Relationship between cerebral palsy severity and cognition, aids and education.

BACKGROUND: Cerebral palsy (CP) refers to a non-progressive permanent lesion of the developing brain, which can manifest with motor function disability and various comorbidities and complications. However, there is little data on the correlation between motor and mental function in CP, as cognitive assessments are rarely the main focus of studies on children with CP. METHODS: We studied a large cohort of 381 children and adolescents with CP. We analyzed the relationship between severity of CP and the presence of developmental disturbances (motor, motor-linguistic, global) including cognition, the number of aids and education. RESULTS: We found a strong correlation between the severity of CP according to the Gross Motor Function Classification System (GMFCS) and developmental disturbances. In line with this finding, the number of aids per individual also correlated significantly with CP severity and the extent of developmental disturbance. Going beyond the number of aids most patients already received special education in kindergarten. Later, the type of schooling correlated significantly with severity of CP and developmental disturbance. While developmental disturbance and cognition correlated, this was not the case for CP severity and cognition. The latter indicates a wide range in individual manifestation and resources. CONCLUSIONS: Our data underline that cognition does not necessarily correlate with CP severity. Thus, in addition to the evaluation and treatment of motor deficits, cognitive assessment should be offered early-on to improve patient-centered counselling and support with respect to appropriate education.

Clinical Phenotype of Cerebral Palsy Depends on the Cause: Is It Really Cerebral Palsy? A Retrospective Study.

Cerebral palsy is the most common motor disability in childhood. Still, the precise definition in terms of causes and timing of the brain damage remains controversial. Several studies examine the clinical phenotype of cerebral palsy types. The aim of our study was to determine to what extent the clinical phenotype of cerebral palsy patients depends on the underlying cause. We retrospectively evaluated the clinical phenotype, abnormalities during pregnancy, and cerebral palsy cause of 384 patients, treated at Charité-Medicine University, between 2015 and 2017. The cause of cerebral palsy was identified in 79.9% of cases. Causes prior to the perinatal period were, compared to perinatal brain damage, associated significantly with different comorbidities. The term cerebral palsy does not describe a single disease but is an umbrella term covering many different diseases. Depending on the cause, a varying clinical phenotype can be found, which offers great potential in terms of individual treatment and preventing comorbidities.

Neurodevelopmental outcome at 2 years after neuroendoscopic lavage in neonates with posthemorrhagic hydrocephalus.

OBJECTIVE: A standardized guideline for treatment of posthemorrhagic hydrocephalus in premature infants is still missing. Because an early ventriculoperitoneal shunt surgery is avoided due to low body weight and fragility of the patients, the neurosurgical treatment focuses on temporary solutions for CSF diversion as a minimally invasive approach. Neuroendoscopic lavage (NEL) was additionally introduced for early elimination of intraventricular blood components to reduce possible subsequent complications such as shunt dependency, infection, and multiloculated hydrocephalus. The authors report their first experience regarding neurodevelopmental outcome after NEL in this patient cohort. METHODS: In a single-center retrospective cohort study with 45 patients undergoing NEL, the authors measured neurocognitive development at 2 years with the Bayley Scales of Infant Development, 2nd Edition, Mental Developmental Index (BSID II MDI) and graded the ability to walk with the Gross Motor Function Classification System (GMFCS). They further recorded medication with antiepileptic drugs (AEDs) and quantified ventricular and brain volumes by using 3D MRI data sets. RESULTS: Forty-four patients were alive at 2 years of age. Eight of 27 patients (30%) assessed revealed a fairly normal neurocognitive development (BSID II MDI ≥ 70), 28 of 36 patients (78%) were able to walk independently or with minimal aid (GMFCS 0-2), and 73% did not require AED treatment. Based on MR volume measurements, greater brain volume was positively correlated with BSID II MDI (rs = 0.52, 95% CI 0.08-0.79) and negatively with GMFCS (rs = -0.69, 95% CI -0.85 to -0.42). Based on Bayesian logistic regression, AED treatment, the presence of comorbidities, and also cerebellar pathology could be identified as relevant risk factors for both neurodevelopmental outcomes, increasing the odds more than 2-fold-but with limited precision in estimation. CONCLUSIONS: Neuromotor outcome assessment after NEL is comparable to previously published drainage, irrigation, and fibrinolytic therapy (DRIFT) study results. A majority of NEL-treated patients showed independent mobility. Further validation of outcome measurements is warranted in an extended setup, as intended by the prospective international multicenter registry for treatment of posthemorrhagic hydrocephalus (TROPHY).

