Abnormal Psychomotor Development Research Articles

Thrombocytopenia with absent radii syndrome with delayed presentation of thrombocytopenic episodes: a case report.

The authors reported a case of a 6-month-old baby girl who experienced thrombocytopenia at the age of 6 months for the first time, as a result of cow's milk being introduced for 45 days with chronic diarrhea and growth failure. She had a deviation of the axis of the hand laterally, and a bilateral absent of radii with the presence of both thumbs. In addition, she had abnormal psychomotor development, marasmus case manifestations. Our aim in publishing the current case report is that clinicians caring for patients with thrombocytopenia with absent syndrome will be aware of the myriad of complications, which may occur in the other organ systems so that they can promptly diagnose and treat any associated abnormalities.

Joubert syndrome in an adult: A case report

Joubert syndrome (JS) is a rare autosomal recessive central nervous system malformation characterized by hypotonia, ataxia, abnormal psychomotor development and mental retardation, variable ophthalmologic features such as oculomotor apraxia and neonatal breathing abnormalities. Many patients die in infancy or childhood, but some survive into adulthood with variable cognitive and motor impairments. Radiographically it is characterized by “molar tooth sign” in MRI, includes cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and abnormally deep interpeduncular fossa.

Nieprawidłowy rozwój psychomotoryczny dzieci. Część I: podstawowa definicja i informacje

Nieprawidłowy rozwój psychomotoryczny dzieci. Część I: podstawowa definicja i informacje

Nieprawidłowy rozwój psychomotoryczny dzieci. Część II: diagnostyka elektroencefalograficzna

Nieprawidłowy rozwój psychomotoryczny dzieci. Część II: diagnostyka elektroencefalograficzna

Neuroimaging Results, Short-term Assessment of Psychomotor Development and the Risk of Autism Spectrum Disorder in Extremely Premature Infants (≤28 GA) - a Prospective Cohort Study (preliminary Report)

Infants ≤28 GA are at particular risk of psychomotor and neurological developmental disorder. They also remain at a higher risk of developing autism spectrum disorder (ASD), characterized by persistent deficits in communication/social interactions and restricted, repetitive behaviors, activities and interests. Monitoring their development by a team of specialists (a neurologist, psychologist, psychiatrist) allows us to make an early diagnosis and to implement appropriate therapy. Neuroimaging studies during the neonatal period may be helpful in clarifying diagnosis and prognosis.ObjectiveThe aim of the study was to search for the interrelation between the results of neuroimaging and the neurological, psychological and psychiatric evaluation at the age of 2.Material and methodsNeonates born at ≤28 weeks between 01.06.2013 and 31.12.2015 and hospitalized at NICU were enrolled. We present the results of the first 12 children who have attained 2 years of corrected age and have undergone both neuroimaging, and neurological, psychological and psychiatric assessments. Transfontanel ultrasound was performed according to general standards, MRI between 38 and 42 weeks of corrected age. Neurological examination based on the Denver scale, ASD screening with use of the STAT test and psychological DSR assessment were performed at 2 years of corrected age.ResultsMedian GA was 26 weeks and median weight 795 g. The ultrasound examination was normal in 9 cases (75%) and MRI in 4 (33%). Abnormalities in the cerebellum were the main additional information found in MRI as compared to US. Neurological examination was normal in 8 infants (67%), in 4 of whom neuroimaging was normal. In 4 (33%) infants the neurological examination was abnormal. Psychomotor development at an average level or above was found in seven (58%) children. In 4 of them neuroimaging was normal, whereas 3 had ventricular dilatation and haemorrhagic infarct. There were no abnormalities within the cerebellum in this group. In the remaining 5 children (42%) psychomotor development was rated as delayed. All of them had cerebellar haemorrhage. An increased risk of ASD was observed in 4 children who developed cerebellar hemorrhage.Conclusions1. The use of MRI at a term-equivalent age may contribute to the prognosis of neurodevelopmental outcomes in extremely premature infants, allowing risk stratification and thus enhancing early monitoring of a child’s development and functional status 2. There is a clear tendency towards abnormal psychomotor development and positive screening for ASD to co-occur with abnormal MRI findings in the cerebellum.

