Susac’s syndrome: a clinical and radiological challenge

We describe a patient with Susac’s syndrome presenting with the triad of encephalopathy, branch retinal artery occlusion and hearing loss. MRI has some characteristic features and can be helpful in establishing the diagnosis, particularly in an early stage before the classic triad is complete. Besides MRI often allows to differentiate this rare disorder from more common neurologic diseases like multiple sclerosis

Preterm cerebral microcirculation assessed with colour doppler: A pilot study

AIM: Pilot study to explore feasibility of a color Doppler technique for monitoring cerebral perfusion at the level of microvessels. METHODS: Between March 1st, 2011, and January 30th, 2013, all admitted infants born before 29 weeks of gestation were eligible for Doppler imaging. Perfusion images were acquired in a standard coronal plane. Image quality was assessed by two authors (MR, PG). The region of interest (ROI) was manually selected. A segmentation tool was developed to separate color data from the greyscale 2D images, leading to a percentage and number of color pixels in the image (Doppler color index; DCI). Intra-and inter-observer agreement was analyzed. RESULTS: Intra-and inter-observer agreement for placement of ROIs was good (bias-0.24 resp.-0.74 percentage points). Color Doppler was able to depict microvessels in cortex, white matter and deep grey matter. The median DCI in a region of cortex-white matter was 7.8% with a wide range (1.4%-25.6%). There was no significant difference between the left and right hemisphere (Mann-Whitney U, P-value 0.61). Clinically relevant observations were tabulated, e.g. distant effect of germinal matrix hemorrhage (GMH) on regional perfusion. CONCLUSION: Sonographic small vessel visualisation may help understand pathogenetic mechanisms related to perfusion and is valuable to monitor effects of treatment

A new ultrasound marker for bedside monitoring of preterm brain growth

BACKGROUNDANDPURPOSE: Preterm neonates are at risk for neurodevelopmental impairment, but reliable, bedside-Available markers to monitor preterm brain growth during hospital stay are still lacking. The aim of this study was to assess the feasibility of corpus callosum-fastigium length as a new cranial sonography marker for monitoring of preterm brain growth. MATERIALS AND METHODS: In this longitudinal prospective cohort study, cranial ultrasound was planned on the day of birth, days 1, 2, 3, and 7 of life; and then weekly until discharge in preterm infants born before 29 weeks of gestational age. Reproducibility and associations between clinical variables and corpus callosum-fastigium growth trajectories were studied. RESULTS: A series of 1- 8 cranial ultrasounds was performed in 140 infants (median gestational age at birth, 27+2 weeks (interquartile range, 26+1 to 28+1; 57.9% male infants). Corpus callosum-fastigium measurements showed good-To-excellent agreement for interand intraobserver reproducibility (intraclass correlation coefficient >0.89). Growth charts for preterm infants between 24 and 32 weeks of gestation were developed. Male sex and birth weight SD score were positively associated with corpus callosum-fastigium growth rate. CONCLUSIONS: Corpus callosum-fastigium length measurement is a new reproducible marker applicable for bedside monitoring of preterm brain growth during neonatal intensive care stay

Early Ultrasonic Monitoring of Brain Growth and Later Neurodevelopmental Outcome in Very Preterm Infants

BACKGROUND AND PURPOSE: In infants born very preterm, monitoring of early brain growth could contribute to prediction of later neurodevelopment. Therefore, our aim was to investigate associations between 2 early cranial ultrasound markers (corpus callosum-fastigium and corpus callosum length) and neurodevelopmental outcome and the added value of both markers in the prediction of neurodevelopmental outcome based on neonatal risk factors and head circumference in very preterm infants. MATERIALS AND METHODS: This prospective observational study included 225 infants born at,30 weeks' gestational age, of whom 153 were without any brain injury on cranial ultrasound. Corpus callosum-fastigium and corpus callosum length and head circumference were measured at birth, 29 weeks' gestational age, transfer from the neonatal intensive care unit to a level II hospital, and 2 months' corrected age. We analyzed associations of brain markers and their growth with cognitive, motor, language, and behavioral outcome at 2 years' corrected age. RESULTS: In infants without brain injury, greater corpus callosum-fastigium length at 2 months was associated with better cognitive outcome. Corpus callosum length at 2 months was positively associated with cognitive, motor, and language outcome. Faster growth of the corpus callosum length between birth and 2 months was associated with better cognitive and motor function. Prediction of neurodevelopmental outcome based on neonatal risk factors with or without head circumference was significantly improved by adding corpus callosum length. CONCLUSIONS: Both corpus callosum-fastigium and corpus callosum length on cranial ultrasound are associated with neurodevelopmental outcome of very preterm infants without brain injury at 2 years, but only corpus callosum length shows the added clinical utility in predicting neurodevelopmental outcome