Jeong-Won Jeong1,2, Senthil Sundaram1,2,
Benjamin Wilson1,2, Harry T. Chugani, 2,3
1Pediatrics and Neurology, Wayne State University, Detroit,
MI, United States; 2PET center, Children's Hospital of Michigan,
Detroit, MI, United States; 3Pediatrics, Neurology, and Radiology, Wayne State
University, Detroit, MI, United States
Angelman
syndrome (AS) is a genetic disorder characterized by mental retardation,
speech impairment, and gait apraxia. Speech impairment is universal but
severity differs, which can be characterized by myelination delay or deficits
of white matter associated with language production and conception,
especially of arcuate fasciculus (AF) bridging Brocas and Wernickes
areas. This study presents new DT-MRI methodology to identify aberrant shapes
of arcuate fibers and quantify abnormal tracts in terms of their geometry. We
found that the AF of AS patients have steeper lateral-curvatures cauisng them
not to reach Wernicke area and also their FA values were significantly
reduced.
Keywords