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Aim: Our aim was to contribute new findings related to the pre-regressional verbal development of females with a variant of Rett syndrome (RTT) as the loss of spoken language is one of the key clinical features of RTT, and it would be of particular interest to study the early speech-language development of females who are considered to have preserved some speech-language abilities., Method: We analysed 461 minutes of audio-video recordings containing play situations and the daily routines of six females (aged 7 to 24 months; mean birthweight 3057g, SD 195g) with the preserved speech variant (PSV) of RTT. All videos were recorded by parents and analysed retrospectively after the diagnosis PSV was made., Results: From the age of 7 months onwards, we observed two types of vocalizations, appearing intermittently: (1) apparently normal sequences; and (2) atypical (i.e. inhalatory, pressed, or high-pitched crying-like) vocalizations. Some participants failed to reach the milestone of canonical babbling. We observed a limited phonological and lexical complexity and a restricted compositional variability. Volubility was reduced during the whole period under observation. Hand stereotypies with simultaneous atypical vocalizations appeared only during the second year of life., Interpretation: The intermittent character of normal versus abnormal verbal behaviours might contribute to an early identification of children with a possible genetic mutation, and provides evidence that speech-language functions are abnormal from the very beginning., (© The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.)

To assess whether there are qualitatively deviant characteristics in the early vocalizations of children with Rett syndrome, we had 400 native Austrian-German speakers listen to audio recordings of vocalizations from typically developing girls and girls with Rett syndrome. The audio recordings were rated as (a) inconspicuous, (b) conspicuous or (c) not able to decide between (a) and (b). The results showed that participants were accurate in differentiating the vocalizations of typically developing children compared to children with Rett syndrome. However, the accuracy for rating verbal behaviors was dependent on the type of vocalization with greater accuracy for canonical babbling compared to cooing vocalizations. The results suggest a potential role for the use of rating child vocalizations for early detection of Rett syndrome. This is important because clinical criteria related to speech and language development remain important for early identification of Rett syndrome., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Objective: There is little systematic data on early neurodevelopmental functioning of infants with Smith-Magenis syndrome, since early diagnosis is rare., Methods: A boy with cytogenetically confirmed Smith-Magenis syndrome was videotaped at 4 months and 1 week of age. His posture and spontaneous movements were analysed without knowing the diagnosis., Results: The motor repertoire appeared significantly reduced; fidgety general movements, which are typical of that age, were missing. Posture was abnormal and overall movements were jerky and monotonous. The findings indicate a severe motor impairment by no more than 4 months of age., Conclusion: It was concluded that an absence of fidgety movements that goes along with subtle dysmorphic features indicates an increased risk of maldevelopment and justifies the need to refer for genetic evaluation with the potential of facilitating earlier diagnosis.

Aim: To determine the predictive value of the early motor repertoire for the level of self-mobility in children with cerebral palsy (CP) at school age., Method: Video recordings were made at 11 to 17 weeks post-term of 37 preterm infants (20 males, 17 females) who later developed CP. The early motor repertoire was assessed by obtaining a motor optimality score. At 6 to 12 years, children were classified according to the Gross Motor Function Classification System (GMFCS)., Results: Of 37 children (mean gestational age 29.1wks, SD 1.9; mean birthweight 1273g, SD 324), nine had unilateral and 28 had bilateral spastic CP. Twelve children were in GMFCS level I, three level II, 10 level III, four level IV, and eight level V. The absence of the age-adequate motor repertoire, a cramped motor repertoire, an abnormal kicking pattern, and a non-flat supine posture were associated with lower levels of self-mobility (chi(2) for trend test, p<0.05). Predictive for a low level of self-mobility was a cramped motor repertoire/non-flat supine posture (positive predictive values [PPV] 100%, negative predictive values [NPV] 54%). Predictive for a high level of self-mobility was a non-cramped repertoire/flat supine posture (PPV 80%, NPV 74%)., Interpretation: Several aspects of the motor repertoire at 11 to 17 weeks post-term predicted the degree of functional limitations in children with CP at school age.

The subject of the present study is the development of a girl with the preserved speech variant of Rett disorder. Our data are based on detailed retrospective and prospective video analyses. Despite achieving developmental milestones, movement quality was already abnormal during the girl's first half year of life. In addition, early hand stereotypies, idiosyncratic vocalizations, asymmetric eye opening, and abnormal facial expressions are early signs proving that this variant of the Rett complex, too, manifests itself within the first months of life.

