Association of Deep Gray Matter Damage With Cortical and XXXX in Primary Progressive @entity15 .

@entity1 @entity75 @entity16 @entity602 @entity194 @entity19383 @entity15
IMPORTANCE: The investigation of cortical gray matter (GM), deep GM nuclei, and @entity602 in @entity1 with @entity19383 ( @entity19383 ) provides insights into the neurodegenerative process responsible for clinical progression of @entity15 . OBJECTIVE: To investigate the association of magnetic resonance imaging measures of cortical, deep GM, and @entity602 and their effect on clinical disability. DESIGN, SETTING, AND @entity1 : Cross-sectional analysis of 26 @entity1 with @entity19383 (mean age, 50.9 years; range, 31-65 years; including 14 @entity1 ) and 20 healthy control @entity1 (mean age, 51.1 years; range, 34-63 years; including 11 @entity1 ) enrolled at a single US institution. Clinical disability was measured with the Expanded Disability Status Scale, 9-Hole Peg Test, and 25-Foot Walking Test. We collected data from January 1, 2012, through December 31, 2013. Data analysis was performed from January 21 to April 10, 2015. MAIN OUTCOMES AND MEASURES: Cortical lesion burden, @entity75 , spinal cord area and volume, and scores on the Expanded Disability Status Scale (score range, 0 to 10; higher scores indicate greater disability), 9-Hole Peg Test (measured in seconds; longer performance time indicates greater disability), and 25-Foot Walking Test (test covers 7.5 m; measured in seconds; longer performance time indicates greater disability). RESULTS: The 26 @entity1 with @entity19383 showed significantly smaller mean (SD) brain and spinal cord volumes than the 20 control group @entity1 (normalized brain volume, 1377.81 [65.48] vs 1434.06 [53.67] cm3 [P = .003]; normalized white matter volume, 650.61 [46.38] vs 676.75 [37.02] cm3 [P = .045]; normalized gray matter volume, 727.20 [40.74] vs 757.31 [38.95] cm3 [P = .02]; normalized neocortical volume, 567.88 [85.55] vs 645.00 [42.84] cm3 [P =   .001]; normalized spinal cord volume for C2-C5, 72.71 [7.89] vs 82.70 [7.83] mm3 [P < .001]; and normalized spinal cord volume for C2-C3, 64.86 [7.78] vs 72.26 [7.79] mm3 [P =.002]). The amount of @entity75 , especially with respect to the thalamus, was correlated with the number and volume of @entity75 (mean [SD] thalamus volume, 8.89 [1.10] cm3; cortical lesion number, 12.6 [11.7]; cortical lesion volume, 0.65 [0.58] cm3; r = -0.52; P < .01). Thalamic @entity194 also showed an association with cortical lesion count in the frontal cortex (mean [SD] thalamus volume, 8.89 [1.1] cm3; cortical lesion count in the frontal lobe, 5.0 [5.7]; r = -0.60; P < .01). No association was identified between magnetic resonance imaging measures of the brain and @entity602 . CONCLUSIONS AND RELEVANCE: In this study, the neurodegenerative process occurring in @entity19383 appeared to spread across connected structures in the brain while proceeding independently in the spinal cord. These results support the relevance of anatomical connectivity for the propagation of @entity16 in the PP phenotype.
