The case reported a progressive neurological condition (headache that progress to dementia) with early onset of cognitive impairment and behavioral symptoms at the age of 47 years, a Brain-MRI showing moderate to severe cortico-subcortical trophic changes, and basal nuclei hyperintensity, added to a positive Western-Blot quantification of CSF 14-3-3 protein (sensitivity of 94% and specificity of 84%),5,8 hence the condition was classified as spongiform encephalopathy, probable CJD. The gene discussed is YWHAQ; the disease is prion disease.