Background: Qualitative aspects of the motor repertoire, at 11-16 weeks post-term are predictive for minor neurological dysfunction (MND) at 7 to 11 years of age. Predictive value of quantitative aspects is unknown so far., Aim: To investigate whether quantitative aspects of the motor repertoire between 6 and 24 weeks post-term also have predictive value for neurological outcome at 7 to 11 years of age., Study Design: Prospective cohort study., Subjects: Preterm infants from whom several quantitative aspects of the motor repertoire were assessed between 6 and 24 weeks post-term., Outcome Measures: Neurological outcome at 7-11 years of age was assessed according to Touwens' neurological examination. Children were classified as neurologically normal, or as having complex MND or cerebral palsy (CP)., Results: Eighty-two children were included. At 7 to 11 years of age 15 children (18%) had developed CP, 49 (60%) were neurologically normal, and 18 (22%) had MND. Multiple logistic regression analysis showed that, when the qualitative aspects of the motor repertoire known to predict neurological outcome were taken into account, only the asymmetric tonic neck (ATN) posture provided additional predictive value. In case of normal fidgety movements (FMs) accompanied by an abnormal concurrent motor repertoire, the presence of an obligatory ATN increased the risk for developing complex MND to 75%; absence of an obligatory ATN reduced the risk to 15% (p<0.05)., Conclusions: Quantitative aspects of the motor repertoire at 11-16 weeks post-term, in particular the presence of an obligatory ATN posture, contribute to the prediction of neurological outcome at 7 to 11 years of age.

The aim of the study was to examine the reaching behavior at the age of 5 months, and to determine whether and to what extent there is a relationship between hand use at this age and manual laterality at preschool age. 20 participants (13 girls and 7 boys) were investigated on two occasions: At the age of 5 months we assessed the hand use for reaching for four different objects placed at the infant's body midline or in their right or left hemispaces, respectively. At the age of 5 years and 7 months, we assessed the hand use for 22 motor tasks. The handedness status was calculated in order to reflect the sinistrality-dextrality continuum. All but one infant were unimanual reachers at 5 months of age. Preferential reaching was space dependent rather than object dependent at this stage. Children reaching for objects in the right hemispace predominantly with their right hand showed a significantly greater right hand laterality at late preschool age than children who at the age of 5 months had shown inconsistent hand use (p < .05). Children who at the age of 5 months had reached for objects in the left hemispace with their left hand proved to be less lateralized in their right hand use approximately 5 years later (p < .05)., ((c) 2008 Wiley Periodicals, Inc.)

Objective: The quality of a child's motor repertoire at age 3 to 4 months postterm is predictive of later cerebral palsy (CP). Its predictive power for minor neurologic dysfunction (MND) is unclear. This study aimed to investigate the predictive value of the quality of the early motor repertoire for the development of MND at school age., Study Design: We assessed the motor repertoire from video recordings made at 6 to 24 weeks postterm in 82 preterm infants (mean gestational age, 29.7 +/- 1.9 weeks; mean birth weight, 1183 +/- 302 g). At age 7 to 11 years, Touwen's neurologic examination was performed, and the children were classified as normal (n = 49; 60%), MND (n = 18; 22%), or CP (n = 15; 18%)., Results: Multiple logistic regression analysis showed that the quality of fidgety movements (FMs) and the quality of the concurrent motor repertoire had independent prognostic value for MND at school age. Abnormal FMs evolved into MND in 64% of the children. Nine of the 28 children with normal FMs and an abnormal concurrent motor repertoire developed abnormally (32%). Only 1 child of the 21 children with normal FMs and a normal concurrent motor repertoire developed MND (5%)., Conclusions: Assessment of the quality of the early motor repertoire can accurately identify individual infants at high and low risk for MND at school age.

General movements (GMs) are a distinct movement pattern carried out spontaneously without external stimulation and seen in fetuses of 9 weeks gestational age till 21 weeks postterm. GMs are helpful in the early diagnosis of an impaired central nervous system and the specific prediction of later neurological deficits. Autism spectrum disorder (ASD) is a neurodevelopmental disorder involving a life-long deficit in several aspects of the social and communicative behavior. Recently there appeared studies proving that children with ASD demonstrate disorders of motor development. To detect whether abnormalities in spontaneous motor activity can be observed already in the first months of life in infants with ASD. A retrospective study was performed by analyzing the family videos provided by parents of 20 children (male 17, female 3) later diagnosed as ASD. Home videos provided by parents of a control group of healthy children (n=20; male 10, female 10) matched for age with the ASD subjects and recorded in similar conditions were also analysed. In total 70 sequences were studied. Two independent observers, blind of the infants' outcome (ASD or normal), assessed the cases applying a global and a more detailed assessment of GMs. Hence, the age-specific GM pattern (normal or abnormal) as well as motor optimality scores were determined for each video sequence. Cohen kappa was 0.614. During the writhing movement period 70.0% sequences of infants with ASD showed poor repertoire GMs. In the control group, poor repertoire GMs were only seen in 12.5% of the sequences. In the fidgety movement period 20.8% of sequences were assessed as absent fidgety movements, 29.2% as abnormal fidgety movements. The large majority of the videos for the control cases were scored as normal (88.9%), 11.1% had no fidgety movements. According to the Mann-Whitney U test there were significant differences between the ASD and the control groups' optimality scores. The optimality scores were lower in the ASD group. The reduced optimality scores were mainly due to a lack of variable sequences, amplitude and speed of writhing GMs and an altered quality of fidgety and other spontaneous movements in the ASD group. Infants with ASD had more often poor repertoire writhing GMs as well as abnormal or absent fidgety movements than control infants. These data encourage further studies involving a larger number of family videos.

