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Background: Neonatal branching echogenic streaks in the basal ganglia or 'lenticulostriate vasculopathy' (LSV) has no clear comprehensive aetiology., Objective: To provide some clinical evidence, possibly relevant to aetiology, by analysis of a large series., Materials and Methods: Seventy cases (9 deaths, 3 post mortem) identified between 1981 and 2000 out of 9,138 neonates with routine brain sonograms (578 in a protocol for neonates from HIV+ mothers). Review of maternal/neonatal charts for clinical data and serologic status. Retrospective analysis of sonograms for grading and time course of LSV and coexistent abnormalities., Results: LSV was unilateral in 31 cases (22 right), bilateral in 39. Grading separated 8 major, 27 moderate and 35 minor patterns. In 42 cases, LSV was isolated, and in 28 it was mixed with peri-intraventricular haemorrhage grades I or II and/or leucomalacia. LSV was detected in the first postnatal week in 56 instances and appeared later in 13. Disappearance occurred mostly within 2 months. Perinatal circumstances: 13 recipient monochorionic twins (polycythaemia from twin-twin transfusion syndromes), 20 HIV+ mothers (possible immunological disturbances; only 2 infected neonates), 10 congenital anomalies, 1 toxoplasmosis, 1 postnatal cytomegalovirus infection, 25 miscellaneous conditions. Numerous maternal and neonatal factors were non-contributory., Conclusions: These results do not support congenital toxoplasmosis, rubella, cytomegalovirus, and herpes (TORCH) infections as the main causative factor. Polycythaemia and various immunological disturbances may be involved, as well as infectious agents not appropriately screened by routine serodiagnoses.

Objective: Advances in obstetrical and neonatal care have achieved dramatic reductions in the mortality of extremely low birth weight infants, but concerns regarding the long-term neurodevelopmental prognosis have increased. The study was performed to assess the physical and developmental status of premature infants weighing < 1,000 g at birth who were appropriate for gestational age (AGA)., Methods: A follow-up study including school level, physical and developmental examination and/or interviews of parents, pediatricians, teachers and social workers was carried out from January 1, 1981 to December 31, 1991, on 176 subjects who had been AGA premature neonates weighing < 1,000 g and were admitted to the Port Royal Neonatal Intensive Care Unit. Eighty-nine survived (50 girls, 39 boys). Five children were lost to follow-up after 3 years of age. Neurological sequelae, developmental and/or intellectual quotients, school level and results, vision, hearing, language, weight, height, and behavior were evaluated., Results: At the last evaluation, 37% of children were completely normal (24% had always been normal); 52% were normal in the 4th year of life, decreasing to 31% in the 8th year. Minor disabilities increased from 31% in the 4th year to 53% in the 8th year. Moderate disabilities ranged yearly between 8 and 14%. Five children had major handicaps diagnosed before 3 years of age (4 cerebral palsies following severe neonatal brain lesions and 1 severe retardation with renal dwarfism). No difference was found between children with or without bronchopulmonary dysplasia. Twelve children (14%) had hearing loss (3 deafness) and 27 (32%) had visual deficits (no blindness). Nursery school begins at 3 for French children: 86% of the followed survivors were at nursery school at 3 years of age, 74% of 6-year-olds were in the first grade, primary school, and 50% of 7-year-olds were in second grade. Half the children with high socioeconomic/cultural risks had some school difficulties. Similar results were collected from various cross-sectional studies in the recent literature., Conclusions: While 20% of the children had moderate to severe disabilities, an increasing number of minor deficiencies appeared with age. Socio-economic/cultural factors had a deleterious effect even in structurally normal children, but they had an even worse effect in children with minor deficiencies, owing largely to the frequent lack of proper management at home.

The new radiological technics show the incidence of intraventricular hemorrhage (IVH), intraparenchymal hemorrhage (IPH), or of porencephaly (PL), in the neonatal period. Discrepancies between the initial extent of lesions and the outcome have been observed. We tried to appreciate the EEG value in infants having had such lesions during the first month of life. We studied serial EEGs and neuroradiological exams in 34 babies: with IVH (group I, 17 cases), with IPH and/or PL (group II, 17 cases). Their gestational age (GA) was between 27 and 34 weeks; they all had a neurological follow-up between one and five years. Infants with favourable outcome (76% in gr. I, 47% in gr. II) had normal or slightly abnormal EEGs, whatever the extent of lesions. Nine babies had very abnormal EEGs (numerous positive rolandic spikes (PRSs) and/or EEG background without physiological rhythms); one developed moderate sequelae, the other 8 major sequelae (2 with infantile spasms). In gr. II, babies with major sequelae had a higher GA than babies with good outcome. The PRSs were more often observed with periventricular lesions than with IVH only and in babies with higher GA; they lasted up to 12 weeks on serial EEGs in one case with porencephaly (major sequelae with infantile spasms); they appeared before scan abnormality in another case which developed later porencephaly (major sequelae). Thus, EEGs give valuable information on brain function and help to forecast outcome in premature babies.