Child Neurology; Children and Adults; Developmental Neurology; Neurology; Pediatrics
My research was focused originally on using astrocyte cell cultures to shed light upon processes related to astro-gliosis, the key response of developing and mature brain to injury. The laboratory succeeded in modeling many immunochemical features of gliosis in primary brain cell culture. More recently, I have been involved in the clinical care of, and research regarding, children with leukodystrophies. This includes coordinating several clinical research projects in roles as Clinical Director of the Hunter James Kelly Research Institute (HJKRI), and as President of the New York State Krabbe Disease Consortium. Projects include expansion of the World Wide Registry for Krabbe Disease (WWR), a database maintained at HJKRI that now has clinical and genetic information for over 150 affected patients afflicted with this rare disease, and examining ways to involve the WWR in national data-sharing initiatives promoted by the NIH, including the Newborn Screening Translational Research Network (NBSTRN). HJKRI is also conducting long-term follow-up studies of affected children, in conjunction with the Rare Clinical Disease Research Network (RDCRN) examining neuro-developmental parameters before and after therapeutic transplantation. Additional projects include exploration of the genotype/phenotype relationship in Krabbe, including exome and whole genome analyses with collaborators, and participating in research to uncover new biomarkers and to develop novel therapies. I am also the Director of the Headache and Concussion clinics at Children’s Hospital of Buffalo. These clinical leadership roles in Western New York and New York State can facilitate significant recruitment into multi-center research studies.
Developmental Neurology; Neurology
My laboratory has a longstanding interest in myelin and its diseases. Myelin surrounds large axons and permits rapid conduction of signals. It is formed by oligodendrocytes in the central nervous system, and Schwann cells in the peripheral nervous system. During development, these cells migrate with the axons that they will myelinate, and depend on those same axons for appropriate signals to survive and differentiate. Myelin-forming glia coordinately express a unique set of genes encoding myelin structural proteins, and enzymes that synthesize myelin lipids-this coordination is in large part transcriptionally-mediated. Given the unique three dimensional transformation of the cell required for myelination, many of the involved proteins include adhesion among their functions. Therefore, our projects include studies of transcriptional regulation, axonal signals to myelinating glia, the role of adhesion in myelination and the characterization of animal models of human demyelinating diseases.
Neurology; Neuroradiology - Radiology; Vascular and Interventional Radiology; Parkinson's; Multiple Sclerosis; Alzheimer Disease / Memory Disorders; Developmental Neurology; General Neurology; Neurodegenerative disorders; Neuroimaging
I direct the Buffalo Neuroimaging Analysis Center (BNAC) and have established the center as a world leader in performing quantitative MRI analysis in neurodegenerative disorders. I also direct the Translational Imaging Center at UB’s Clinical Translational Research Center (CTRC). I strive to extend the boundaries of current knowledge about neurological diseases and disorders through innovative imaging research techniques and the application of bioinformatics resources. My efforts are directed toward advancing technical, basic and translational research at UB which will, in turn, advance patient care. I have secured more than $30 million in research grants for collaborative research projects involving UB investigators as well as national and international collaborators. My research interests include structural and functional quantitative MRI analysis for humans and animals, including lesion/tumor identification and segmentation; perfusion and dynamic contrast-enhanced (DCE) mapping and quantification; fluid flow quantification; functional MRI analysis; diffusion tensor reconstruction and tractography; voxel-wise mapping and image-based group statistical analysis; longitudinal change analysis and tissue/pathology/structure volumetry. I study the application of these techniques in healthy individuals and in patients with various disease states such as multiple sclerosis (MS), stroke, Alzheimer’s disease, Parkinson’s disease, epilepsy, systemic lupus erythematosus and traumatic brain injury. I also concentrate on therapeutic interventions, including therapy directed toward assessing neuroprotective efforts in neurodegenerative disorders as well as the venous function, genetic and neuroepidemiology fields of these diseases. I direct the neurology resident research program. Over a period of two years, I guide third- and fourth-year medical residents through a rigorous assigned scientific research project that is a critical, required part of their training. In addition, I mentor and supervise undergraduate, master’s and doctoral students and MRI fellows. In this role, I help to educate these trainees on clinical MRI use as well as neuroimaging analysis. I also oversee students and fellows conducting research in neurological disorders. One of the most rewarding experiences in my career is helping young physicians and researchers start successful clinical or research careers.