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Myositis ossificans is characterized by heterotopic bone formation in skeletal muscle that may present as a tender, warm, palpable swelling, and mimicking soft tissue infection. We present the clinical and imaging evolution of the disease with a favorable outcome in a preterm baby with complicated postnatal course.

Aim To find early predictors for poor neurodevelopmental outcome after neonatal group B streptococcal meningitis. Methods We retrospectively analyzed clinical characteristics of 23 patients with neonatal group B streptococcal meningitis and their neurodevelopmental outcome at 18 months. Available group B Streptococcus strains were serotyped and their genomes characterized. Results We found several differences between patients with early- (n = 5) and late-onset (n = 18) disease. Nine children had neurologic abnormalities at 18 months and 4 had epilepsy, all of them after late-onset disease. Most important risk factors for poor outcome were impaired consciousness at admission, hemodynamic instability, seizures, or abnormal electroencephalogram during the acute illness and abnormal neurologic and ophthalmologic examination at the end of treatment, whereas abnormalities in laboratory and imaging studies were not predictive. Hypervirulent serotype III, multilocus sequence type 17 group B Streptococcus was the predominant pathogen. Conclusions Neurodevelopmental impairment after neonatal group B streptococcal meningitis is likelier in those with clinical and neurophysiological features indicating worse disease severity.

Carboxyhemoglobin (COHb) is an index of endogenous carbon monoxide formation during the hem degradation process and could be used to confirm hemolysis in neonates. The influence of other clinical factors on COHb values in neonates has not been fully investigated. We aimed to evaluate the influence of hemolysis, sepsis, respiratory distress, and postnatal age on COHb values. We retrospectively analyzed COHb measurements determined with a carbon monoxide-oximeter in 4 groups of term neonates: A-sepsis, B-respiratory distress, C-hemolysis, and D-healthy neonates. The mean COHb values were 1.41% (SD: 0.26), 1.32% (SD: 0.27), 2.5% (SD: 0.69), and 1.27% (SD: 0.19) (P

IntroductionPrediction of outcome in newborns with hypoxic-ischemic encephalopathy (HIE) has been modulated by hypothermia treatment (HT). We assessed the predictive value of diagnostic methods commonly used in neonates with HIE for short-term neurodevelopmental outcome and long-term neurological outcome.Materials and MethodsThis longitudinal cohort study followed up 50 term newborns who underwent HT after HIE between July 2006 and August 2015, until preschool age. We estimated sensitivity and specificity for short-term neurodevelopmental outcome at 18 months and long-term neurological outcome at five years based on Amiel-Tison Neurological Assessment (ATNA), electroencephalography (EEG), and magnetic resonance imaging (MRI) performed in the neonatal period.ResultsThe accuracy of all neonatal methods tested was higher for long-term neurological outcome compared to the predictive accuracy for short-term neurodevelopmental outcome at 18–24 months. Sensitivity and specificity in predicting unfavorable long-term neurological outcome were: MRI (sensitivity 1.0 [95%CI 0.96–1.0]; specificity 0.91 [95%CI 0.86–1.0]), EEG (sensitivity 0.94 [95%CI 0.71–1.0]; specificity 1.0 [95% CI 0.89–1.0]), and ATNA (sensitivity 0.94 [95%CI 0.71–1.0]; specificity 0.91 [95%CI 0.76–0.98]).ConclusionMRI is a powerful predictor of long-term neurological outcome when performed in the first week after HIE in HT treated infants, as are EEG and ATNA performed in the second or third week postnatally.

Objective - Small for gestational age (SGA) infants have an increased risk of developing metabolic syndrome (MS) later in life. The aim of our study was to analyze some of the potential MS biomarkers in SGA neonates and to find the most indicative neonatal biomarker associated with MS in SGA. Materials and Methods - Serum adiponectin, fasting glucose, insulin, total cholesterol, high density lipoprotein (HDL) cholesterol, low density lipoprotein (LDL) cholesterol, and triglycerides were evaluated in 43 small (SGA) and 25 appropriate for gestational age (AGA) neonates in the first month of life. Glucose/insulin (G/I) ratio and the homeostasis model assessment of insulin resistance index (HOMA-IR) were calculated. Results - Mean serum adiponectin in SGA was significantly lower than in AGA neonates (36.6 vs. 42.6 mg/L, P=0.021). In SGA infants a statistically insignificant higher fasting insulin levels (9.8 vs. 6.4 mIU/L), lower G/I ratio (1.9 vs. 3.0 mol/IU) and higher HOMA-IR (2.6 vs. 1.4) was observed. The mean serum triglyceride level in SGA neonates was significantly higher than in AGA neonates (1.7 vs. 1.4 mmol/L, P=0.031). Conclusion - SGA neonates demonstrated significantly reduced serum adiponectin and significantly increased serum triglyceride levels in comparison to AGA infants in the first month of life. Triglycerides appeared to be the most promising biomarker reflecting metabolic tendencies in SGA newborns and could possibly be used in predicting the future development of MS.

