Variant late-infantile Batten disease is a neuronal ceroid lipofuscinosis caused by mutations in CLN6. It is a recessive genetic lysosomal storage disease characterised by progressive neurodegeneration. It starts insidiously and leads to blindness, epilepsy and dementia in affected children. Sheep that are homozygous for a natural mutation in CLN6 have an ovine form of Batten disease Here, we used in vivo magnetic resonance imaging to track brain changes in 4 unaffected carriers and 6 affected Batten disease sheep. We scanned each sheep 4 times, between 17 and 22 months of age. Cortical atrophy in all sheep was pronounced at the baseline scan in all affected Batten disease sheep. Significant atrophy was also present in other brain regions (caudate, putamen and amygdala). Atrophy continued measurably in all of these regions during the study. Longitudinal MRI in sheep was sensitive enough to measure significant volume changes over the relatively short study period, even in the cortex, where nearly 40% of volume was already lost at the start of the study. Thus longitudinal MRI could be used to study the dynamics of progression of neurodegenerative changes in sheep models of Batten disease, as well as to assess therapeutic efficacy.

As rivers flow toward the coast, freshwater mixes with saltwater in estuaries. The mixing here creates a wide range of environments for many organisms. The Satilla River Estuary has been cut eight times, which has altered the salinity gradients that are a result of natural tidal flow. Altered salinity gradients pose a threat to migratory fish species because they are no longer able to pick up on directional cues that these gradients provide, ultimately affecting species richness in the estuary. The purpose of this study is to determine if Noyes Cut has affected salinity gradients at five collection sites in Umbrella and Dover Creeks. Experimental gill nets were set one hour before max flood tide and soaked for two hours. All fish were identified to species with total and fork lengths measured to the nearest centimeter. Noyes Cut and Parsons Creek had the most species richness while River Marsh Landing and Todd Creek experienced the lowest diversity. We believe these large salinity fluctuations are due to a sediment deposit that blocks water flow as a result of Noyes Cut. When Noyes Cut is closed, we expect fish to redistribute into Umbrella and Dover Creeks as a result of restored salinity gradients.