outcome was better with the Amiel-Tison assessment (kappa = 0.39, 0.77) than with the observation of general movements (kappa = 0.38, 0.37).

Child Development↗
PubMed2005

Amiel-Tison Neurological Assessment at term age: clinical application, correlation with other methods, and outcome at 12 to 15 months.

The aims of this study were: (1) to perform the Amiel-Tison Neurological Assessment (ATNA) in a group of infants with different risk factors for brain damage; (2) to analyze the results of the examinations in light of the risk factors and presumed aetiology; (3) to compare results of examinations with results of cranial ultrasound, electroencephalography (EEG), and cerebral function monitoring (CFM); and (4) to evaluate neurological outcome at 12 to 15 months of age using the Amiel-Tison and Gosselin method, and developmental outcome using the Bayley Scales of Infant Development. Participants were 52 term, newborn infants (31 males, 21 females) with risk factors for brain damage. Mean birthweight was 3288g (SD 661g) and mean gestational age was 39.4wks (SD 1.2wks); range 38 to 41.3wks. Mean age at admission to a neonatal special care unit was 75h, (SD 13.7h). The group with a dynamic (evolving) clinical profile differed significantly from the group with a static (stable) profile in terms of aetiology, while the group with signs of prenatal brain damage differed from the group without these signs regarding aetiology and the level of severity of neurological signs. Sensitivity of the ATNA to detect infants with abnormal ultrasound was 0.97, with EEG 0.89, and with CFM 0.88. At follow-up at 12 to 15 months 47 children were examined: neurological examination was normal in 25 and five children had a minor, five a moderate, and 12 a severe neurological deficit. Agreement of the ATNA with neurological and developmental assessment at follow-up was very good. Our findings suggest that the ATNA is also of value in assessing aetiology and timing of brain lesions.

Brain Damage, Chronic↗
PubMed2004

Congenital hypotonia: is there an algorithm?

This study was performed with the aim of determining the diagnostic profile of newborns with hypotonia and of analyzing the usefulness of different procedures in the diagnostic process. One hundred thirty-eight hypotonic newborns were identified through the search of hospital records in a 10-year period: 121 (88%) had central hypotonia and 13 (9%) had peripheral hypotonia, whereas 4 (3%) remained unclassified. Analysis of the contribution of clinical data and results of investigations led to the construction of an algorithm, by which all cases in the group were diagnosed. Step 1, which included clinical data and results of examinations, solved 50% of all diagnosed cases. Neuroimaging techniques made up step 2 and contributed to the diagnosis in 13%. Step 3 was accomplished by a search through Oxford Medical Databases, which yielded the final diagnosis in 9%, whereas karyotyping and fluorescent in situ hybridization for Prader-Willi syndrome comprised step 4 and contributed to the diagnosis in 6.5%. Biochemical tests formed step 5 and contributed to the diagnosis in 6%. Step 6, which included specific investigations of muscle and nerve, was diagnostic in 6%. The remaining cases (6.5%) were diagnosed only after several follow-up examinations. These results could assist the neonatologist when deciding the diagnostic approach to floppy newborns.

Algorithms↗
PubMed2002

An early onset form of methylenetetrahydrofolate reductase deficiency: a report of a family from Kuwait.

Methylenetetrahydrofolate reductase deficiency (MTHFR) is a rare autosomal recessive disorder. There have been 68 cases reported to date in the literature [Eur J Pediatr 1998;157 (Suppl 2):S77]. It affects intracellular folate metabolism and results in homocystinuria and hypomethionemia. We report a family in which three children (two boys and one girl) died before the age of 3 months with severe MTHFR deficiency. A fourth affected boy was treated with betaine and he improved clinically and biochemically. We demonstrate the unique dermatological and brain imaging features in a kindred from Kuwait.

Brain↗
PubMed2002

Epidemiologic features of infantile spasms in Slovenia.

PURPOSE: To determine the epidemiologic features of infantile spasms (ISs) in Slovenia. METHODS: Medical records of all children with ISs in Slovenia in the period from 1985 to 1995, based on community pediatrician referrals to four hospitals, including all pediatric EEG laboratories, were retrospectively studied. The outcome was assessed by a follow-up study in 1998. RESULTS: Forty-seven children with ISs were identified over an 11-year interval. The cumulative incidence was 2.06 per 10,000 live births. Among 29 (61.7%) children with symptomatic etiology, 14 cases had prenatal etiology [tuberous sclerosis (TS), seven; vascular insult, three; cerebral malformations, three; Down syndrome, one child], 14 perinatal and one postnatal cause: anoxic brain damage after cardiac surgery. Cryptogenic and idiopathic etiology were diagnosed in 13 (27.6%) and five (10.6%) of 47 cases, respectively. The age of onset of ISs ranged from 2 to 10 months. As initial treatment, steroids were used in 19 children (remission in 10); vigabatrin in seven (remission in four), and other antiepileptic drugs (AEDs) in 20 children (remission in six). According to the follow-up study, 18 (38.3%) children were seizure free, and 14 of them had normal mental development. Among 29 mentally retarded children (14 severely), 26 belonged to the symptomatic group. Four children died. CONCLUSIONS: The incidence of ISs in Slovenia is similar to that in some parts of the United States, but lower than that in Finland or Sweden. The outcome depends mainly on etiology. Additional neuroimaging studies are needed for evaluation of cryptogenic cases.

Adrenocorticotropic Hormone↗