Febrile Convulsions in Infants at the Pediatrics University Hospital Center Charles de Gaulle of Ouagadougou (Burkina Faso)

Context: Seizures rank high among the commonest emergencies encountered in Pediatrics. As far as the etiologies are concerned, the most frequently confronted cause is infectious diseases. Therefore, particularly in the present work context, febrile seizures have been inadequately investigated. The study aimed at assessing the prevalence of febrile convulsions in our pediatrics department. Materials and Methods: This retrospective study was performed in the Pediatrics Medical Service Department of the Pediatrics University Hospital Charles de Gaulle of Ouagadougou in Burkina Faso (West Africa). Infants from one and thirty months of age, hospitalized between January 1, 2011 and December 31, 2012, were included in this study. Seizures are defined as those who accompany fevers above or equal to 38°C. However, the exclusions from the study included those infants recognized as epileptics; those with abnormal psychomotor development; those afflicted with encephalitis and meningitis; and children with hypoglycemia or dehydration with ionic disorders, as well as those infants who lacked lumbar puncture results. Data were analyzed using the Epi Info software version 3.5.1. Results: While the average age of the patients was 13 months, the average incidence of the febrile seizures was 2.5%. The seizures occurred all through the year, peaking in October (14.1%). The peak frequency (38.7%) was recorded in children from 12 to 24 months. About one-half of the patients (46.2%) registered a temperature from 38.5°C to 39.4°C. In 68.9% of the cases, the tonic convulsions were of the common type of convulsions. The number of convulsions was in the range of >2 episodes/24 h in 83.3% of the children. The pathologies commonly associated with tonic convulsions included acute gastroenteritis (29.4%), malaria (25.8%) and bronchopneumopathies (23.3%). The evolution was favorable in 95.3% of the cases. Conclusion: While this study confirms the benign character of the febrile convulsions, their recurrent quality necessitates the codification of a prospective study, for clearer identification and closer case monitoring.

Impact of cerebral redistribution on neurodevelopmental outcome in small-for-gestational-age or growth-restricted babies: a systematic review

To review systematically the evidence on impact of cerebral redistribution, as assessed by fetal middle cerebral artery (MCA) Doppler, on neurological outcomes in small-for-gestational-age (SGA) or growth-restricted fetuses. For this systematic review, MEDLINE was searched for all controlled studies reporting neurological outcomes in SGA or growth-restricted babies with cerebral redistribution based on MCA Doppler indices, from inception to September 2013. We used relative risk or odds ratios, with 95% CI, to identify the association of cerebral redistribution with neurological outcomes. The search yielded 1180 possible citations, of which nine studies were included in the review, with a total of 1198 fetuses. Definitions of SGA and cerebral redistribution were variable, as was study quality. Data could not be synthesized in meta-analyses because of heterogeneity in outcome reporting. Cerebral redistribution was not associated with increased risk of intraventricular hemorrhage in neonates (five studies; n = 806). When present in preterm fetuses, cerebral redistribution was associated with normal Neonatal Behavioral Assessment Scale (NBAS) scores at 40 weeks (one study; n = 62) but abnormal psychomotor development at 1 year of age on the Bayley scale (one study; n = 172). When present in term SGA fetuses, cerebral redistribution was associated with increased risk of motor and state organizational problems on NBAS (two studies; n = 158), and lower mean percentile scores in communication and problem solving at 2 years of age on the Ages and Stages Questionnaire (one study; n = 125). SGA fetuses with cerebral redistribution may be at higher risk of neurodevelopmental problems. More data are needed from adequately controlled studies with long-term follow-up before conclusions can be drawn. If these findings are true, there is a need to re-evaluate timing of delivery in the management of SGA fetuses, particularly when cerebral redistribution is found at term gestation.

Neonatal sepsis as a risk factor for neurodevelopmental changes in preterm infants with very low birth weight

Objectiveto evaluate neonatal sepsis as a risk factor for abnormal neuromotor and cognitive development in very low birth weight preterm infants at 12 months of corrected age. Methodsthis was a prospective cohort study that followed the neuromotor and cognitive development of 194 very low birth weight preterm infants discharged from a public neonatal intensive care unit. The Bayley Scale of Infant Development (second edition) at 12 months of corrected age was used. The outcomes were the results of the clinical/neurological evaluation and the scores of the psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scale of Infant Development II. The association between neonatal sepsis and neuromotor development and between neonatal sepsis and cognitive development was verified by logistic regression analysis. Resultsmean birth weight was 1,119g (SD: 247) and mean gestational age was 29 weeks and 6 days (SD: 2). Approximately 44.3% (n=86) of the infants had neonatal sepsis and 40.7% (n=79) had abnormal neuromotor development and/or abnormal psychomotor development index (PDI<85) at 12 months of corrected age. On the mental scale, 76 (39.1%) children presented abnormal cognitive development (MDI<85). Children with neonatal sepsis were 2.5 times more likely to develop changes in neuromotor development (OR: 2.50; CI: 1.23-5.10). There was no association between neonatal sepsis and cognitive development impairment. Conclusionneonatal sepsis was an independent risk factor for neuromotor development impairment at 12 months of corrected age, but not for mental development impairment.

