Abstract #4263
Assessing The Level Of Pathology Of The Corticospinal Pathway In Patients With PLP1 Mutations Using Diffusion Tensor Imaging.
Malek I Makki 1 and Jeremy J Laukka 2
1
MRI Research, University Children Hospital
of Zurich, Zurich, Switzerland,
2
Department
of Neuroscience and Neurology, University of Toledo,
Toledo, Ohio, United States
Pelizaeus-Merzbacher disease (PMD) is an X-linked
disorder of the central nervous system, caused by
mutations affecting proteolipid protein 1 (PLP1) the
major protein in myelin. Twelve PMD patients were
examined with DTI. Mutation was characterized as null
mutation (N=3), moderate (N=5) and severe (N=4). We
selected three levels along the cortico-spinal tract:
posterior-limb of the internal capsule, pons and
cerebral peduncle. In the pons we observed significantly
higher radial diffusion both in severe compared to null
(p=0.002) and in severe compared to moderate mutation
(p=0.015). We observed higher axial diffusion in the
posterior-limb in severe compared to null mutation
(p=0.008).
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