Anna Beke

Newborn infants process pitch intervals.

OBJECTIVE We investigated whether the auditory system of newborn babies extracts the constancy of a pitch interval from exemplars varying in absolute pitch. METHODS Event-related brain potentials (ERP) were recorded from healthy newborn infants in an oddball paradigm consisting of frequent standard and infrequent deviant tone pairs. Tone pairs varied in absolute frequency. Standard and deviant pairs differed in the amount of pitch difference within the pairs, but not in the direction of pitch change. RESULTS Deviant tone pairs elicited a discriminative ERP response. CONCLUSIONS This result suggests that the neonate auditory system represents pitch intervals similarly to adults. SIGNIFICANCE Adult-like processing of pitch intervals allows newborn infants to learn music, speech prosody, and to process various important auditory cues based on spectral acoustic features.

Associated malformations in congenital diaphragmatic hernia cases in the last 15 years in a tertiary referral institute

This is a review of cases of perinatally diagnosed congenital diaphragmatic hernia (CDH) with associated malformations with regard to time of diagnosis, side of hernia, associated malformations, and outcome. The authors analyzed the data of CDH cases with associated malformations from records of the I. Department of Obstetrics and Gynecology, Semmelweis University Faculty of Medicine, Budapest, between July 1, 1990 and June 30, 2005. The observed period was analyzed in two parts. The pre‐ and postnatal examinations verified CDH in 100 cases, 71% of which were associated with other malformations. In 52% (37/71) CDH was diagnosed before the 24th week of gestation. The rate of early diagnosed cases doubled in the second period. Ten percent (7/71) of cases were verified postnatally. Between 1990 and 1997, the percentage of right‐sided hernia was 6% (2/34) while in the second period it was 19% (7/37). The association with cardiovascular, chest, and craniofacial anomalies doubled in the second period, while association of central nervous system anomalies halved. Chromosome anomalies were verified in four cases. Pregnancy was terminated in 53% (34/64) and 13% (4/30) of newborn infants survived the perinatal period. Intrauterine or intrapartum death occurred in 8% (5/64) of cases. The prevalence of cardiovascular, pulmonary, and craniofacial anomalies doubled, while that of central nervous system malformations decreased. The prevalence of other associated malformations has not changed significantly between the two periods. The improvement of technical facilities and accumulated experience make it possible to identify a higher number of associated malformations before the 24th week of gestation.

Can neurophysiological assessment improve timing of intervention in posthaemorrhagic ventricular dilatation

Objective Intraventricular haemorrhage is still the most common cause of brain lesion in preterm infants and development of a posthaemorrhagic ventricular dilatation (PHVD) can lead to additional neurological sequelae. Flash visual evoked potentials (fVEP) and amplitude-integrated electroencephalography (aEEG) are non-invasive neurophysiological monitoring tools. The aim of the study was to evaluate fVEPs and aEEGs in preterm infants with progressive PHVD prior to and after neurosurgical intervention for cerebrospinal fluid removal and to correlate the findings with severity of ventricular dilatation. Design fVEPs and aEEGs were performed weekly in infants with developing PHVD. As soon as the ventricular index reached the 97th percentile recordings were performed twice a week. Methods 17 patients admitted to the neonatal intensive care unit of the Medical University of Vienna who developed progressive PHVD were evaluated using fVEP and aEEG until and after reduction of intracranial pressure by placement of an external ventricular drainage. Results In all 17 cases (100%) wave latencies of fVEP increased above normal range and aEEG showed increased suppression in 13 patients (76%) with increasing ventricular dilatation. Both methods showed normalisation of patterns mostly within a week of successful therapeutic intervention (mean 8.5 days). Both changes in fVEP latencies and aEEG background patterns were detected before clinical signs of elevated intracranial pressure occurred. In only 10 patients (58.8%) ventricular width exceeded the 97th percentile+4 mm. Conclusions fVEP and aEEG are useful additional tools for the evaluation of preterm infants with progressive PHVD.