Neonatal sepsis as a risk factor for neurodevelopmental changes in preterm infants with very low birth weight

Objectiveto evaluate neonatal sepsis as a risk factor for abnormal neuromotor and cognitive development in very low birth weight preterm infants at 12 months of corrected age. Methodsthis was a prospective cohort study that followed the neuromotor and cognitive development of 194 very low birth weight preterm infants discharged from a public neonatal intensive care unit. The Bayley Scale of Infant Development (second edition) at 12 months of corrected age was used. The outcomes were the results of the clinical/neurological evaluation and the scores of the psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scale of Infant Development II. The association between neonatal sepsis and neuromotor development and between neonatal sepsis and cognitive development was verified by logistic regression analysis. Resultsmean birth weight was 1,119g (SD: 247) and mean gestational age was 29 weeks and 6 days (SD: 2). Approximately 44.3%(n=86) of the infants had neonatal sepsis and 40.7% (n=79) had abnormal neuromotor development and/or abnormal psychomotor development index (PDI < 85) at 12 months of corrected age. On the mental scale, 76 (39.1%) children presented abnormal cognitive development (MDI<85). Children with neonatal sepsis were 2.5 times more likely to develop changes in neuromotor development (OR: 2.50; CI: 1.23‐5.10). There was no association between neonatal sepsis and cognitive development impairment. Conclusionneonatal sepsis was an independent risk factor for neuromotor development impairment at 12 months of corrected age, but not for mental development impairment.

Early neurodevelopment in very low birth weight infants with mild intraventricular hemorrhage or those without intraventricular hemorrhage

PurposeThis study aimed to assess early development in very low birth weight (VLBW) infants with mild intraventricular hemorrhage (IVH) or those without IVH and to identify the perinatal morbidities affecting early neurodevelopmental outcome.MethodsBayley Scales of Infant Development-II was used for assessing neurological development in 49 infants with a birth weight <1,500 g and with low grade IVH (≤grade II) or those without IVH at a corrected age of 12 months.ResultsAmong the 49 infants, 19 infants (38.8%) showed normal development and 14 (28.6%) showed abnormal mental and psychomotor development. Infants with abnormal mental development (n=14) were mostly male and had a longer hospitalization, a higher prevalence of patent ductus arteriosus (PDA) and bronchopulmonary dysplasia (BPD), and were under more frequent postnatal systemic steroid treatment compared with infants with normal mental development (n=35, P<0.05). Infants with abnormal psychomotor development (n=29) had a longer hospitalization and more associated PDA compared to infants with normal psychomotor development (n=20, P<0.05). Infants with abnormal mental and psychomotor development were mostly male and had a longer hospitalization and a higher prevalence of PDA and BPD compared to infants with normal mental and psychomotor development (n=19, P<0.05). Using multiple logistic regression analysis, a longer duration of hospitalization and male gender were found to be significant risk factors.ConclusionApproximately 62% of VLBW infants with low grade IVH or those without IVH had impaired early development.

Epilepsia farmacorresistente: uso de la nueva definición y factores de riesgo relacionados. Estudio en población mexicana de un centro de tercer nivel

The International League against Epilepsy (ILAE) has recently proposed a consensus to define refractory epilepsy. A retrospective, analytical, descriptive study was conducted involving 206 patients who were attended at the epilepsy clinic of a third-level centre in Mexico. The ILAE's new definition of pharmacoresistance was used to classify the patients. The frequency and risk factors involved in the refractoriness were reported. The tendency to prescribe antiepileptic drugs (AED) in this population was analysed using secondary data. Of the 206 patients analysed, 57 (28%) were free of seizures, 115 (56%) continued to have seizures, 17 (8%) were classified as undetermined and 17 (8%) were pseudo-refractory. The statistically significant factors associated to pharmaco-resistance that were found in our population were: focal versus generalised epilepsy (p = 0.02), catamenial epilepsy (p = 0.02), abnormal psychomotor development (p = 0.02), history of epilepsy in the family (p = 0.025), neurological examination with focal abnormality (p = 0.04), perinatal hypoxia (p = 0.06) and prior psychiatric illness (p = 0.03). The mostly frequently AED used in mono- and polytherapy were valproate and carbamazepine. Of all the patients who were free of seizures, 47% were treated using monotherapy. The frequency of pharmacoresistance in the population analysed was estimated to be 56%. Identification of the factors associated with pharmacoresistance makes it possible to consider surgical treatment or to optimise the treatment with AED. There is a tendency to use the new AED, above all in combination therapy. Our findings are consistent with those described in the literature.