Objective - Bacterial infection of the scrotal appendages (scrotal pyocele) is a rare condition, especially in neonates. It is easy to confuse with other scrotal emergencies that require surgical intervention and orchiectomy. We present a case where pre- and intraoperative diagnosis was not clear and orchiectomy was performed; additionally, review of the literature, diagnostics, treatment options and lessons learned are discussed. Case report - A ten-day old full term neonate, born with bilateral hydroceles and glandular hypospadias, presented with a six day history of poor feeding and irritability; clinical examination revealed an enlarged, erythematous and tender right hemiscotum. Blood cultures, routine laboratory examinations and ultrasound (US) imaging was performed. Postnatal torsion with non-viable testicle, testicular malignancy and epididymo-orchitis were considered in the differential diagnosis. Upon surgical exploration, the testicle appeared non-viable due to severe inflammation and was removed. The patient was treated initially with ampicillin and gentamicin. After Streptococcus pneumoniae was cultured from specimens, treatment was continued with benzyl penicillin. Conclusion - Neonatal scrotal pyocele is an extremely rare condition. The main suggestive features are clinical presentation with signs of infection and an enlarged painful scrotum. US is the investigation of choice to differentiate from a surgical emergency. Free fluid in the scrotal cavity with septae, normal looking testicle on US and no signs of torsion at exploration are diagnostic signs. If diagnosed early, the first line of management is surgical aspiration or decompression, followed by broad-spectrum antibiotic treatment. With delay in diagnosis, the testicle may look non-viable and push surgeons for orchiectomy.

From the very beginning of life, biological events in the intrauterine environment influence the developing child, its growth, maturation and adaptation. The aim of this study was to assess the impact of maternal vitamin D and adiponectin status on offspring growth, general and bone health.162 healthy pregnant women were included in the study, with their vitaminD and adiponectin levels measured in the 32 nd week of pregnancy. Body weight and bone mineral density measurements of their offspring were performed at birth and at the age of three, six, nine and twelve months. Information on children's infectious, allergic and chronic disease was collected from their medical records.Vitamin D insufficiency/deficiency was present in 44% of pregnant women. There was no significant association between maternal vitamin D during pregnancy and offspring body weight at birth or later, as well as between maternal vitamin D and newborn bone mineral density. Additionally, there was no significant association between maternal vitamin D and infectious, allergic or other chronic diseases in offspring. A negative correlation between maternal adiponectin and offspring's body weight at birth was observed (r = - 0.37, p = 0.002), while association with bone mineral density in newborns was not significant.Despite the significant prevalence of vitamin D insufficiency among pregnant women, it did not influence growth or health of their offspring in this study. Maternal adiponectin levels showed an inverse relationship with birth weight of the infants, which may highlight the important link between maternal health and the offspring's growth.

We commonly use chloral hydrate sedation in newborns, though its cardiorespiratory side effects have not yet been fully investigated. Our study aimed to analyze the impact of chloral hydrate on cardiorespiratory parameters in term newborns. We performed a prospective, pre-post single-arm interventional study in 42 term, respiratorily and hemodynamically stable newborns. Oxygen saturation (SpO2), end-tidal CO2 (ETCO2), the apnea-hypopnea index and the respiratory and heart rates were recorded by polygraphy, starting 0.5-1 hour before oral administration of chloral hydrate at a dose of 40 mg/kg and ceasing 4 hours post-administration. After administration of chloral hydrate, the mean basal SpO2 dropped by 2.0% (from 97.1% to 95.1%; p < 0.001) and the mean basal ETCO2 increased by 3.9 mmHg (25.6 to 29.5 mmHg; p < 0.001). We found a significant decrease in the minimal SpO2 values (p < 0.001) and an increase in the percentage of time spent with SpO2 < 95% and < 90% (p < 0.001). The mean increase in the estimated apnea-hypopnea index was 3.5 events per hour (p < 0.001). The mean respiratory and heart rates were significantly lower 150 min after the administration of chloral hydrate when compared with pre-sedation values (51/min and 127/min versus 61/min and 138/min respectively; p < 0.001). A considerable number of patients exhibited changes in cardiorespiratory parameters that differed considerably from the normal ranges. In conclusion, SpO2, ETCO2, the estimated apnea-hypopnea index and the respiratory and heart rates changed after the administration of chloral hydrate. They remained within normal limits in most newborns, but the inter-individual variability was high in the studied population.