Laboratory markers of perinatal acidosis are poor predictors of neurodevelopmental impairment in very low birth weight infants.

BACKGROUND: In asphyxiated term and near-term infants, therapeutic hypothermia increases survival without neurologic morbidity, and extending this new treatment to preterm infants is being debated. AIMS: To investigate the association of low pH and base excess (BE) at birth or admission, as used as entry criteria in cooling trials, and evolving brain damage in preterm infants. STUDY DESIGN AND MEASUREMENTS: Rates of death and neurodevelopmental impairment at 12 and 20 months corrected age were assessed in a cohort of 1137 preterm infants with a gestational age <35 weeks and birth weight <1500 g in relation to severe perinatal acidosis (umbilical artery pH≤7.0, pH at admission ≤7.0, BE at admission ≤-16 mmol/l, lowest BE during first 12 h of life ≤-16 mmol/l). RESULTS: Umbilical artery pH was not linked to death or neurodevelopmental impairment. There was only weak predictive power of pH or BE at admission for death (positive predictive values [PPV] 0.36/0.30, receiver operator characteristics [ROC] areas 0.591/0.701), and lowest 12-h BE for death or neurodevelopmental impairment at 12 or 20 months (PPV 0.29/0.30/0.27, ROC 0.720/0.656/0.658). CONCLUSION: In very preterm infants, there is little association between laboratory markers of severe perinatal acidosis and neurodevelopmental outcome at 12 or 20 months.

Dysphonia at 12 months corrected age in very low-birth-weight-born children.

Preterm newborn infants may suffer laryngeal injuries after multiple intubations and long-term mechanical ventilation. Former studies have focused on acute laryngeal injuries diagnosed by endoscopy, performed within the neonatal period. This retrospective case-control study aims to investigate the prevalence and clinical risk factors for voice disorders in former very low-birth-weight (< 1,500 g) infants (VLBW) at 1-year follow-up examinations. We screened former VLBW infants for presence of dysphonia at the corrected age of 1 year and compared cases with unaffected infants matched by birth weight and gestational age. Of the 843 former VLBW infants, admitted from January 1998 to May 2006, 18 subjects had persistent dysphonia. All cases had a birth weight below 1,000 g. Surgical ligation of a ductus arteriosus had been performed in ten infants. Duration of ventilation and number of intubations were not different between cases and controls, but a documented difficult intubation was a predictor of subsequent dysphonia. The rate of dysphonia at 1 year of life was 6.6% among formerly ventilated infants with birth weights <1,000 g (extremely low-birth-weight infants). Persistent dysphonia has to be added to the list of specific long-term consequences of extremely immature birth and given attention at follow-up examinations.

Echocardiography predicts closure of patent ductus arteriosus in response to ibuprofen in infants less than 28 week gestational age.

BACKGROUND: Patent ductus arteriosus (PDA) is a frequent problem in preterm infants, and its incidence is inversely correlated with gestational age. The efficacy of medical treatment decreases with decreasing gestational age (GA), and failure rates as well as ductus ligation rates of 40% have been reported in <28 week GA newborns. The aim of this study was to determine whether echocardiographic parameters can predict response to ibuprofen treatment of PDA. STUDY DESIGN: In a longitudinal study, 29 infants born <28 week GA were screened for a significant PDA (left atrial to aortic root ratio>1.4, anterior cerebral artery resistance index>0.8, and oxygen requirement>35%) at 24-72 h of life and, if a PDA was found, treated with 10-5-5mg/kg ibuprofen intravenously every 24h. Ductal parameters were monitored by serial echocardiography. Infant neurodevelopmental outcomes were assessed at 24 month corrected age. RESULTS: All 15 infants with significant PDA responded to the ibuprofen loading dose indicated by reduced PDA diameters or increased PDA maximum flow velocities (PDA V(max)), and 7 patients showed an ongoing response resulting in a closed PDA after the 1st cycle (47%). Of the 8 non-responders, 7 received a 2nd cycle with 2 further responders (29%). All non-responders to the 2nd course had a PDA V(max)

Parental bilingualism is associated with slower cognitive development in very low birth weight infants.

