PMID- 24768314 OWN - NLM STAT- MEDLINE DCOM- 20141230 LR - 20211021 IS - 1878-5883 (Electronic) IS - 0022-510X (Print) IS - 0022-510X (Linking) VI - 341 IP - 1-2 DP - 2014 Jun 15 TI - Tractography reveals diffuse white matter abnormalities in Myotonic Dystrophy Type 1. PG - 73-8 LID - S0022-510X(14)00226-3 [pii] LID - 10.1016/j.jns.2014.04.005 [doi] AB - Cerebral involvement in Myotonic Dystrophy Type 1 (DM1) is well-established but not well characterized. This study applied new Diffusion Tensor Imaging (DTI) tractography to characterize white matter disturbance in adults with DM1. Forty-five participants with DM1 and 44 control participants had MRIs on a Siemens 3T TIM Trio scanner. Data were processed with TRActs Constrained by UnderLying Anatomy (TRACULA) and 7 tracts were evaluated. Bilateral disturbances in white matter integrity were seen in all tracts in participants with DM1 compared to controls. There were no right-left hemisphere differences. The resulting DTI metrics were correlated with cognitive functioning, particularly working memory and processing speed. Motor speed was not significantly correlated with white matter microstructural integrity and, thus, was not the core explanation for the working memory and processing speed findings. White matter integrity was correlated with important clinical variables including the muscular impairment rating scale (MIRS). CTG repeat length was moderately associated with white matter status in corticospinal tract and cingulum. Sleepiness (Epworth Sleepiness Scale) was moderately associated with white matter status in the superior longitudinal fasciculus and cingulum. Overall, the results add to an emerging literature showing widespread white matter disturbances in both early-onset and adult-onset DM1. Results suggest that further investigation of white matter pathology is warranted in DM1 and that non-invasive measures such as DTI have a potentially important clinical value in characterizing the status of individuals with DM1. CI - Copyright (c) 2014 Elsevier B.V. All rights reserved. FAU - Wozniak, Jeffrey R AU - Wozniak JR AD - Department of Psychiatry, University of Minnesota-Twin Cities, F256/2B West, 2450 Riverside Ave., Minneapolis, MN 55454, United States. Electronic address: jwozniak@umn.edu. FAU - Mueller, Bryon A AU - Mueller BA AD - Department of Psychiatry, University of Minnesota-Twin Cities, F256/2B West, 2450 Riverside Ave., Minneapolis, MN 55454, United States. FAU - Lim, Kelvin O AU - Lim KO AD - Department of Psychiatry, University of Minnesota-Twin Cities, F256/2B West, 2450 Riverside Ave., Minneapolis, MN 55454, United States. FAU - Hemmy, Laura S AU - Hemmy LS AD - Department of Psychiatry, University of Minnesota-Twin Cities, F256/2B West, 2450 Riverside Ave., Minneapolis, MN 55454, United States. FAU - Day, John W AU - Day JW AD - Department of Neurology, Stanford University, 300 Pasteur Drive, Room A301, MC 5325, Stanford, CA 94305, United States. LA - eng GR - P41 EB015894/EB/NIBIB NIH HHS/United States GR - P41 RR008079/RR/NCRR NIH HHS/United States GR - R01 NS056592/NS/NINDS NIH HHS/United States GR - P30 NS076408/NS/NINDS NIH HHS/United States GR - P30 NS057091/NS/NINDS NIH HHS/United States GR - P01 NS058901/NS/NINDS NIH HHS/United States PT - Journal Article DEP - 20140413 PL - Netherlands TA - J Neurol Sci JT - Journal of the neurological sciences JID - 0375403 SB - IM MH - Adult MH - *Diffusion Tensor Imaging MH - Female MH - Humans MH - Image Processing, Computer-Assisted MH - Leukoencephalopathies/*diagnosis/*etiology MH - Male MH - Middle Aged MH - Myotonic Dystrophy/*complications MH - Neuropsychological Tests PMC - PMC4042407 MID - NIHMS585635 OTO - NOTNLM OT - Brain OT - Diffusion Tensor Imaging OT - MRI OT - Myotonic Dystrophy OT - Neuropsychology OT - White matter EDAT- 2014/04/29 06:00 MHDA- 2014/12/31 06:00 PMCR- 2015/06/15 CRDT- 2014/04/29 06:00 PHST- 2014/01/31 00:00 [received] PHST- 2014/04/03 00:00 [revised] PHST- 2014/04/05 00:00 [accepted] PHST- 2014/04/29 06:00 [entrez] PHST- 2014/04/29 06:00 [pubmed] PHST- 2014/12/31 06:00 [medline] PHST- 2015/06/15 00:00 [pmc-release] AID - S0022-510X(14)00226-3 [pii] AID - 10.1016/j.jns.2014.04.005 [doi] PST - ppublish SO - J Neurol Sci. 2014 Jun 15;341(1-2):73-8. doi: 10.1016/j.jns.2014.04.005. Epub 2014 Apr 13.