Neuropathologic and molecular data of study subjects.

<p>PRNP = prion protein gene, M = methionine, V = valine, PSPr = protease sensitive proteinopathy.</p>a<p>It was unknown whether tissue was from an autopsy or biopsy in 3 cases.</p>b<p>Any White race vs. any non-White race (Fisher’s exact test, 2-sided, p = 0.02).</p>c<p>We could not confirm PRNP codon 129 genotype in 2 cases.</p>d<p>Defined as PRNP codon 129 genotype and prion protein type(s).</p

Time to correct diagnosis of sporadic Creutzfeldt-Jakob disease subjects by race/ethnicity.

<p>White race includes non-Hispanic and Hispanic Whites (Mantel-Cox, chi-square  = 7.1, p = 0.008).</p

Clinical symptoms of subjects throughout the disease course.

<p><u>Cognitive</u>: cognitive decline, executive dysfunction, amnesia, agnosia, apraxia, alexia, language impairment, disorientation, and/or concentration impairment; <u>Cerebellar</u>: ataxia, nystagmus, and/or vertigo; <u>Movement disorder</u>: tremor, chorea, and/or extrapyramidal symptoms (does not include myoclonus); <u>Visual</u>: diplopia, oculomotor palsy, hemianopia, cortical blindness, visuospatial impairment, and/or visual hallucinations; <u>Mood</u>: depression, anxiety, mania, hypomania, emotional lability, and/or apathy; <u>Psychosis</u>: delusions, hallucinations, and/or psychosis not otherwise specified.</p

Brain magnetic resonance imaging (MRI) characteristics of study subjects.

<p>Brain MRI was not done for 2 subjects and results were unknown in 4 subjects.</p>a<p>imes could not be calculated for 7 subjects.</p>b<p>p<0.05.</p>c<p>13/15 cases also had basal ganglia hyperintensity.</p>d<p>Hyperintensity on DWI/FLAIR in 2 or more cortical areas (temporal, parietal, or occipital lobes) <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0038884#pone.0038884-Zerr1" target="_blank">[15]</a>.</p>e<p>High signal abnormalities in caudate nucleus and putamen or at least two cortical regions (temporal-parietal-occipital) either in DWI or FLAIR <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0038884#pone.0038884-Zerr1" target="_blank">[15]</a>.</p

Diagnostic testing data of study sample.

<p>EEG = electroencephalogram; PSWC  =  periodic sharp wave complexes.</p>a<p>Unknown in 9 cases.</p>b<p>Unknown in 8 cases.</p>c<p>Unknown in 4 cases.</p