Background: The qualitative assessment of general movements (GMs) proved to be a highly sensitive and specific diagnostic tool for the assessment of the integrity of the young nervous system. It is essential that the quality of GMs remains consistent in an individual during a given recording at a certain date., Objectives: The aim of the study was to investigate the intra-individual consistency of the quality of GMs during one recording., Methods: 39 preterm infants were recorded at least twice; some were recorded three times. In all, 88 recordings were available but three recordings were excluded due to frequent crying, seizures or hypokinesia. Three scorers assessed 2-3 sequences of these 85 GM recordings., Results: The inter-scorer agreement was high (kappa 0.85-0.94). Intra-individual consistency revealed a kappa of 0.90 with a 95% CI (0.51, 1.00) for preterm GMs, 0.96 with a 95% CI (0.57, 1.00) for writhing GMs, and 0.92 with a 95% CI (0.53, 1.00) for fidgety GMs., Conclusions: The individual quality of GMs remains consistent for a neonate or young infant at a certain date., (Copyright (c) 2007 S. Karger AG, Basel.)

Background: Prechtl's method on the qualitative assessment of general movements (GMs) is a powerful tool for early and specific prediction of cerebral palsy. However, it is uncertain whether the GM assessment can be used to predict mild neurological impairment., Aims: To determine whether the quality of general movements (GMs) from the age of 3 to 5 months, i.e. fidgety movements, is related to the presence of complex minor neurological dysfunctions (MND) 13 to 15 years later., Study Design: Prospectively collected data on the quality of GMs during infancy were retrospectively analysed on the basis of MND at puberty., Subjects: Twenty-eight participants (14 girls and 14 boys) with a median gestational age of 40 weeks (range: 35 to 42 weeks) and an appropriate birth weight (median 3390 g; range 1900 to 4200 g)., Outcome Measures: Touwen's neurological examination., Results and Conclusions: Abnormal fidgety movements were not related to later complex MND, but to fine manipulative disabilities (p<0.05). Normal fidgety movements, which are continually present in the whole body, might be required for optimal calibration of the proprioceptive system.

The aim of the study was to describe a developmental trend of hand use for picking up and stacking blocks from the age of 18 months to 7 years. A second aim was to determine whether there is a relationship between right-hand use while building a tower and manual laterality at school age. A total of 28 children were asked to build a tower at five longitudinal assessments. The use of the right hand fluctuated considerably with age (p<.05), but no age-related increase was observed. Right-hand use was rather prominent at 18 months, decreased significantly at 26 months, increased at age 4;7, decreased again at 5;7, and finally increased again at age 7. From age 4;7 onwards, the percentage of right-hand use for stacking the blocks was significantly (p<.05) related to the laterality quotient at school age.

Background: Obstetrical and neonatal complications and/or an adverse parenting environment are risk factors for language impairment, but little is known about their effects on early word production (late talking)., Aims: To determine obstetrical and neonatal risk factors in children with delayed word production; to assess the influence of the parenting environment on word production; to determine whether the toddler's vocabulary competence is related to his/her social competence; to document the neurodevelopmental outcome at the late preschool age., Study Design: Prospective follow-up study. Based on word production at 18 months, children were retrospectively classified into consistent or transient late talkers and controls., Subjects: Full-term infants, N=30 (mean GA 40.3+/-1.1 weeks, mean birth weight 3577+/-267 g) were assessed at 18 and 24 months; and 5 years and 7 months., Methods: Austrian Communicative Development Inventories; Bayley Scales of Infant Development; Griffiths Developmental Scales., Outcome Measures: Peabody Picture Vocabulary Test; Kaufman Assessment Battery for Children; Touwen's neurological examination., Results and Conclusions: Lower Apgar scores and the need for neonatal intensive care were associated with delayed word production. The parents' educational and vocational levels were lower in late talkers. Early vocabulary competence correlated with social competence. With regard to the prediction of lexical and cognitive outcomes, late talkers did not differ from controls. Minor neurological dysfunctions (MND) were seen in nearly half of the late talkers and indicate the necessity to follow these children into school age, when MND might become more obvious and literacy can be assessed.

Background: The assessment of the quality of general movements (GMs) in young infants is a reliable and valid diagnostic tool for detecting brain dysfunction early in life. Poor repertoire GMs are the most frequently observed abnormal GMs during the preterm, term and early postterm period. However, their predictive value for the neurological outcome is low., Aim: To find out whether a detailed scoring of poor repertoire GMs might lead to a better prediction of the neurological outcome., Subjects: We studied 18 preterm infants who were repeatedly videoed from birth to 22 weeks postterm age, including several recordings assessed as poor repertoire GMs. At 8 to 10 years, six children were neurologically normal, six had mild neurological abnormalities, and the remaining six were classified as cerebral palsy., Study Design: Each GM globally assessed as poor repertoire was scored in details according to several aspects of neck and trunk, arm and leg movements applying Prechtl's optimality concept., Results: By and large, the detailed score of poor repertoire GMs was not related to the neurological outcome., Conclusion: For the clinical application of the GM assessment, it remains important to assess the fidgety movements of those infants with poor repertoire GM trajectories in order to predict their outcome.