Follow-up studies of newborn-babies with congenital ventriculomegaly

Abstract The authors present the results of their follow-up studies of data from the last four years on patients with congenital ventriculomegaly. Objective: To study the psychomotor and mental developmental outcome of neonates with congenital ventriculomegaly diagnosed prenatally. In addition, patients were also classified into subgroups with subgrouping based on the etiology and complications of congenital ventriculomegaly and on the absence of ventriculoperitoneal shunt placement, and the findings compared between the different subgroups. Methods: Level 3 fetal ultrasonography was used for the prenatal diagnosis of congenital ventriculomegaly in 30 infants. Using neurological examination and the modified Brunet-Lézine infant test performed postnatal follow-up of motor and sensory development and intelligence, respectively. Results: The results show thirteen symptomless, well-developing patients, ten moderately handicapped patients and seven severely handicapped patients. Newborns with isolated, moderate ventriculomegaly have the best outcome.

Effect of maturation on suprasegmental speech processing in full- and preterm infants: a mismatch negativity study.

Infants born prematurely are at higher risk for later linguistic deficits present in delayed or atypical processing of phonetic and prosodic information. In order to be able to specify the nature of this atypical development, it is important to investigate the role of early experience in language perception. According to the concept of Gonzalez-Gomez and Nazzi (2012) there is a special intrauterine sensitivity to the prosodic features of languages that should have a special role in language acquisition. Therefore, we may also assume that pre- and full-term infants having months difference in intrauterine experience show different maturation patterns of processing prosodic and phonetic information present at word level. The aim of our study was to investigate the effect of these differences on word stress pattern vs. phoneme information processing. Two age groups of infants (6 and 10 month-olds) were included in our study. 21 of 46 of the total of infants investigated were prematurely born with low birth weight. We used the mismatch negativity (MMN) event related brain potential (ERP) component, a widely used electrophysiological correlate of acoustic change detection, for testing the assumed developmental changes of phoneme and word stress discrimination. In a passive oddball paradigm we used a word as standard, a pseudo-word as phoneme deviant, and an illegally uttered word as stress deviant. Our results showed no differences in MMN responses in the phoneme deviant condition between the groups, meaning a relatively intact maturation of phoneme processing of preterm infants as compared to their contemporaries. However, the mismatch responses measured in the stress condition revealed significant between-group differences. These results strengthen the view that the total length of intrauterine experience influences the time of emergence of prosodic processing.

Mental State Understanding in Children with Agenesis of the Corpus Callosum.

Impaired social functioning is a well-known outcome of individuals with agenesis of the corpus callosum. Social deficits in nonliteral language comprehension, humor, social reasoning, and recognition of facial expression have all been documented in adults with agenesis of the corpus callosum. In the present study, we examined the emotional and mentalizing deficits that contributing to the social-cognitive development in children with isolated corpus callosum agenesia, including emotion recognition, theory of mind, executive function, working memory, and behavioral impairments as assessed by the parents. The study involved children between the age of 6 and 8 years along with typically developing children who were matched by IQ, age, gender, education, and caregivers education. The findings indicated that children with agenesis of the corpus callosum exhibited mild impairments in all social factors (recognizing emotions, understanding theory of mind), and showed more behavioral problems than control children. Taken together, these findings suggest that reduced callosal connectivity may contribute to the development of higher-order social-cognitive deficits, involving limits of complex and rapidly occurring social information to be processed. The studies of AgCC shed lights of the role of structural connectivity across the hemispheres in neurodevelopmental disorders.

A 7 és 15 éves vak gyermekek emlékezeti, nyelvi és téri feldolgozás mutatói

A latasserult populacio kognitiv vizsgalata meresmetodikai problemak miatt nehezsegbe utkozik. Ez kifejezetten ervenyes a vak gyermekek meresere. Tanulmanyunk celja a korabban es jelenleg is hasznalt populaciospecifikus tesztbatteriak bemutatasa, majd az altalunk kialakitott es pszichometriai elemzesnek alavetett feladatsor hasznalataval felmert 7–15 eves teljes magyar ep ertelmű vak populacio es illesztett lato kontrollcsoport kognitiv profiljanak elemzese. A feladatok megvalasztasi szempontjainal merlegelesre kerultek a jelenlegi intelligenciakutatasok altal megerősitett főbb dimenziok, az alkalmazhatosag es a tesztek elerhetősege. A kivalasztott vagy kialakitott feladatok magjat a NEPSY®-I es a WISC-IV tesztek elmeleti es gyakorlati tapasztalatai adtak. Eredmenyeink kozul kiemelendő, hogy a vak csoport a teri feldolgozas teruleten nagymertekű elmaradast mutat a lato kontrollhoz kepest. A lato mintaban a koraszulott gyermekek a teri feladatok eseten rosszabbul teljesitenek időre szuletett tarsaikhoz kep...