Resistance to Antiretroviral Drugs: A Threat to the Prevention and Treatment of Pediatric HIV Infection

Mother-to-child transmission (MTCT) of HIV was recognized early in the worldwide AIDS pandemic and by the late 1980s abnormal psychomotor development candida esophagitis and recurrent invasive bacterial infections were recognized as signatures of HIV infection in infants. Pneumocystis pneumonia occurred in many infants within the first 3-6 months of life often preceding death by as little as 1 month. Although symptoms of HIV infection generally appeared much later children who developed symptoms in the first year of life generally died by 2-3 years of age and AIDS became the seventh leading cause of mortality in young children in the United States by 1996. Nucleoside analogue reverse-transcriptase inhibitor (NRTI) antiretroviral therapy delayed the progression of disease and zidovudine sometimes caused dramatic improvements in cognitive function but these salutary effects were usually modest and transient in nature. Fortunately striking progress in both the prevention and treatment of HIV infection of children was made in early to mid-1990s. Administration of zidovudine during pregnancy labor and delivery accompanied by 6 weeks of postnatal therapy for the infant was shown to reduce MTCT of HIV by 70%. A series of studies in the 1990s identified virological immunological and obstetrical variables linked with perinatal infection of infants. (excerpt)

Subtypes of autism by cluster analysis based on structural MRI data

The aim of our study was to subcategorize Autistic Spectrum Disorders (ASD) using a multidisciplinary approach. Sixty four autistic patients (mean age 9.4+/-5.6 years) were entered into a cluster analysis. The clustering analysis was based on MRI data. The clusters obtained did not differ significantly in the overall severity of autistic symptomatology as measured by the total score on the Childhood Autism Rating Scale (CARS). The clusters could be characterized as showing significant differences: Cluster 1: showed the largest sizes of the genu and splenium of the corpus callosum (CC), the lowest pregnancy order and the lowest frequency of facial dysmorphic features. Cluster 2: showed the largest sizes of the amygdala and hippocampus (HPC), the least abnormal visual response on the CARS, the lowest frequency of epilepsy and the least frequent abnormal psychomotor development during the first year of life. Cluster 3: showed the largest sizes of the caput of the nucleus caudatus (NC), the smallest sizes of the HPC and facial dysmorphic features were always present. Cluster 4: showed the smallest sizes of the genu and splenium of the CC, as well as the amygdala, and caput of the NC, the most abnormal visual response on the CARS, the highest frequency of epilepsy, the highest pregnancy order, abnormal psychomotor development during the first year of life was always present and facial dysmorphic features were always present. This multidisciplinary approach seems to be a promising method for subtyping autism.

Endogenous Attention in 5-Month-Old Full-Term and Premature Infants

Recent research has demonstrated that a deficit of endogenous attention (EA) is a main and stable characteristic of the mental development in healthy extremely premature (HEP) infants from the age of 2 years. A study was made of the differences in EA (anticipatory attention) between full-term (FT) and HEP infants, both aged 5 months (the HEP infants’ ages were corrected for prematurity). The general psychomotor development of the HEP infants was comparable to that of the age-matched FT infants. However, the HEP infants significantly differed from the FT infants in the ability to keep the attention on an object absent in the visual field. Unlike the HEP infants, the FT infants demonstrated a predominance of EA over all other types of attention in the visual expectation paradigm, as confirmed by ANOVA with the factors Maturity and Attention Type. Comparison of the frequency of different types of attention between the two groups showed that the EA frequency in the HEP infants was significantly lower than that in the FT infants. The findings showed that HEP infants, even those with a low risk for abnormal psychomotor development, demonstrate the impairment of EA control as early as at the age of 5 months.

