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Sumit N Niogi

Publications and source records attributed to Sumit N Niogi.

3 recordsLinked to original sources

Diffusion tensor imaging segmentation of white matter structures using a Reproducible Objective Quantification Scheme (ROQS).

Reproducible Objective Quantification Scheme (ROQS) is a novel method for regional white matter measurements of diffusion tensor imaging (DTI) parameters that overcomes the limitations of previous approaches for analyzing large cohorts of subjects reliably. ROQS is a semi-automated technique that exploits the fiber orientation information from the diffusion tensor in conjunction with a binary masking and chain-linking algorithm to segment anatomically distinct white matter tracts for subsequent quantitative analysis of DTI parameters such as fractional anisotropy and apparent diffusion coefficient. When applied to 3-T whole-brain DTI of normal adult volunteers, ROQS is shown to segment the corpus callosum much faster than manual region of interest (ROI) delineation, and with better reproducibility and accuracy.

Adult↗

Left lateralized white matter microstructure accounts for individual differences in reading ability and disability.

Diffusion tensor imaging (DTI) was used to investigate the association between variation in white matter microstructure and individual differences in reading skill within children. Unlike previous DTI studies of reading, our sample examined children in both the average reading range as well as several children in the performance range of reading disability (RD). Results replicate previous findings of a strong correlation between fractional anisotropy (FA) values in a left temporo-parietal white matter region and standardized reading scores of typically developing children. Furthermore, FA values in this same region accounted for differences between children scoring in the average range and children scoring in the RD range, suggesting that the role of white matter tract microstructure is best characterized as an extreme range on a continuum of typical variation. Furthermore, significant correlations between working memory and frontal white matter tract regions were present in this same population, yet were demonstrated to be independent of the relationships found between reading and more posterior regions. Results form a "correlational double dissociation" that demonstrates domain specificity in the influence of white matter tract structures to individual differences in cognitive performance.

Adolescent↗

Effective gene therapy for an inherited CNS disease in a large animal model.

Genetic diseases affecting the brain typically have widespread lesions that require global correction. Lysosomal storage diseases are good candidates for central nervous system gene therapy, because active enzyme from genetically corrected cells can be secreted and taken up by surrounding diseased cells, and only small amounts of enzyme (<5% of normal) are required to reverse storage lesions. Injection of gene transfer vectors into multiple sites in the mouse brain has been shown to mediate widespread reversal of storage lesions in several disease models. To study a brain closer in size to the human brain, we evaluated the extent of storage correction mediated by a limited number of adeno-associated virus vector injections in the cat model of human alpha-mannosidosis. The treated cats showed remarkable improvements in clinical neurological signs and in brain myelination assessed by quantitative magnetic resonance imaging. Postmortem examination showed that storage lesions were greatly reduced throughout the brain, even though gene transfer was limited to the areas surrounding the injection tracks. The data demonstrate that widespread improvement of neuropathology in a large mammalian brain can be achieved using multiple injection sites during one operation and suggest that this could be an effective treatment for the central nervous system component of human lysosomal enzyme deficiencies.

Animals↗