Background The quality of general movements (GMs) has proven to have predictive value for the developmental outcome, but this has not yet been studied in twins. Aims Our aim was to analyse the quality of GMs and neurological and developmental outcome in relation to the gestational age (GA), mode of conception and other perinatal risk factors in a group of twins. Study design The documentation of twins referred for follow-up in the period from 1998 to 2016 was studied retrospectively. Data concerning the quality of GMs, perinatal risk factors and developmental outcome were analysed. Subjects Eighty-nine twin pairs, GA from 24 to 38 weeks (median 35.0; IQR 3), birth weight 670 g–3820 g (median 2323; IQR 645) were included. Outcome measures Results of neurological, psychological and speech/language development and school outcome were analysed. Results GMs at term age and at three to four months postterm age did not differ with regard to the mode of conception. Preterm birth was significantly related to GMs at three to four months postterm age. At term age, GMs were significantly related to neurological outcome, while at three to four months postterm age, GMs were related to both the neurological and psychological outcome. Difficulties in speech/language development were diagnosed in almost half of the children, more frequently in boys and children with lower Apgar scores. Conclusion The study highlights the value of GMs for predicting the developmental outcome in twins and indicates the importance of developmental, especially speech/language, follow-up in twins.

The focus of this paper is to review the data on the classic and nonclassic role of VD with regards to pregnancy and the newborn period. Over the past decade, new evidence has shown that vitamin D (VD) deficiency, in addition to its classical role in calcium metabolism and bone homeostasis, may contribute to the risk of developing a wide range of chronic diseases. VD may produce a wide array of favourable biological effects via genomic, non-genomic or intracrine mechanisms, and therefore contributes to the improvement of human health. Some of these effects may be even more important during pregnancy. Data from animal and human studies implicate maternal VD deficiency as a significant risk factor for several adverse outcomes affecting maternal, foetal and child health. In the newborn period, these comprise bone health, growth and immune response. Conclusion − Recent evidence supports the fact that low maternal vitamin D status is associated with an increased risk of adverse pregnancy outcomes. This paper investigates the effects of vitamin D on the placento-foetal unit and the mother, in terms of calcium metabolism and non-calcium effects.

Background The aim of the study was to explore the correlation between clinical signs and confirmatory tests for cow's milk allergy (CMA) in the neonatal period and their relation to family history and the occurrence of food allergies in the postneonatal period. Methods The medical documentation of 361 newborns with suspected CMA and exclusion of other comorbidities was analyzed. The correlations between clinical signs and methods to confirm CMA [elevated levels of total immunoglobulin E (IgE) and/or specific IgE for cow's milk, improvement after the introduction of a cow's milk-free diet and positive challenge procedure] were studied. In 90 children, the data were additionally analyzed in relation to outcome (at the age of 1–11 years), evaluated by questionnaires, which inquired about signs and symptoms of food allergy, methods of CMA confirmation, and the presence of other food allergies. Results There was a positive correlation between exanthema and confirmed CMA in the neonatal period ( R = 0.184; p = n = 361), and hematochezia and confirmed CMA in the neonatal ( R = 0.203; p n = 361) and postneonatal period ( R = 0.215; p = 0.042; n = 90). Additional food allergies in the postneonatal period were positively correlated with neonatal CMA ( R = 0.275; p = 0.009; n = 90). No correlation was found between a positive family history of food allergies and CMA in the neonatal ( R = −0.66; p = 0.398; n = 165) and postneonatal periods ( R = 0.00; p = 1.000; n = 116). Conclusion Neonatal exanthema and hematochezia were the predominant clinical signs in neonates with CMA. Allergies to other food allergens appeared more frequently in children with CMA in the neonatal period. Neonatal CMA did not occur more frequently in families with food allergies.