An apparently normal early development was one of the initial criteria for classical Rett syndrome. However, several investigators considered Rett syndrome to be a developmental disorder manifesting very soon after birth. Videos of 14 infants with Rett disorder were carefully assessed for their spontaneous movements, in particular general movements (GMs), during the first 4 months of life. A detailed analysis clearly demonstrated that none of the infants had normal GMs. However, a specific abnormal GM pattern could not be detected for Rett disorder. The abnormal GMs described here, and their individual developmental trajectories are different from the abnormal GMs described in infants with acquired brain lesion. Our study is the first to apply specific standardised measures of early spontaneous movements to infants with Rett syndrome, proving conclusively that the disorder is manifest within the first weeks of life.

Background: Prechtl's method on the qualitative assessment of general movements (GMs) is a highly sensitive and specific diagnostic tool for the assessment of the integrity of the young nervous system., Aim: To find out whether the ability to assess GMs correctly could be gained after receiving a few days' standardised training., Study Design: We evaluated 700 scoring sheets (containing a total of 8019 assessments) from the final tests of 18 training courses held between 1997 and 2002., Results: Eighty-three per cent of the assessments were correct after completing a 4- to 5-day training course. The correct discrimination between normal and abnormal GMs was 92%. It proved more difficult to assess infants correctly if they had been recorded around term age., Conclusion: Standardised training courses enable professionals in the field of infant and child neurology to apply Prechtl's method of GM assessment accurately.

An apparently normal early development was one of the initial criteria for classical Rett syndrome. However, several investigators considered Rett syndrome to be a developmental disorder manifesting very soon after birth. Videos of 22 Rett cases were assessed carefully for movements, posture, and behavior during the first 6 mo of life. All signs that deviated from the normal standard were recorded meticulously. Special attention was paid to the face, the hands, and body movements. A detailed analysis clearly demonstrated an abnormal quality of general movements (100%), tongue protrusion (62%), postural stiffness (58%), asymmetric eye opening and closing (56%), abnormal finger movements (52%), hand stereotypies (42%), bursts of abnormal facial expressions (42%), bizarre smile (32%), tremor (28%), and stereotyped body movements (15%). Our study is the first to apply specific standardized measures of early spontaneous movements to Rett infants, proving conclusively that the disorder is manifested within the first months of life. Although not necessarily specific, the signs that we have observed will be of value in alerting clinicians to the possibility of the diagnosis at an early stage, when intervention is likely to be most effective.

General movements (GMs) are part of the spontaneous movement repertoire and are present from early fetal life onwards until the end of the first half a year of life. GMs are complex, occur frequently, and last long enough to be observed properly. They involve the whole body in a variable sequence of arm, leg, neck, and trunk movements. They wax and wane in intensity, force and speed, and they have a gradual beginning and end. Rotations along the axis of the limbs and slight changes in the direction of movements make them fluent and elegant and create the impression of complexity and variability. If the nervous system is impaired, GMs loose their complex and variable character and become monotonous and poor. Two specific abnormal GM patterns reliably predict later cerebral palsy: 1) a persistent pattern of cramped-synchronized GMs. The movements appear rigid and lack the normal smooth and fluent character. Limb and trunk muscles contract and relax almost simultaneously. 2) The absence of GMs of fidgety character. So-called fidgety movements are small movements of moderate speed with variable acceleration of neck, trunk, and limbs in all directions. Normally, they are the predominant movement pattern in an awake infant at 3 to 5 months. Beside a sensitivity and specificity of 95% each, the assessment of GMs is quick, noninvasive, even nonintrusive, and cost-effective compared with other techniques, e.g., magnetic resonance imaging, brain ultrasound, and traditional neurological examination.

Background: The assessment of the quality of general movements (GMs) in young infants is a reliable and valid diagnostic tool for detecting brain dysfunction early in life. Of special interest is a type of GMs called fidgety movements (FMs) characteristic for 3- to 5-month-old infants. GMs are part of an infant's spontaneous motor repertoire and as such endogenously generated by the nervous system. Visual, acoustic and social stimuli hardly had any influence on FMs., Aim: Our main purpose was to find out whether FMs are sensitive to load perturbation., Study Design: Spontaneous motility in supine position, with and without weighting was recorded on video and the data were semiquantitatively analysed. Weights were attached to the ankles and wrists of all four limbs; on one side of the body only; or without visual feedback of the weighted arm., Subjects: We studied 29 healthy infants with normal FMs at the age of 12 weeks., Results: Spontaneous motility remained symmetrical during all the experimental trails. Weighting had no influence on the quality or temporal organisation of FMs., Conclusion: This study demonstrated that the mechanisms responsible for FMs in 3-month-old infants are all but impervious to weight perturbation, at least not with the loads studied. FMs is the stable and predominant motor pattern of this age.

Objective: Sudden infant death syndrome (SIDS) remains a challenge for health professionals despite decreasing rates in recent years. The figures for different areas and time periods are hardly comparable, because of differences in postmortem investigations and classification criteria. In 1992, the European Society for the Study and Prevention of Infant Deaths (ESPID) proposed a classification for any sudden and unexpected death in infancy. This proposal has been used in our study since 1993 to better classify sudden infant death (SID) cases., Method: 56 consecutive SID cases observed between 1993 and 2002 in Styria, the south-eastern province of Austria, were analysed by a multidisciplinary team of health professionals. The study group consisted of pediatricians, forensic pathologists, pathologists, psychologists, nurses, members of the parents' association and health authorities. SID cases were analysed with regard to potential risk factors during pregnancy and early life, the circumstances of death (death scene) and post-mortem findings. From the latter, every SID was classified as either 1) classic SIDS, 2) borderline SIDS, 3) non-autopsied SID or 4) explained death., Results: Of the 56 SID cases, 22 were assigned to category 1, 19 to category 2, four to category 3, and in 11 cases death could be explained by major post-mortem findings. For 17/22 cases in category 1 and 11/19 cases in category 2, the death scene investigation showed the typical risk profile of manner of bedding and/or environmental conditions. In three cases, child abuse or infanticide was considered possible but could not be proven despite careful autopsy. In recent years, SIDS incidence in Styria has decreased to approximately 0.18/1,000 live-born infants, and the few deaths still occurring mainly present with the typical risk profile., Conclusion: An extensive analysis of SID events is a prerequisite for reliable and comparable SIDS statistics. Our data show that in several SID cases careful post-mortem examinations led to an explanation of death. In other cases, minor alterations may have contributed to the lethal event. These findings should therefore be considered in the classification of SIDs. The ESPID classification of 1992 appears to be very useful for this purpose and its use may therefore be recommended.

Hand-mouth contacts (HMCs) and other spontaneous movements of five low-risk preterm infants were studied longitudinally after their birth until 60 weeks postmenstrual age. For all infants, HMCs that emerged in the preterm period could not be observed transiently after 45 weeks, however, they re-emerged after 50 weeks postmenstrual age. In actograms of the infants' behaviors, the frequency of other spontaneous movements, such as head rotation, showed the same re-emerging pattern. Movements such as cloni, which were also observed in the preterm period, decreased after the term period, with no subsequent increase. Only general movements were continuously present throughout the entire observation period; these changed from writhing to fidgety in nature around the third month. These findings clarify which spontaneous movements of preterm infants are important for later behavioral development.

Background: Developmental testing in children is concerned mainly with a pass or fail on tasks such as grasping, manipulating and inserting. Knowledge about the qualitative development of hand movements in young children is scarce., Aim: We studied the qualitative development of manipulative hand movements in 14-, 18- and 25-month-olds., Study Design and Subjects: Twenty-six children were videoed during grasping, manipulating and inserting cubes into a cup or box and pegs into a peg-board., Results: We found clear-cut, object-related results. The right hand was used mainly to grasp, manipulate and insert the pegs, while no hand preference was found for the cube. Generally, the quality of grasping and inserting did not change with age. At 18 months, the performance was more optimal if the children used pegs rather than cubes. The adjustment of hand opening became more adapted with increasing age when grasping the cubes; this was not observed for the pegs. Associated movements occurred less during pegging than while playing with the cubes. A variety of patterns were used for pegging. Besides a difference in hand preference between girls and boys, some patterns became more prominent with increasing age. The success rate increased with age but was independent of the pattern used., Conclusion: This study stresses the need of a clear understanding of the entire spectrum of normal motor development. This knowledge forms the basis on which abnormalities, even of such common movement patterns as grasping, manipulating and inserting, can be reliably diagnosed in neurologically impaired infants and children.

Background: The assessment of the quality of general movements (GMs) in infants proves to be a reliable and valid diagnostic tool for detecting brain dysfunction early in life. Of special interest, particularly for the prediction of cerebral palsy, is the fidgety kind of GMs, the so-called fidgety movements (FMs) observable in 3- to 5-month-old infants. GMs are part of an infant's spontaneous motor repertoire and as such endogenously generated by the nervous system itself., Aim: The question was raised as to what extent the temporal organisation of FMs can be modulated by visual and acoustic stimulation., Study Design: Spontaneous motility in supine position with and without stimulation was recorded on video and the data were semiquantitatively analysed. We studied the effect of visual stimulation (red ring, red puppet), unanimated acoustic stimulation (68, 77, 88 dB) and of the mother, approaching her infant in a talkative manner after an absence of a quarter of an hour., Subjects: Twenty-nine healthy infants at the age of 12 weeks who all showed normal FMs., Results: Visual stimulation demonstrated that only the presentation of a red puppet elicited a significant level of focussed attention and led to a decrease of FMs. A red ring, unanimated acoustic stimulation as well as the interaction with the mother had no influence on the temporal organisation of FMs., Conclusion: This study demonstrated that in 3-month-old infants, FMs is a predominant motor pattern and that it is possible to assess FMs during (playful) social interaction.

Objective: To ascertain whether specific abnormalities (ie, cramped synchronized general movements [GMs]) can predict cerebral palsy and the severity of later motor impairment in preterm infants affected by brain lesions., Design: Traditional neurological examination was performed, and GMs were serially videotaped and blindly observed for 84 preterm infants with ultrasound abnormalities from birth until 56 to 60 weeks' postmenstrual age. The developmental course of GM abnormalities was compared with brain ultrasound findings alone and with findings from neurological examination, in relation to the patient's outcome at age 2 to 3 years., Results: Infants with consistent or predominant (33 cases) cramped synchronized GMs developed cerebral palsy. The earlier cramped synchronized GMs were observed, the worse was the neurological outcome. Transient cramped synchronized character GMs (8 cases) were followed by mild cerebral palsy (fidgety movements were absent) or normal development (fidgety movements were present). Consistently normal GMs (13 cases) and poor repertoire GMs (30 cases) either lead to normal outcomes (84%) or cerebral palsy with mild motor impairment (16%). Observation of GMs was 100% sensitive, and the specificity of the cramped synchronized GMs was 92.5% to 100% throughout the age range, which is much higher than the specificity of neurological examination., Conclusions: Consistent and predominant cramped synchronized GMs specifically predict cerebral palsy. The earlier this characteristic appears, the worse is the later impairment.

Background: The International Child Care Practices Study (ICCPS) has collected descriptive data from 21 centres in 17 countries. In this report, data are presented on the infant sleeping environment with the main focus being sudden infant death syndrome (SIDS) risk factors (bedsharing and infant using a pillow) and protective factors (infant sharing a room with adult) that are not yet well established in the literature., Methods: Using a standardised protocol, parents of infants were surveyed at birth by interview and at 3 months of age mainly by postal questionnaire. Centres were grouped according to geographic location. Also indicated was the level of SIDS awareness in the community, i.e. whether any campaigns or messages to "reduce the risks of SIDS" were available at the time of the survey., Results: Birth interview data were available for 5488 individual families and 4656 (85%) returned questionnaires at 3 months. Rates of bedsharing varied considerably (2-88%) and it appeared to be more common in the samples with a lower awareness of SIDS, but not necessarily a high SIDS rate. Countries with higher rates of bedsharing appeared to have a greater proportion of infants bedsharing for a longer duration (>5 h). Rates of room sharing varied (58-100%) with some of the lowest rates noted in centres with a higher awareness of SIDS. Rates of pillow use ranged from 4% to 95%., Conclusions: It is likely that methods of bedsharing differ cross-culturally, and although further details were sought on different bedsharing practices, it was not possible to build up a composite picture of "typical" bedsharing practices in these different communities. These data highlight interesting patterns in child care in these diverse populations. Although these results should not be used to imply that any particular child care practice either increases or decreases the risk of SIDS, these findings should help to inject caution into the process of developing SIDS prevention campaigns for non-Western cultures.

For a better understanding of the contribution vision makes to the development of other sensory systems and to movement and posture, we studied effects of early blindness by examining video recordings of 14 totally blind infants. Infants were born at term or preterm and showed no evidence of brain damage. During preterm and term periods no noticeable changes in motor activity were observed. Around 2 months postterm all infants showed clear delay in head control and abnormal, exaggerated type of 'fidgety movements'. Later, postural control was characterized by a prolonged period of ataxic features. Results indicate a lack of normal calibration exerted by vision on proprioceptive and vestibular systems. Early visuomotor coordination such as coordinated eye-head scanning and head orientating were present but disappeared after several weeks.

Unlabelled: The aim of the study was to document the early developmental course of neurological signs in a group of preterm infants at risk for hemiplegia due to unilateral intraparenchymal echodensity (UIPE). Sixteen preterm infants with UIPE and sixteen controls were given serial neurological examinations, according to the protocols currently adopted in the different NICUs of the project. Moreover, the quality assessment of their general movements (GMs) was assessed subsequently from videotapes, from birth until around four months postterm. At two years, 12 of the UIPE infants showed hemiplegia and one suffered from asymmetrical diplegia. The findings of the traditional neurological examination were abnormal for the large majority of the UIPE infants, although normal findings were also recorded in some cases, especially during the preterm period. Asymmetries were found after term age in nine UIPE and in two control infants. From the first observation onwards, all infants with UIPE showed bilaterally abnormal GMs and in those with unfavourable outcome fidgety movements (FMs) were absent. At the FMs period (9-16 weeks postterm), all infants with subsequent hemiplegia showed asymmetry of distal segmental movements which were reduced or absent on the side contralateral to the lesion., Conclusions: Unilateral brain lesions induce clear neurological signs and abnormal GMs in particular, although these abnormalities are not initially asymmetrical. A reduction of segmental movements on one side of the body during the third month postterm is highly predictive of hemiplegia.

The possibility to assess the functional condition of the fetal nervous system is of great importance to the obstetrician, since a considerable part of early brain damage is of prenatal origin. Several attempts to develop such a technique are reviewed. In addition, a new method, the qualitative assessment of fetal general movements, is described as a successful tool to obtain reliable data on the fetal brain dysfunction. This new method is robust, non-intrusive and cost-effective. There is also the advantage that the same criteria for the diagnostic assessment can be used for the fetus as well as for the young infant.

The qualitative assessment of general movements (GMs) has been shown to be a better predictor of neurological outcome than the traditional neurological examination in brain-damaged preterm infants. The aim of this study was to compare the results of the two techniques in term infants. Off-line assessment of GMs from videorecordings and neurological examinations were carried out, from birth till about 6 months of postterm age, in a group of 58 term infants, the majority of which were affected by mild to severe hypoxic-ischaemic encephalopathy. The agreement between the two techniques and their predictive power, with respect to the neurological outcome at 2 years, were evaluated for four age groups. The range of agreement between neurological and GM findings was between 78 and 83%. At all ages the results of GM observation correlated highly with the neurological outcome; their sensitivity and specificity with respect to outcome were consistently slightly superior to those of neurological examination. In infants normalize after an initial period of transient abnormalities, GMs normalize earlier than the neurological results.

