PMID- 32936994 OWN - NLM STAT- MEDLINE DCOM- 20210714 LR - 20210714 IS - 1552-6569 (Electronic) IS - 1051-2284 (Linking) VI - 31 IP - 1 DP - 2021 Jan TI - Cognitive Decline and White Matter Integrity Degradation in Myotonic Dystrophy Type I. PG - 192-198 LID - 10.1111/jon.12786 [doi] AB - BACKGROUND AND PURPOSE: Myotonic Dystrophy Type I (DM1) is a neurodegenerative, genetic, and multisystemic disorder with a large variety of symptoms due to a CTG trinucleotide expansion located on Dystrophia Myotonica Protein Kinase (DMPK) gene. Previous reports have shown cognitive deterioration in these patients. Given that white matter (WM) degradation has also been reported in DM1 patients, here we explored if alterations in the cognitive profile of DM1 patients could be related to the deterioration of WM. METHODS: A total of 22 classic DM1 patients with age range (18-56 years) and 22 matched healthy control subjects were neuropsychological evaluated by the Cambridge Neuropsychological Test Automated (CANTAB). Patients were evaluated with the Muscular Impairment Rating Scale (MIRS). We then evaluated the cerebral WM integrity using the Fractional Anisotropy (FA) index obtained from the Diffusion Tensor Imaging (DTI) data acquired with a 3T MR scanner. RESULTS: DM1 patients showed generalized reduction of WM integrity across the brain. Similarly, patients' neuropsychological evaluation showed significant deficits in memory and problem-solving tasks. Correlation analyses showed a significant correlation between FA deterioration at frontal, temporomedial, and parietal lobes and delayed matched to sample deficits. CONCLUSIONS: Our results suggest that despite the pervasive WM integrity loss in DM1 disorder, specific memory impairments can be associated to discreet areas of WM deterioration in these patients. CI - (c) 2020 American Society of Neuroimaging. FAU - Lopez-Titla, Maria Margarita AU - Lopez-Titla MM AUID- ORCID: 0000-0001-9359-0772 AD - Instituto de Neuroetologia, Universidad Veracruzana, Xalapa, 91190, Mexico. FAU - Chirino, Amanda AU - Chirino A AD - Departamento de Fisiologia, Facultad de Medicina, Universidad Nacional Autonoma de Mexico, Ciudad de Mexico, 04510, Mexico. FAU - Cruz Solis, Sara Vanessa AU - Cruz Solis SV AD - Departamento de Fisiologia, Facultad de Medicina, Universidad Nacional Autonoma de Mexico, Ciudad de Mexico, 04510, Mexico. FAU - Hernandez-Castillo, Carlos R AU - Hernandez-Castillo CR AUID- ORCID: 0000-0003-1208-5577 AD - CONACYT-Instituto de Neuroetologia, Universidad Veracruzana, Xalapa, 91190, Mexico. FAU - Diaz, Rosalinda AU - Diaz R AD - Departamento de Fisiologia, Facultad de Medicina, Universidad Nacional Autonoma de Mexico, Ciudad de Mexico, 04510, Mexico. FAU - Marquez-Quiroz, Luz Del Carmen AU - Marquez-Quiroz LDC AD - Laboratorio de Medicina Genomica, Departamento de Genetica (CENIAQ), Instituto Nacional de Rehabilitacion-Luis Guillermo Ibarra Ibarra (INR-LGII), Ciudad de Mexico, 14389, Mexico. FAU - Magana, Jonathan J AU - Magana JJ AUID- ORCID: 0000-0003-4399-4618 AD - Laboratorio de Medicina Genomica, Departamento de Genetica (CENIAQ), Instituto Nacional de Rehabilitacion-Luis Guillermo Ibarra Ibarra (INR-LGII), Ciudad de Mexico, 14389, Mexico. AD - Escuela de Ingenieria, Departamento de Biotecnologia, Instituto Tecnologico y de Estudios Superiores de Monterrey-Campus, Ciudad de Mexico, 14380, Mexico. FAU - Beltran-Parrazal, Luis AU - Beltran-Parrazal L AUID- ORCID: 0000-0002-4765-9051 AD - Centro de Investigaciones Cerebrales, Universidad Veracruzana, Xalapa, 91190, Mexico. FAU - Fernandez-Ruiz, Juan AU - Fernandez-Ruiz J AUID- ORCID: 0000-0002-4038-0904 AD - Departamento de Fisiologia, Facultad de Medicina, Universidad Nacional Autonoma de Mexico, Ciudad de Mexico, 04510, Mexico. AD - Facultad de Psicologia, Universidad Veracruzana, Xalapa, 91700, Mexico. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200916 PL - United States TA - J Neuroimaging JT - Journal of neuroimaging : official journal of the American Society of Neuroimaging JID - 9102705 SB - IM MH - Adolescent MH - Adult MH - Anisotropy MH - Cognitive Dysfunction/*complications MH - Diffusion Tensor Imaging MH - Humans MH - Male MH - Memory MH - Middle Aged MH - Myotonic Dystrophy/complications/diagnostic imaging/*pathology/*physiopathology MH - Neuropsychological Tests MH - White Matter/diagnostic imaging/*pathology/physiopathology MH - Young Adult OTO - NOTNLM OT - CANTAB OT - DM1 OT - DTI OT - MRI OT - memory EDAT- 2020/09/17 06:00 MHDA- 2021/07/15 06:00 CRDT- 2020/09/16 17:13 PHST- 2020/07/02 00:00 [received] PHST- 2020/08/29 00:00 [accepted] PHST- 2020/09/17 06:00 [pubmed] PHST- 2021/07/15 06:00 [medline] PHST- 2020/09/16 17:13 [entrez] AID - 10.1111/jon.12786 [doi] PST - ppublish SO - J Neuroimaging. 2021 Jan;31(1):192-198. doi: 10.1111/jon.12786. Epub 2020 Sep 16.