PMC3288419_01
PMC3288419_02
Interpersonal Adjectives Scales (IAS): (a) Chart shows 8 personality traits tested organized according to axes denoting the degree of power and affiliation associated with each trait, (b) Mr. X's pre-morbid personality is predominantly agreeable, gregarious and ingenuous with average levels of dominance, (c) Mr. X's personality during the illness reflects a shift to cold-hearted, aloof, and arrogant with unchanged levels of dominance.
nihms298171f1.jpg
PMC3288419_02_nihms298171f1.jpg
F1
His self-report describing his current personality matched his wife's retrospective account (Figure 1).
Voxel-based morphometry analysis (SPM5) of the patient's 1.5T T1-weighted structural MRI scan, compared to 15 male age-matched controls. Due to the reduced power afforded by this single-subject analysis, the threshold has been set at a low, uncorrected level (t-value threshold > 2.5) to better match the degree of atrophy shown on his raw structural MRI scan (also shown).
nihms298171f2.jpg
PMC3288419_02_nihms298171f2.jpg
F2
X's T1-weighted structural MRI scan at initial evaluation with 15 healthy control males matched for age and education (mean age 50.5, range 39-57) (see Table 2, Figure 2, and Supplementary Materials for methods).
Neuropathology. (A-C) Hematoxylin and eosin staining revealed nonspecific signs of neurodegenerative, including microvacuolation and gliosis. These changes were moderate in right medial orbitofrontal cortex (A) and severe in right uncus (B) and CA1/subiculum (C), where they were accompanied by profound neuronal loss (hippocampal sclerosis). Immunohistochemical stains revealed neuronal cytoplasmic and nuclear inclusions that were positive for ubiquitin (D) and FUS (F) but negative for TDP-43 (E). The neuronal nuclear inclusions adopted a "vermiform" shape characteristic of FTLD-FUS inclusions. Scale bars represent 100 mM in A-C, 20 mM in D and E, 20 mM in F (upper panel), and 10 mM in F (lower panels).
nihms298171f3.jpg
PMC3288419_02_nihms298171f3.jpg
F3
Additional immunohistochemical analyses demonstrated that the ubiquitinated inclusions were immunoreactive for FUS (Figure 3) but not for hyperphosphorylated tau (CP-13 antibody), TDP-43, alpha-synuclein, and alpha-internexin.