Background: In normal awake infants, fidgety movements are seen from the age of 6 weeks to 20 weeks. The aim of the study was to test the predictive value of absent or abnormal spontaneous movements in young infants for the later development of neurological deficits., Methods: In a collaborative study involving five hospitals we collected data on the normal and abnormal quality of fidgety movements of 130 infants and compared it with assessments of neurological development done longitudinally until the age of 2 years. On the basis of ultrasound scans infants were classified as at low-risk or at high-risk of neurological deficits. Infants were videoed for 1 h every week from birth to discharge and then for 15 min every 3 to 4 weeks; quality of general movements was assessed. Repeated neurological assessments were also done until 24 months of corrected age., Findings: 67 (96%) of 70 infants with normal fidgety movements had a normal neurological outcome. Abnormal quality or total absence of fidgety movements was followed by neurological abnormalities in 57 (95%) of the 60 infants (49 had cerebral palsy and eight had developmental retardation or minor neurological signs). Specificity and sensitivity of fidgety movement assessment were higher (96% and 95%, respectively) than of ultrasound imaging of the infants' brain (83% and 80%, respectively)., Interpretation: Our technique of assessing spontaneous motor activity can identify and distinguish between those infants who require early intervention for neurological abnormalities and those who do not. Our technique is simple, non-intrusive, reliable, quick, and can be done on very young infants.

Objective: The Prechtl method of qualitative assessment of general movements (GMs) has been shown to be a good predictor of neurologic outcome in fetuses, preterm infants, and term infants. The aim of this study was to compare the results of this new technique with those of traditional neurologic examination and of cranial ultrasonography in preterm infants., Methods: Serial videotape recordings (with off-line assessment of GMs), ultrasound examination of the brain, and neurologic examinations were performed from birth until about 6 months of corrected age, on a group of 66 preterm infants whose gestational age ranged from 26 to 36 weeks (mean 30.7 weeks). The agreement between the two techniques and their predictive power, with respect to the neurologic outcome at 2 years of corrected age, were evaluated for five different age groups from preterm age to 65 weeks of postmenstrual age., Results: Overall agreement of the neurologic and GM findings was 80.3% and strongly age related (lower during the preterm and term periods and higher thereafter). At all ages the results of GM observation correlated highly with neurologic outcome; they showed higher sensitivity and specificity than the neurologic examination. This held true in particular before term age, when poor neurologic responses might be related to transient complications, and at term age, mainly because of infants with normal neurologic examination results but unfavorable outcome. During the preterm period the ultrasound results showed a better specificity and a lower sensitivity to outcome than GM findings., Conclusions: The results of this study indicate that quality assessment of GMs should be added to traditional neurologic assessment, neuroimaging, and other tests of preterm infants for diagnostic and prognostic purposes.

Fourteen infants of 2 months or 6 months of age were video-recorded during polysomnography. Four were normal infants, five had a history of ALTE (apparent life threatening event) and five had repeated and prolonged apnoea during sleep. Two ALTE infants have been recorded at 2 months as well as at 6 months of age. Movements during sleep could be classified into general movements, isolated movements of the upper extremity, startles, head rotations, and trunk rotations. In the ALTE cases at 2 months of age, the motility was quantitatively not different from the control infants but was markedly reduced at 6 months of age. (All cases had their event before 8 weeks of age.) In contrast to these findings, infants with repeated apnoea did not show a clear change in the quantity of their movements. With the exception of one ALTE case at 2 months, all observed cases of ALTE and apnoeic infants showed an abnormal quality of their spontaneous movements during sleep. As reported in a previous study, all these cases had also been found moving abnormally during wakefulness. It is suggested that the abnormal motility is a sequelae of the event (ALTE or repeated apnoeas) with as a consequence, an impairment of neural functions.

89 of 119 parents of infants with apnea home monitoring answered to a standard questionnaire concerning different aspects of home monitoring. In almost all cases the home monitoring was finished at the begin of the study, lasting from six months to 12 months. The indications for an apnea monitor were: apparent life threatening event (n = 8), SIDS-sibling (n = 24), sleep apnea syndrome (n = 40) and parents pressing request (n = 17). 90% of parents felt to have got enough information about the function of the apnea monitor (mostly Graseby MR-10 monitor) and what they have to do when there is an apnea alarm. 6% of parents changed the monitor because of technical problems. Most of the alarms were registrated by almost all parents between 24 h and 6 h. 35% of parents stimulated their infants in case of an apnea alarm gently to vigorously, one infant had a mouth to mouth resuscitation. Questions concerning the psychological and social impact of home monitoring on parents demonstrated that there was some stress like anxiety, sleep disturbances mainly in the mothers. We conclude from parents experience with apnea home monitoring the need for an adequate medical, technical and psychological support.