Prenatally diagnosed fetal ventriculomegaly: prognosis and outcome

The purpose of the present study was to determine the postnatal outcome and prognostic factors of prenatally diagnosed ventriculomegaly, and to establish the relationship between prenatal sonographic measurements and postnatal psychomotor development. A total of 42 singleton pregnancies with sonographically determined fetal ventriculomegaly at 20-38 weeks' gestation were reviewed, together with follow-up data on postnatal outcome at a mean of 29 months after delivery. Sonographic measurements included head circumference, cerebral lateral ventricular diameter at the anterior and posterior horn level, and hemisphere diameter. Classification of psycho-motor development consisted of assessment of motoric behaviour, speech, communication and social skills ('Van Wiechen' classification). Perinatal mortality rate was 38 per cent, of which half were directly associated with cephalocentesis. Only the ventricle/hemisphere ratio for the anterior and posterior horn of the lateral cerebral ventricles was significantly higher among perinatal deaths than amongst the survivors. Within the subset of survivors (n = 26), psycho-motor development was normal in 46 per cent. Postnatal examination revealed syndrome anomalies in five infants, of which four were associated with psycho-motor retardation. Prenatally diagnosed ventriculomegaly has a poor postnatal outcome with more than 50 per cent of the live-born infants demonstrating abnormal psycho-motor development. The predictive value of fetal biometric measurements is poor. The presence of syndromal anomalies emphasizes the need for genetic counselling in future pregnancies.

Prenatally diagnosed fetal ventriculomegaly; prognosis and outcome.

The purpose of the present study was to determine the postnatal outcome and prognostic factors of prenatally diagnosed ventriculomegaly, and to establish the relationship between prenatal sonographic measurements and postnatal psychomotor development. A total of 42 singleton pregnancies with sonographically determined fetal ventriculomegaly at 20-38 weeks' gestation were reviewed, together with follow-up data on postnatal outcome at a mean of 29 months after delivery. Sonographic measurements included head circumference, cerebral lateral ventricular diameter at the anterior and posterior horn level, and hemisphere diameter. Classification of psycho-motor development consisted of assessment of motoric behaviour, speech, communication and social skills ('Van Wiechen' classification). Perinatal mortality rate was 38 per cent, of which half were directly associated with cephalocentesis. Only the ventricle/hemisphere ratio for the anterior and posterior horn of the lateral cerebral ventricles was significantly higher among perinatal deaths than amongst the survivors. Within the subset of survivors (n = 26), psycho-motor development was normal in 46 per cent. Postnatal examination revealed syndrome anomalies in five infants, of which four were associated with psycho-motor retardation. Prenatally diagnosed ventriculomegaly has a poor postnatal outcome with more than 50 per cent of the live-born infants demonstrating abnormal psycho-motor development. The predictive value of fetal biometric measurements is poor. The presence of syndromal anomalies emphasizes the need for genetic counselling in future pregnancies.

Plasma infusions in the Hurler syndrome. Influence during the first year of life.

An attempt has been made to determine if plasma infusions begun in the neonatal period influence the course of the Hurler syndrome. The progression of the disorder was assessed in terms of clinical and developmental standards and mucopolysaccharide metabolism. Weekly infusions of plasma have been associated with a progression of the physical stigmata of the Hurler syndrome and abnormal psychomotor development at 1 year of age. There was no significant change in urinary excretion of either the total amount or the ratio of large molecular weight mucopolysaccharides to fragments during daily or weekly plasma infusions. There is no evidence that plasma, in the quantities infused, alters the development of the clinical phenotype of the Hurler syndrome.

Factores De Riesgo Presentes En Una Muestra De Poblacion Epileptica De Manizales, Caldas, Manizales

Introducción: La epilepsia es una de las enfermedades neurológicas más frecuentes; existen múltiples estudios sobre la prevalencia en la población general, con cifras que oscilan entre 1,5 y 57 casos por 1.000 habitantes. Los factores de riesgoasociados a esta patología no se han logrado determinar de forma definitiva. Materiales y métodos: mediante un muestreo por conveniencia realizado a los asistentes a controles neurológicos en la Liga Caldense contra la Epilepsia y los acudientes a consulta neuropediátrica al Hospital Infantil de la Cruz Roja Colombiana - Seccional Caldas, se realizó un estudio descriptivo correlacional a partir de encuestas y revisiónde historias clínicas. Resultados: El 73,3% de los pacientes estaban entre los 0 y 20 años, predominó el género masculino (61,3%). El 83,8% pertenecía a un estrato socioeconómico bajo (I-II); el 69,6% fue producto de un primer o segundo embarazo; un 38,2% tuvo un desarrollo psicomotor anormal y un 39,7% presentó un desarrollo del lenguaje inadecuado. En el 58% el inicio de las crisis se desarrolló en menores de10 años, el tipo de crisis más común fue la tónico clónica generalizada, con 65,3%. Conclusión: Los factores de riesgo en epilepsia siguen siendo desconocidos en su totalidad, pese a los múltiples estudios realizados; por ello es indispensable crearestrategias a partir de estos hallazgos, para que de manera significativa disminuya el impacto biopsicosocial de esta enfermedad.