Date of download: 5/29/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Abnormal Functional Brain Connectivity and Personality Traits in Myotonic Dystrophy Type 1 JAMA Neurol. 2014;71(5): doi: /jamaneurol Distribution of Elevated Minnesota Multiphasic Personality Inventory-2 (MMPI-2) Scale Scores in Patients With Myotonic Dystrophy Type 1 (DM1)A, The number of DM1 patients with elevated T scores (>65) in the different MMPI-2 Clinical scales. B, The number of DM1 patients with elevated T scores (>65) in the different Harris-Lingoes Clinical subscales. D indicates Depression; Hs, Hypochondriasis; Hy, Hysteria; Ma, Hypomania; Mf, Masculinity/Femininity; Pa, Paranoia; Pd, Psychopathic Deviate; Pt, Psychasthenia; Sc, Schizophrenia; Si, Social Introversion. Harris-Lingoes Clinical subscales include D 1, Subjective Depression; D 2, Psychomotor Retardation; D 3, Physical Malfunctioning; D 4, Mental Dullness; D 5, Brooding; Hy 1, Denial of Social Anxiety; Hy 2, Need for Affection; Hy 3, Lassitude-Malaise; Hy 4, Somatic Complaints; Hy 5, Inhibition of Aggression; Ma 1, Amorality; Ma 2, Psychomotor Acceleration; Ma 3, Imperturbability; Ma 4, Ego Inflation; Pa 1, Persecutory Ideas; Pa 2, Poignancy; Pa 3, Naïveté; Pd 1, Familial Discord; Pd 2, Authority Problems; Pd 3, Social Imperturbability; Pd 4, Social Alienation; Pd 5, Self-alienation; Sc 1, Social Alienation; Sc 2, Emotional Alienation; Sc 3, Lack of Ego Mastery–Cognitive; Sc 4, Lack of Ego Mastery–Conative; Sc 5, Lack of Ego Mastery–Defective Inhibition; and Sc 6, Bizarre Sensory Experiences. Figure Legend:

Date of download: 5/29/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Abnormal Functional Brain Connectivity and Personality Traits in Myotonic Dystrophy Type 1 JAMA Neurol. 2014;71(5): doi: /jamaneurol Conventional Magnetic Resonance Imaging (MRI) of a Patient With Myotonic Dystrophy Type 1 (DM1) Compared With a Healthy ControlMacroscopic abnormalities typically detected in patients with DM1. A, Fluid-attenuated inversion recovery (FLAIR), T2- weighted, and modified driven equilibrium Fourier transformation (MDEFT) images from a representative patient of our cohort. B, A similar set of images obtained from an age- and sex-matched healthy control. Abnormalities include white matter hyperintensities in T2-weighted and FLAIR images, with a predominant distribution to the temporal areas, and signs of diffuse atrophy. R indicates right. Figure Legend:

Date of download: 5/29/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Abnormal Functional Brain Connectivity and Personality Traits in Myotonic Dystrophy Type 1 JAMA Neurol. 2014;71(5): doi: /jamaneurol Pattern of Reduced Functional Connectivity (FC) in Patients With Myotonic Dystrophy Type 1 (DM1)The main effect of default mode network (DMN) FC (blue) across participants (DM1 patients and healthy controls [HS]). In addition, those DMN areas in which DM1 patients, compared with controls, have a significant increase in FC (red) are shown (P <.05, family-wise error [FWE] corrected [Corr] at the cluster level). The scatterplot shows the between-group difference in FC in a cluster located in the posterior cingulate cortex. The results are overlaid on the SPM8 (Statistical Parametric Mapping 8) T1-weighted template in the Montreal Neurological Institute (MNI) coordinates. L indicates left. Figure Legend:

Date of download: 5/29/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Abnormal Functional Brain Connectivity and Personality Traits in Myotonic Dystrophy Type 1 JAMA Neurol. 2014;71(5): doi: /jamaneurol Positive Effect of the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) Code-Type Component (CTc) Score on Default Mode Network (DMN) Functional Connectivity (FC) in Patients With Myotonic Dystrophy Type 1Positive association (P <.05, family- wise error [FWE] corrected [Corr] at cluster level) between DMN FC and the most frequent cluster of personality traits on the MMPI- 2 Clinical scales (Schizophrenia-Paranoia-Hypochondriasis [8-6-1 CTc score]). The results are overlaid on the SPM 8 (Statistical Parametric Mapping 8) T1-weighted template in the Montreal Neurological Institute (MNI) coordinates. L indicates left. Figure Legend:

Date of download: 5/29/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Abnormal Functional Brain Connectivity and Personality Traits in Myotonic Dystrophy Type 1 JAMA Neurol. 2014;71(5): doi: /jamaneurol Negative Effect of the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) Code-Type Component (CTc) Score on Default Mode Network (DMN) Functional Connectivity (FC) in Patients With Myotonic Dystrophy Type 1Negative association (P <.05, family- wise error [FWE] corrected [Corr] at cluster level) between DMN FC and the most frequent cluster of personality traits on the MMPI- 2 Clinical scales (Schizophrenia-Paranoia-Hypochondriasis [8-6-1 CTc score]). The results are overlaid on the SPM 8 (Statistical Parametric Mapping 8) T1-weighted template in the Montreal Neurological Institute (MNI) coordinates. L indicates left. Figure Legend: