Our prospective cohort study of extremely low gestational age newborns evaluated the association of neonatal head ultrasound abnormalities with cerebral palsy at age 2 years. Cranial ultrasounds in 1053 infants were read with respect to intraventricular hemorrhage, ventriculomegaly, and echolucency, by multiple sonologists. Standardized neurological examinations classified cerebral palsy, and functional impairment was assessed. Forty-four percent with ventriculomegaly and 52% with echolucency developed cerebral palsy. Compared with no ultrasound abnormalities, children with echolucency were 24 times more likely to have quadriparesis and 29 times more likely to have hemiparesis. Children with ventriculomegaly were 17 times more likely to have quadriparesis or hemiparesis. Forty-three percent of children with cerebral palsy had normal head ultrasound. Focal white matter damage (echolucency) and diffuse damage (late ventriculomegaly) are associated with a high probability of cerebral palsy, especially quadriparesis. Nearly half the cerebral palsy identified at 2 years is not preceded by a neonatal brain ultrasound abnormality. © 2009 Sage PublicationsAddress correspondence to: Karl C. K. Kuban, MD, SM Epi, 1 Boston Medical Center Place, Dowling 3 South, Boston, MA 02118; karl.kuban@bmc.org. Reprints: http://www.sagepub.com/journalsReprints.navThe authors have no conflicts of interest to disclose with regard to this article. Cranial ultrasound studies are used both to identify acute cerebral events in newborns and to assist with prognosis of motor and cognitive dysfunctions. For example, white matter damage, most often identified by a cranial ultrasound abnormality, is the single strongest predictor of cerebral palsy. [1][2][3][4][5][6][7][8][9][10][11] One limitation of many previous prognostic studies of neonatal ultrasound lesions is the reliance on a single sonologist to interpret scans. Because of the inherent variability in interpreting cranial sonograms, multiple readers may increase reliability. 12 Another limitation of previous prognostic studies is the lack of replicable operational definitions of cerebral palsy and its types. [1][2][3][4][5][6][7][8][9][10][11][13][14][15][16][17][18][19] In this article, we report how well cranial ultrasound scans obtained in the neonatal intensive care unit predicted cerebral palsy types and severity of motor dysfunction when children were 2 years old, corrected age. Our study of 1053 children born before the 28th postmenstrual week differs from previous studies in several ways. First, the protocol scans of these children were read by at least 2 independent sonologists for congruence about major abnormalities including intraventricular hemorrhage, moderate/severe ventriculomegaly, echogenic lesion, and echolucent lesion. Second, the sonologists' evaluation included specifically information about the location, extent, and laterality of these lesions. Third, the children were given a standardized neurological examination; the standardization of the examination res...

An 8-month-old girl who seemed certain to die from the infantile form of hypophosphatasia, an inborn error of metabolism characterized by deficient activity of the tissue-nonspecific isoenzyme of alkaline phosphatase (TNSALP), underwent the first trial of bone marrow cell transplantation for this heritable type of rickets. After cytoreduction, she was given T-cell-depleted, haplo-identical marrow from her healthy sister. Chimerism in peripheral blood and bone marrow became 100% donor. Three months later, she was clinically improved, with considerable healing of rickets and generalized skeletal remineralization. However, 6 months post-transplantation, worsening skeletal disease recurred, with partial return of host hematopoiesis. At the age of 21 months, without additional chemotherapy or immunosuppressive treatment, she received a boost of donor marrow cells expanded ex vivo to enrich for stromal cells. Significant, prolonged clinical and radiographic improvement followed soon after. Nevertheless, biochemical features of hypophosphatasia have remained unchanged to date. Skeletal biopsy specimens were not performed. Now, at 6 years of age, she is intelligent and ambulatory but remains small. Among several hypotheses for our patient's survival and progress, the most plausible seems to be the

Background-Neurosonography can assist clinicians and can provide researchers with documentation of brain lesions. Unfortunately, we know little about the reliability of sonographically derived diagnoses.