In a prospective study (1988-1990) the SIDS risk questionnaire SRFB was applied to 6000 infants born in the Department of Obstetrics, University Hospital Graz. In all infants at an increased statistical risk for SIDS a standardized intervention including pediatric and polysomnographic investigations was performed. As a result the SIDS incidence decreased from an average of 1.92 per thousand lifeborns (1984-1988) to 0.83 per thousand lifeborns (1989-1990) in our test group, which means a striking reduction of the SIDS incidence by 57%. In addition to this effect, the intensified information of parents and physicians about possible signs of an increased risk for SIDS lead to a general reduction of SIDS in Styria.

Based on the results from semistructured interviews with parents of fifty SIDS victims and of fifty matched controls we developed a SIDS risk questionnaire, the so-called SRFB Graz. In a retrospective study this questionnaire was applied to 65 SIDS victims and 195 comparable controls. By statistical analysis a cut point was computed, which discriminates SIDS cases from controls with a sensitivity of 86% and a specificity of 95%.

Because of the growing demand to evaluate cardiorespiratory dysfunctions the short-time oxycardiorespirography becomes a more and more important and powerful tool in the daily clinical routine. Therefore, from about one hour lasting records we studied the distribution of MA-values (mean apnea duration), duration of the longest apnea, percentage of periodic breathing time, lowest heart rate values, lowest transcutaneous PO2 values and highest transcutaneous PCO2 values in an obstetrical optimal low-risk group of 90 infants and a obstetrical non-optimal group of 152 infants between four and ten weeks of age. Percentiles of each variable were calculated, and we suggest the 90th percentiles as new threshold values to judge these short-time recordings. In comparison to relevant data from the literature we could demonstrate that deviating threshold values have to be applied to short-time recordings in comparison to long-time recordings. In addition, the respiratory behaviour of the optimal group is compared to that of the non-optimal group of the same age. Interestingly enough, no difference in the respiratory behaviour between these groups could be demonstrated.

A study was carried out to investigate the correlation between sleep apnea frequency, blood oxygenation and neurological condition in 21 infants at six weeks of age with inconspicuous medical history. Polygraphic recordings of respiratory behaviour and transcutaneous blood gas monitoring lasted for at least five hours. To quantify the results of the neurological examination we established a neurological optimality score (NOS). We found statistically significant correlations between several indexes of apneas and indexes of blood oxygenation and NOS. Thus, a reduced NOS was strongly correlated with higher apnea frequencies and with pronounced drops of transcutaneous PO2-values. In addition all but one infant who were diagnosed as having a sleep apnea syndrome showed a greater than ten percent reduction of NOS. Some pathophysiological considerations to explain our findings are put forward and the possible role of the neuromodulator adenosine is emphasized.

We correlated the incidence of the sudden infant death syndrome (SIDS) and the degree of air pollution in each district of Graz over a time period of five years. The degree of air pollution was derived from a mapping of epiphytical lichen vegetation by Grill et al. (1988). We found an increased incidence of SIDS in districts with poor air quality. Although this result is not statistically significant a possible increase in SIDS risk because of air pollution cannot be excluded. Some pathophysiological explanations are discussed.

A computerized, PC-based system is introduced for sleep monitoring in infants. The system is capable of digitizing and storing 22 physiological variables over a whole-night sleep period. The preprocessed data can be displayed on a single page; further processing can be done on the digitally stored data.

The question was posed, whether a relationship could be established between prenatal and perinatal events and subsequent sudden infant death. The birth records of eighty mothers, whose infants died of sudden infant death syndrome (SIDS) between 1982 and 1987, were reviewed for obstetric complications. The records immediately following the respective SIDS cases served as controls. A family history of SIDS was found significantly more often in the SIDS group than in the control group (p less than 0.001). Oxytocin was administered significantly more often in the SIDS group than in the control group: 54/80 (= 68%) and 20/80 (= 25%, p less than 0.001), respectively. Smoking, hypotension requiring treatment and the administration of tocolytic agents were found significantly more frequently in the SIDS group than in the control group (p less than 0.01, p less than 0.001, and p less than 0.05). Transient fetal hypoxia, as a result of reduced flow in the uterine artery, seems possibly to be connected to the vasoactive agent Oxytocin when occurring in the three above named groups. Whether these situations are connected to a later development of SIDS, has not, to date, been confirmed.

The parents of 80 victims of SIDS (Sudden Infant Death Syndrome) participated in this retrospective study. The semistructured interview included questions concerning events preceding infants' death and the situation at the discovery of the death. Fifty-eight of the 80 SIDS victims were male, twenty-two were female. Fourty-four of the babies underwent a postmortem examination. There was a high incidence of death during the winter months and concerning the hour of the day in the morning hours. Most families (91%) had no socioeconomic difficulties--in contrast to reports of other authors. In 34% of the SIDS cases the parents reported about stress or irregularities of the daily family life cycle (journey, mayor family events, quarrels) immediately before the death of the baby. About 20% of the victims were not in the usual surrounding at the time of death. In about 30% of the SIDS victims an infection was observed. Since between the 2nd and 4th month of life a major neuronal reorganization in the central nervous system is assumed (Prechtl, 1984), stress during this period may be particularly effective and dangerous for the baby. This would explain the high incidence of SIDS during this vulnerable phase (60%) in which the baby, apparently, is unable to react properly.