A corpus callosum agenesia viselkedéses és kognitív profilja - összefoglaló

Background and purpose Agenesis of corpus callosum is a relatively frequent congenital cerebral malformation including dysplasia, total or partial absence of corpus callosum. The agenesis of corpus callosum can be occured in isolated form without accompanying somatic or central nervous system abnormalities and it can be associated with other central nervus system malformations. The behavioral and cognitive outcome is more favorable for patients with isolated agenesis of corpus callous than syndromic form of corpus callosum. The aim of this study is to review recent research on behavioral and social-cognitive functions in individuals with agenesis of corpus callosum. Developmental delay is common especially in higher-order cognitive and social functions. Methods An internet database search was performed to identify publications on the subject. Results Fifty-five publications in English corresponded to the criteria. These studies reported deficits in language, social cognition and emotions in individuals with agenesis of corpus callosum which is known as primary corpus callous syndrome. Conclusion The results indicate that individuals with agenesis of corpus callosum have deficiency in social-cognitive domain (recognition of emotions, weakness in paralinguistic aspects of language and mentalizing abilities). The impaired social cognition can be manifested in behavioral problems like autism and attention deficit hyperactivity disorder.

P248: Chorionic villus sampling: our experience

The authors describe experiences gained over the period of 1984 and 1999 at two medical centers with chorionic villus sampling (CVS). Altogether 1149 CVSs had been performed between the 10th and 32nd gestational week. Prior to 1993 the transcervical approach (TC-CVS), after 1994 the transabdominal method (TACVS) was used. Analysis of data collected within the framework of this study was based on the following factors: indications for sampling, complications and incidence of pregnancy loss. 91.6% of the CVSs was carried out for the purposes of cytogenetic examination of the fetus. Over the past few years an increasing number of procedures had been carried out for molecular-genetic tests (7.6% of the total number of cases). Although the primary indication for cytogenetic tests was the advanced age of the mother, a remarkable increase in the number of samplings had taken place for the purpose of examining ‘‘suspicious ultrasound findings’’, minor anomalies detected by ultrasound. In this group the proportion of pathological cases was significantly higher (14%) than in all the other samplings, carried out for other indications. This data in itself underlines the importance of ultrasound-screening performed in the 18–20th weeks of gestation. Over the first half of the period being reviewed (1984–1993, TC-CVS), a fetal loss of 4.8% occurring within three weeks from the date of sampling, dropped to 1.7% in the period subsequent to year 1994 (TA-CVS). In cases of TA-CVS, both the complications and spontaneous abortions were less. In 74.1% of the cases, birth had taken place after the 37th week of gestation. Premature births (6.4%) and still birth-rate (1.1%) did not exceed normal rates observed in the general population. On the basis of our results, it is safe to say that in prenatal diagnosis, TA-CVS is a real alternative method of mid-trimester amniocentesis and it is recommended for use at any stage of the pregnancy.

P066: Prenatal sonographic measurement of the fetal iliac angle in trisomy 21, 18 and 13

OBJECTIVE To determine whether iliac wing angle measurement in second trimester fetuses is a useful sonographic marker for the detection of trisomy 21, 18 and 13. METHODS During the period between September 1998 and September 2001, 406 fetal iliac angle measurements were performed in women in the second trimester of their pregnancies. The iliac angle measurements in fetuses with trisomy 21 (n = 25), trisomy 18 (n = 10) and trisomy 13 (n = 5) were compared with iliac angle measurement in fetuses with normal karyotypes (n = 333). RESULTS The mean iliac wing angle in the fetuses with trisomy 21 was 92.67 and 79.35 degrees and 74 degrees in fetuses with trisomy 18 and 13 (the mean iliac wing angle in the healthy fetuses was 70.09 degrees ). CONCLUSION The proven larger iliac wing angle in neonates with Downs syndrome can be demonstrated sonographically during the pregnancy, especially during the second trimester, and may be useful in prenatal screening of trisomy 21. The sonographic measurement of the fetal iliac angle cannot be used as a marker for trisomy 18 and 13. We have shown that fetuses with trisomy 18 and 13, on average, have iliac angles only a few degrees larger than healthy fetuses.