Cerebral Palsy in Very Preterm Infants: A Nine-Year Prospective Study in a French Population-Based Tertiary Center.

OBJECTIVES: To describe the prevalence of cerebral palsy (CP) at age 2 years in infants born before 33 weeks of gestation and to analyze the fetal neuroprotective effect of the antenatal administration of magnesium sulfate (MgSO4) treatment on CP. STUDY DESIGN: Preterm infants born before 33 weeks of gestation and discharged from the Rouen University Hospital's Neonatal Intensive Care Unit between 2007 and 2015 were included. At age 2 years, pediatricians of the perinatal network of Eure and Seine-Maritime counties administered standardized questionnaires analyzing motor, cognitive, and behavioral items, derived from the Denver and Amiel-Tison scales. A routine protocol based on MgSO4 infusion was introduced in 2010. The primary outcome measure was the occurrence of CP according to the Surveillance of Cerebral Palsy in Europe network definition. RESULTS: A total of 1759 very preterm infants were included, among whom 138 (7.8%) died and 148 (9.1%) were lost to follow-up. Assuming that those lost to follow-up had no CP, at 2 years, 55 of 1621 infants (3.4%; 95% CI, 2.6%-4.4%) had CP. After statistical adjustment for birth term and antenatal corticosteroid use, a significant decrease in CP was observed after implementation of a protocol of MgSO4 administration in mothers before imminent preterm birth at <33 weeks of gestation (aOR, 0.53; 95% CI, 0.29-0.98; P = .04). CONCLUSIONS: The prevalence of CP at 2 years after very preterm birth was low. The implementation of a neuroprotective protocol with MgSO4 was associated with reduced CP occurrence; however, several relevant limitations must be considered for interpretation.

Progressive demyelinating neuropathy correlates with clinical severity in Cockayne syndrome.

OBJECTIVE: Cockayne syndrome (CS) is characterized by postnatal growth failure and progressive multi-organ dysfunctions. CSA and CSB gene mutations account for the majority of cases and three degrees of severity are delineated. A peripheral neuropathy is known to be associated with CS but the type, severity and correlation of the nerve involvement with CS subtypes remain unknown in genetically identified patients. METHODS: Clinical and nerve conduction studies (NCS) in 25 CS patients with CSA (n=13) CSB (n=12) mutations. RESULTS: NCS show a widespread decrease in motor and sensory conduction velocities (CV) in all severe and classical form of CS. In one patient, CV were normal at age 8months but severe slowing was detected at 2years. Conduction block and/or temporal dispersion were observed in 68% of patients. CONCLUSIONS: CS is associated with a progressive sensory and motor neuropathy. Signs of segmental demyelination, including conduction blocks, may not be obvious before the age of 2years. CV slowing is correlated with the CS clinical severity. SIGNIFICANCE: NCS should be performed in patients with suspected CS as an additional tool to guide the diagnosis before molecular studies. Further studies focused on NCS course are required in order to assess its relevance as a biomarker in research therapy projects.