Neonatal cyanosis is rarely due to hemoglobin variants with low oxygen affinity. We describe the clinical course and results of molecular genetic analysis of a boy who presented after birth with severe cyanosis. Arterial blood-gas analysis demonstrated a pronounced shift of the oxygen-hemoglobin dissociation curve to the right and molecular genetic analysis revealed a γ-globin variant, Hb F-Sarajevo. The patient presented is the second reported case of neonatal cyanosis due to this mutation, which was first described in 2012 by Zimmermann-Baer and coauthors. With the introduction of universal screening for congenital heart disease, the finding of low oxygen saturation will uncover more neonates with hemoglobinopathies with low oxygen affinity.

O bjective – To describe the clinical course of enteroviral meningitis in neonates in a tertiary neonatal unit. M aterials and M ethods – We analysed maternal and perinatal history, clinical and laboratory data, therapy and short-term outcomes in neonates who had enterovirus detected in the cerebrospinal fluid (CSF) and compared the group of neonates with early-onset (≤7 day of life (DOL)) meningitis to the group of neonates with late-onset (>7 DOL) meningitis. R esults – A total of 30 term neonates were included (63% male). Majority (73%) presented during the summer and autumn months, 57% were in contact with siblings who had signs of viral disease, 10% of mothers had signs of viral disease at delivery or shortly after. Neonates presented with irritability (97%), fever (83%), feeding intolerance (66%), diarrhoea (56%), nonspecific rash (23%), seizures (10%). Both the C-reactive protein (CRP) and pro-calcitonin (PCT) were elevated in 23% of patients; only CRP was elevated in 57%, only PCT in 37%. Twelve (40%) had elevated leukocyte count in the CSF. Antibiotics were initiated in 80% (median treatment 1.5 days). Majority of the neonates with early-onset meningitis had jaundice, higher urea and creatinine serum concentration, and lower thrombocyte numbers. Conclusion – Enteroviruses are an important cause of meningitis in neonates during summer and autumn months. Neonates in close contact with ill family members or carers are particularly at risk. Neonates present with fever, irritability, feeding intolerance, diarrhoea, normal or mildly elevated CRP and/or PCT values. Neonates with early-onset meningitis had a similar grade of infection and outcome as did the neonates with late-onset disease. Our findings confirm that enteroviral meningitis car- ries a good prognosis in the majority of neonates.

Objective: Because of the restraints on conducting studies on pharmaceutical use in sick newborns, many drugs are used off-label in this population. Moreover, industrially manufactured pharmaceuticals may contain different excipients, which may be either untested or not licensed for use in neonates. The aim of our study was to determine the prevalence and pattern of pharmaceutical and excipient exposure in newborns hospitalized at the Department of Neonatology, Ljubljana, Slovenia. Methods: A longitudinal prospective cross-sectional study was performed during a one-month period and included all hospitalized neonates. Route of administration, site of action, type of manufacture, licensing status, type and concentrations of excipients for all pharmaceuticals given to the neonates were determined. Results: Twenty seven different pharmaceutical preparations were prescribed to a total of 48 hospitalized newborns. In most cases, newborns were prescribed various pharmaceuticals that were not approved for use in this population. Newborns were exposed to 60 different excipients in industrially manufactured pharmaceutical preparations. More than half of the received pharmaceuticals contained potentially harmful and harmful excipients. Conclusions: Two-thirds of pharmaceutical preparations for neonates were used off-label. Newborns receive more auxiliary substances, which may be unsuitable for this age group and may even be toxic to them, via industrially manufactured pharmaceuticals.

Background Neonatal seizures may cause irreversible changes to the immature brain and. A scoring system for early prognostic information could be a useful clinical tool. The aim of the study was to analyze risk factors for epilepsy after neonatal seizures, to validate Garfinkle's scoring system, and to analyze whether a new scoring system is feasible. Methods A retrospective study of 176 newborns (59.1% boys, 40.9% girls, 70.5% term, 29.5% preterm; mean birth weight 2820 g), admitted to the Department of Neonatology, Division of Pediatrics, University Medical Centre, Ljubljana, because of neonatal seizures (clinical and/or neurophysiological), was performed. Epilepsy rate between 2 and 12 years of follow-up was 18.1%. Five independent predictors from Garfinkle's study and other known predictors were entered into hierarchical binary logistic regression models and analyzed through four steps to identify independent predictors of epilepsy. We tested whether any of the predictors was an effect modifier. Results Of five potential predictors from Garfinkle's score, electroencephalograph background findings and etiology were predictive. Etiologies, gestation, mode of delivery, duration of seizures, and other risk factors at birth were found to be independent predictors. Duration of seizures has a different effect on prognosis depending on the gestational age. Conclusion Gestational age determines the association between duration of seizures and epilepsy. Scoring systems to predict development of epilepsy after neonatal seizures need to limit interaction between important predictor variables.

AIM: To report on the neurological presentation and neuroimaging findings in newborn infants with incontinentia pigmenti. METHOD: The clinical and neurological course including neuroimaging and follow-up data of eight newborn infants with the neurological phenotype of incontinentia pigmenti were retrospectively reviewed. RESULTS: While the clinical picture was polymorphic, the neurological manifestations were defined as encephalopathic and comprised lethargy and seizures in all but one of the infants. Magnetic resonance imaging (MRI) abnormalities were predominantly in the white matter. Diffusion-weighted imaging (DWI) was obtained during the acute phase in seven of the eight infants, showing restricted diffusion in the deep and subcortical white matter but also in the corpus callosum, basal ganglia, thalami, cerebellum, and cerebral peduncles. Susceptibility-weighted imaging (SWI), performed in five infants, showed a variable amount of signal loss, mainly in the white matter, within areas of restricted diffusion. Extensive MRI abnormalities in newborn infants were followed by abnormal neurodevelopment, with significant motor, cognitive, and/or visual problems. INTERPRETATION: To assess the extent of central nervous system involvement, MRI is recommended in the clinical evaluation of infants with incontinentia pigmenti. They have a characteristic pattern of brain lesions seen on MRI, best recognized using DWI and SWI in the acute neonatal phase, which allow the identification of and distinction between ischaemic and haemorrhagic lesions.

The basic principle of umbilical cord (UC) care is to keep it clean and dry, as this provides the fastest and safest UC healing.To evaluate Slovenian UC care practice and compare its consistency with current international recommendations.A questionnaire covering UC care from birth to complete healing of the umbilical wound was sent to all Slovenian primary-, secondary- and tertiary-level pediatric centers. Three different clinical conditions of the newborn's umbilicus were defined: healthy umbilicus (HU), umbilicus at risk (UR) and unhealthy umbilicus (UU).The study revealed a correlation between the clinical condition of the umbilicus, the frequency of UC care and the antiseptic usage in both the outpatient and inpatient UC care groups. HU was treated less frequently than UR and significantly less than UU. In both groups, these two conditions were also indications for UC care with antiseptic. The frequency of antiseptic usage for HU care was significantly lower in the outpatient care group.Slovenian UC care follows the general international recommendations. Based on these and our experience, we formulated recommendations for adjustments to UC care, depending on the clinical condition of the umbilicus, in order to prevent important complications.

Background The criteria for identification of children with high risk of cerebral palsy are well documented, but the early identification of children at highest risk of minor motor deficits remains less clear. Aim To analyze the correlation between the quality of general movements (GMs) from term to twenty weeks postterm age and the motor competence between 5 and 6 years of age. Methods In the group of 45 preterm infants, the quality of GMs was assessed using Prechtl's method. The Movement Assessment Battery for Children (M-ABC) was used to test motor competence between 5 and 6 years of age. The correlations between GMs and M-ABC results were analyzed. Results During writhing period, the sensitivity of GMs to identify children with definite motor problem was 0.86 for total impairment, 0.67 for manual dexterity, 0.89 for ball skills and 0.92 for balance. During fidgety period, the sensitivity was higher than during the writhing period: 1.00 for total impairment, 1.00 for manual dexterity, 1.00 for ball skills and 0.83 for balance, respectively. The specificity was low at both ages (total scoring 0.24 at term and 0.21 at 3 months corrected age). Conclusion The sensitivity of GMs to identify children with definite motor problems is higher at the fidgety than at the writhing period. The specificity of GMs at the term and fidgety age to predict later motor abilities is low.

Neonatal electroencephalography (EEG) presents a challenge due to its difficult interpretation that differs significantly from interpretation in older children and adolescents. Also, from the technological point of view, it is more difficult to perform and is not a standard procedure in all neonatal intensive care units (NICUs). During recent years, long-term cerebral function monitoring by the means of amplitude-integrated EEG (aEEG) has become popular in NICUs because it is easy to apply, allows real-time interpretation by the neonatologist treating the newborn, and has predictive value for outcome. On the other side, to record conventional EEG (cEEG), which is still considered the gold standard of neonatal EEG, the EEG technician should not only be well trained in performing neonatal EEG but also has to adapt to suboptimal working conditions. These issues need to be understood when approaching the neonatal cEEG in NICU and the main structure of the article is dedicated to this technique. The authors discuss the benefits of the digitalization and its positive effects on the improvement of NICU recording. The technical aspects as well as the standards for cEEG recording are described, and a section is dedicated to possible artifacts. Thereafter, alternative and concomitant use of aEEG and its benefits are briefly discussed. At the end there is a section that presents a review of our own cEEG and aEEG recordings that were chosen as the most frequently encountered patterns according to Consensus statement on the use of EEG in the intensive care unit.

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. ( J Child Neurol 2004;19:439-442).

To evaluate carboxyhemoglobin (COHb) values measured with a CO-oximeter (Roche-cobas b 221) in jaundiced newborns with or without hemolysis and healthy controls in order to assess whether COHb measurement determined with a CO-oximeter could be used as an indicator of hemolysis in newborns with ABO alloimmunization.A total of 86 term newborn infants were prospectively studied. The study cohort consisted of three subgroups: 18 infants with ABO HDN, 21 infants with hyperbilirubinemia without hemolytic disease who required phototherapy, and 47 healthy controls. The COHb, bilirubin, and Hb levels were measured.The three subgroups did not differ significantly with respect to birth weight, gestational age, gender, Apgar score, or mode of delivery. The ABO HDN infants had significantly higher COHb values than the healthy controls (median 2.4% versus 1.3%, p 0.0005) and the group with hyperbilirubinemia without hemolytic disease (median 2.4% versus 1.3%, p 0.0005), although the infants with hyperbilirubinemia without hemolytic disease did not have significantly higher COHb values compared with the healthy controls. The cut-off value of 1.7% COHb had 72% sensitivity and 97% specificity for confirming hemolysis in ABO alloimmunization.Our data show that COHb values determined with CO-oximeters are higher in newborns with hemolysis than in those without hemolysis. COHb measured with CO-oximeters could be used to confirm hemolysis in infants with ABO alloimmunization.

Background The risk of cognitive disability in preterm infants is higher than in general population. The Amiel-Tison neurological assessment could be a useful tool for early identification of preterm children at risk of cognitive disability in school age. This study investigated the value of categorization of neurological signs assessed by the Amiel-Tison neurological assessment in the first 2 years of life in relation to cognitive performance at school age in a group of preterm children. Methods Preterm children with gestational age from 23 to 36 weeks were included in the prospective study. From the initial group of 45 children, in whom the Amiel-Tison neurological assessment was performed at term age, at 3 months corrected age, and at 2 years, the Wechsler Intelligence Scale for Children–third edition was performed in 39 children after school entry. Results Full scale IQ, Verbal IQ, and Performance IQ of the whole group of preterm children were not significantly different from the normative data; most of the children had IQ scores in the normal range (≥85). The mean cognitive results of children decreased as the number of neurological signs increased. There was a significant correlation between the categories of neurological signs at 2 years and later cognitive results. Conclusions The grade of severity of neurological signs at 2 years was associated with the cognitive results at school age. The categorization of neurological signs according to the Amiel-Tison neurological assessment in preterm children might have prognostic value for cognitive outcome at school age.

Benign neonatal sleep myoclonus (BNSM), first described in 1982 by Coulter and Allen [1], is the non-epileptic condition most frequently confused with epileptic seizures during infancy. The etiology is uncertain: transient dysfunction of the serotoninergic system, a benign disturbance of the brainstem reticular activating system involved with initiation and synchronization of sleep and a genetic predisposition have been suggested by some authors. However none of these mechanisms has been proved [1,2]. The prevalence is also unknown, but it is thought that the condition is under-recognized. It was first described in three boys and in most subsequent publications, the condition was more commonly described in boys. Very recently, it has been shown that BNSM is extremely frequent in the newborn offspring of opiate-dependent mothers [3]. Myoclonic jerks usually present within a few days of birth. In a detailed study of 38 patients with BNSM the jerks appeared within the first 16 days, (median 3 days) and remitted in the first 10 months (median 2 months) of life [4]. It is usually said that the jerks disappear by the age of 3 months, but Egger et al. [5] demonstrated that the condition could persist for months and years. In the first description of BNSM myoclonus was reported to be bilateral, synchronous, and to mainly involve the distal parts of the upper extremities, with flexion of fingers, wrist, and elbow [1]. It is usually de

Benign familial neonatal convulsions (BFNC) is a rare, clinically and genetically heterogenous epileptic disorder. Two voltage gated potassium genes, KCNQ2 and KCNQ3, have been identified as genes responsible for BFNC1 and BFNC2 respectively. While as many as 73 mutations of KCNQ2 have been described up to date, only 4 mutations in KCNQ3, 3 of them appearing in exon 5, have been identified. Mutation in exon 6 was found for the first time in a Chinese family, and here we report the same missense mutation of KCNQ3 within exon 6 in a Caucasian family, whose history and clinical picture were in accordance with BFNC.

Background While the predictive value of general movements for later cerebral palsy is well known, its value to predict minor neurological and developmental impairments is less clear. Aim To analyze the results of the assessment of general movements in relation to the developmental outcome measured by the Bayley scales of infant development in a group of preterm infants. Methods Twenty-six preterm infants (gestational age from 23 weeks to 36 weeks) were included. The results of the assessment of general movements at term age and at 3 months corrected age were compared to the results of the mental and psychomotor developmental index of the Bayley scales assessed between two and three years of chronological age. Results Infants with normal writhing general movements achieved the highest scores on the mental and psychomotor developmental index, and those with cramped-synchronized general movements had the lowest scores. Infants with normal general movements during the fidgety period achieved the highest scores on both scales; those with an absence of fidgety movements achieved the lowest scores. We found the sensitivity of general movements to predict cognitive impairments to be 1.00 during the writhing period and 0.83 during the fidgety period; and 0.85 and 0.54, respectively, to predict motor impairments. The differences in the mental developmental index score between the groups with different qualities of general movements were significant in the writhing period and approached significance in the fidgety period, while for the psychomotor developmental index the differences between the groups with different qualities of general movements were not significant. Conclusion The quality of general movements may be predictive of later development.

Abnormal movement patterns during the fidgety period, as identified by Prechtl's method for qualitative assessment of general movements, and the presence of minor neurologic and cranial signs at age 2 years, as defined by Amiel-Tison, are related to minor developmental disorders. Our study analyzed the relationship between the two assessment methods in 45 preterm infants. Cerebral palsy was identified in 4, minimal cerebral palsy in 2, and the Amiel-Tison triad in 4 children; in all, continuously abnormal patterns of general movements were present. In the intermediate group with 2 signs of the triad, one child exhibited normal movements in the writhing period, and all were abnormal in the fidgety period. The intermediate group, with one sign of the triad, comprised 9 children: abnormal findings in the writhing period were present in 8, and in the fidgety period in 7. Among 16 children without neurologic signs, normal general movements were present in 7 children during the writhing period, and in 5 during the fidgety period. We confirmed good correlation between general movements and neurologic outcome at age 2 years (Pearson's R at term age, 0.51; at fidgety period, 0.62).