BACKGROUND: Speech development is frequently impaired in very low birth weight (VLBW) infants. Few and controversial data have been published on concepts regarding the influence of bilingual education. AIMS: The objectives of the current study were to assess the influence of parental bilingualism on speech development and neurodevelopmental outcome in low risk VLBW infants. STUDY DESIGN: Monocentric prospective controlled cohort study with standardized follow-up. SUBJECTS: We recruited 50 singleton VLBW infants each from monolingual and bilingual families as well as 90 term control infants. The infants were free of disease and congenital malformation. OUTCOME MEASURES: Griffiths scales of infant development at the corrected ages of 6 and 12 months, Bayley Scales of Infant Development II (BSID II) with 22 months. RESULTS: In general, both bilingual and monolingual VLBW infants achieved age-specific milestones at the corrected age of 6, 12 and 22 months. However, bilingual VLBW infants achieved significantly lower scores than their monolingual peers in all cognitive subscales. The influence of maternal education on the neurodevelopmental outcome of the preterm infants was not significant; the subscales' correlation with socioeconomic or biological parameters was poor. However, a clear differentiation between social status and bilingual environment importance for speech development was not possible. CONCLUSIONS: In the setting of the present investigation, parental bilingualism is associated with slower neurodevelopment in VLBW infants during the first 2 years of life.

Impaired word stress pattern discrimination in very-low-birthweight infants during the first 6 months of life.

Prosodic information, such as word stress and speech rhythm, is important in language acquisition, and sensitivity to stress patterns is present from birth onwards. Exposure to prosodic properties of the native language occurs prenatally. Preterm birth and an associated lack of exposure to prosodic information are suspected to affect language acquisition in preterm infants. Fifty healthy very low birthweight (<1500 g) preterm German infants (24 males, 26 females; mean gestational age [GA] 27.6 wks, range 26.4-29.9) and 103 comparison term infants (48 males, 55 females; mean GA 40 wks, range 39.4-40.8) were recruited. Prosodic discrimination performance was assessed using the head-turn preference paradigm, an objective behavioural psycholinguistic test for measuring orientation time (OT) to auditory stress patterns. Among matched preterm and term infants, preterm infants (n=30) did not differentiate stress patterns at the corrected age of 4 or 6 months. In term infants (n=30), the OT was longer towards the trochaic (stress on first syllable, characteristic for German) than the iambic (second syllable) stress patterns (11.64 vs 9.18s, p<0.001, and 11.02 vs 8.32s, p<0.001, at 4 and 6 mo respectively). Neurodevelopmental scores (Bayley Scales of Infant Development, 2nd edn) were not different from reference values in both groups of infants. Preterm birth and deficient early prosodic information affect prosodic processing during the first half year of life.

Genetic deafness in a preterm infant with a critical postnatal course.

OBJECTIVE: We present a case of deafness in a preterm infant with several predisposing factors of an acquired hearing impairment that, however, turned out to have a genetic cause. We describe the severe postnatal course and review the relevant literature. DESIGN: Case report. SETTING: University-based tertiary neonatal intensive care unit. PATIENT: Preterm infant (gestational age, 26/37; wks). MEASUREMENTS AND MAIN RESULTS: A preterm infant exhibited hearing impairment after a complicated clinical course with pneumothoraces, a hemodynamically relevant patent ductus arteriosus, treatment with potentially ototoxic drugs, intraventricular hemorrhage, and periventricular leukomalacia. Despite the absence of a family history for deafness, genetic testing was performed. Surprisingly, genetic analysis revealed the presence of two compound heterozygous mutations in the patient's GJB2 gene as the cause for his early-onset nonsyndromic deafness. CONCLUSION: To elucidate the nature of a hearing disorder, it is worthwhile to consider a genetic cause, despite the fact that it may seem unlikely in a severely sick preterm infant with numerous risk factors for a postnatally acquired hearing impairment and without a positive family history.