Figure 3 Changing appearance of the frontal cortex with age associated with increasing myelination Changing appearance of the frontal cortex with age associated.

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Figure Pedigrees of the SCA42 families identified in this study

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Figure 3 Changing appearance of the frontal cortex with age associated with increasing myelination Changing appearance of the frontal cortex with age associated with increasing myelination Representative images from 2 patients: Im11 p.Gln86Profs*35 (A–D) and Im09 p.Glu154Glyfs*301 (E–H). (A and B) Images obtained when the patient was 6 months old. T2-weighted image (A) shows normal thickness of both frontal lobes but delayed myelination. T1-weighted image (B) shows a pachygyric cortex in the same region. (C and D) Images obtained when the patient was 2 years 6 months. T2-weighted image (C) of the frontal lobe shows mildly thickened cortex, probably because of the poor myelination of the subcortical white matter. (E and F) Images obtained when the patient was 1 year 8 months. In the frontal lobe, T2-weighted (E) and T1-weighted (F) images show the same pattern of pachygyric cortex and severely delayed myelination (E). (G and H) At 3 years, the T2-weighted image (G) shows significant improvement of myelination, although still delayed in the frontal subcortical region: the T1-weighted image (H) shows mildly simplified gyral pattern, with no pachygyria. Nancy Vegas et al. Neurol Genet 2018;4:e281 Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.