Background Benign neonatal sleep myoclonus (BNSM) is characterised by myoclonic jerks that occur only during sleep and stop abruptly when the child is aroused. It is a benign condition that is frequently confused with epileptic seizures during infancy. Aims The purpose of this study was to examine the clinical characteristics of newborns and infants with BNSM. Methods In a retrospective study of 38 newborns with BNSM, the entire inpatient charts were reviewed and data on the clinical picture and results of investigations were studied. The data for analysis of the children's neurological development and the time at which myoclonic jerks ceased were provided from outpatient files and parental interviews. Results Myoclonic jerks started between day 1 and day 16 (median 3 days, 95% confidence interval 2–4 days); they were mostly bilateral synchronous and symmetrical in distribution and never involved the facial muscles. An episode of jerks lasted from a few seconds up to 20 min. In every instance, the jerks occurred only during sleep: when the child was drowsy or fell asleep in 20, when the child had been asleep for more than 20 min in 5, before awaking in 3 and in different stages of sleep in 5 infants. In the majority of cases (32), the jerks were reported to stop spontaneously, and in 6 on arousal. Neurological evaluation revealed optimal results in the majority of infants (22), hyperexcitability in 11 and mild abnormalities of muscle tone in 5. In two infants electroencephalography (EEG) performed during the attack of jerks showed no paroxysmal activity. In the remainder EEG performed between the episodes of jerks was normal, apart from mild abnormalities found in six infants. US revealed normal results in all except eight infants, in whom mild abnormalities were found. Myoclonic jerks remitted between the age of 2 weeks and 10 months (median 2 months, 95% confidence interval 1–2 months). Follow-up between 3 and 12 months of age revealed optimal results of neurological assessment in 30 infants and mildly abnormal neurological signs in 8. Conclusion In the studied group, myoclonic jerks occurred within the first 16 days (median 3 days) of life irrespective of other conditions. They were predominantly observed at the beginning of the sleep and disappeared spontaneously in the first 10 months (median 2 months) of life.

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).

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.

Spinal epidural abscess (SEA) has an incidence of 0.2–1.2 per 10000 hospital admissions and is particularly rare in neonates and infants [6]. It can be potentially devastating, thus prompt diagnosis is essential for a good outcome. A 3-week-old infant boy, who was born after an uneventful pregnancy and delivery, was admitted to our hospital due to progressively decreasing movement of the upper limbs, which had been first noticed 1 week prior to admittance. During this time, he had a brief febrile episode. Upon admission he was irritable, with a tympanic temperature of 37.4°C. Paresis and areflexia of both arms were found, but finger movements were still present in the right arm. No other abnormalities were found upon clinical examination. Laboratory tests showed a slightly elevated level of C-reactive protein (16) and a white blood cell count of 10,400 cells/mm. There was an elevated protein level (1.32 g/L) and pleocytosis (51) in the cerebrospinal fluid (CSF). The blood, CSF and urine cultures were found to be microbiologically negative. Magnetic resonance imaging (MRI) of the cervical spine revealed discitis at level C4/C5 and anterior epidural and prevertebral abscesses (Fig. 1a). The boy was immediately operated on using an anterior approach to C4/C5: after the incision of a glossy, bulging anterior longitudinal ligament, yellowish pus was released and washed-out. Particles of the intervertebral disc C4/C5 were removed and the intervertebral space was enlarged by a bone spreader and irrigated with antibiotics. The postoperative course was uneventful. Beta haemolytic streptococci were grown on culture. Systemic antibiotic therapy with ampicillin, clindamycin and gentamicin was started before surgery and continued for 3 weeks. The boy recovered without any sequaelae. No abnormalities were found on clinical or neurological examination 2 months after surgery. His development was normal on follow-up 2 and 13 months later. MRI 2 months postoperative revealed a complete resolution of the abscess and no abnormalities in the cervical spine or spinal cord (Fig. 1b). While in adults a classic clinical course of SEA with back pain, spinal root pain, paresis and paralysis is obvious, the presentation in infants is unspecific. The localising signs of a SEA are related to the anatomical configuration of the epidural space, which dorsally contains a relatively large amount of areolar tissue and a rich venous plexus, therefore providing an available focus for infection, which may reach the epidural space by direct extension from local vertebral osteomyelitis or by hematogenous spreading [1, 3]. The most frequently isolated etiologic agent is Staphylococcus aureus. Streptococcus sp., Candida and coliform bacteria have also been reported [4]. MRI is considered to be the investigation methodology of choice. Gadolinium-enhanced images have increased the sensitivity of MRI for infectious processes. Our experience shows that early recognition and a combination of surgical intervention and antibiotic therapy provides excellent results, although a good outcome without surgical intervention has been reported [2, 5]. D. Paro-Panjan (*) Neonatal Unit, Division of Pediatrics, University Medical Centre, 1000 Ljubljana, Slovenia e-mail: darja.paro@volja.net Tel.: +386-1-5229274 Fax: +386-1-5229357