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ndrd@chp.edu
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Children's Hospital of Pittsburgh of UPMC
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You are here: Home > Research > Krabbe Neuroimaging (DTI)

NDRD Neuroimaging Studies

DTI as a Tool to Detect Neurodegenerative Disease

With the support of the NIH (NIH R01NS061965) we have developed a method to use information from magnetic resonance imaging (MRI) to quantify neurological damage in the brain. Using data from over 600 patient MRI scans we have created a methodology to measure the amount of disease progression in the brain over time.

Diffusion tensor imaging (DTI) is a type of magnetic resonance imaging (MRI) scan that can produce three-dimensional images of the brain. DTI is a safe noninvasive test that does not require the use of contrast agents or chemical tracers. The purpose of this research study is to better understand early brain development in children with neurodegenerative disease. This technique can provide information about a child's potential learning and motor development and help us predict outcomes after treatment.


Longitudinal study of typical brain development

To understand neurodegenerative disease progression in the brain we must have a clear understanding of normal brain development using the same tools as those with which we measure the disease.The goal of this study is to describe normal brain development in children from birth to 17 years of age to be used as a comparison (control) for studying children with neurodegenerative disease.

These images of typical brain development will be used for:

  1. Early identification of disease progression in children who screen positive for a neurodegenerative disease. These diseases have many different forms that affect when a child starts to show symptoms, how fast it progresses, and whether the brain will be affected. Often doctors cannot tell how exactly the disease will progress. Having images from typically developing children will allow direct comparisons with patients’ brains to determine the progression of the disease. This information can also be used to determine the degree of disease progression (as compared to normal) to allow parents and clinicians make better decisions about potential risks versus benefits of treatment options for an individual child. It will also help understand if a new therapy is improving the brain disease.

    One example is Hunter’s syndrome (MPS II) which has 2 forms of the disease with one affecting the brain and one is generally somatic. The children with the cerebral form must be identified early to begin treatment with intrathecal enzyme or hematopoietic stem cell transplant. It has been shown that early treatment is the one of the strongest predictors of long-term outcomes. Currently it is difficult to know which of the babies who test positive for MPS II will have the cerebral form making treatment before symptom onset unlikely. Therefore, a tool is needed to identify the babies that will have the cerebral form of this disease and those who will not as early in the neonatal period as possible.

  2. Evaluation of current and new treatments. Since these diseases affect the brain, a direct measure of brain damage can make evaluating the effects of a treatment faster and easier. By comparing the brain development of the treated patients to both untreated and typically developing children we can evaluate the differences to see how the treatment is helping and any residual problems that the treatment may not solve.
  3. These images will also be used to study more common neurologic diseases such as cerebral palsy, … The data we collect will be shared with other scientists who are interested in brain development across many neurologic diseases.
KTRN Group Photo

Legacy of Angels LogoSiemens Skyra 3-tesla MRI scanner, generously donated to Children's Hospital of Pittsburgh by The Legacy of Angels Foundation to promote neuroimaging research.


Publications:

DTI registration in atlas based fiber analysis of infantile Krabbe disease. Wang, Y., Gupta, A., Liu, Z., Zhang, H., Escolar, M. L., Gilmore, J. H., et al. (2011). Neuroimage, 55(4), 1577-1586. http://www.ncbi.nlm.nih.gov/pmc/articles/pmc3062693/

Regional differences in fiber tractography predict neurodevelopmental outcomes in neonates with infantile Krabbe disease Gupta A, Poe MD, Styner MA, Panigrahy a, Escolar ML. Neuroimage Clin. 2014 Sep 26;7:792-8 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4375637/

Diffusion tensor imaging detects abnormalities in the corticospinal tracts of neonates with infantile Krabbe disease. Escolar, M. L., Poe, M. D., Smith, J. K., Gilmore, J. H., Kurtzberg, J., Lin, W., et al. (2009). AJNR Am J Neuroradiol, 30(5), 1017-1021. http://www.ajnr.org/content/30/5/1017.long

Thickening of the Cauda Equina Roots: a Common Finding in Krabbe Disease Zuccoli G, Narayanan S, Panigrahy A, Poe MD, Escolar ML. Eur Radiol. 2016 May 2:1-6 https://www.ncbi.nlm.nih.gov/pubmed/?term=27137647

Midbrain morphology reflects extent of brain damage in Krabbe disease Zuccoli G, Narayanan S, Panigrahy A, Poe MD, Escolar ML. Neuroradiology, 2015 Jul;57(7):739-45 https://www.ncbi.nlm.nih.gov/pubmed/?term=25859833