This chapter talks about a 39-year-old woman who was reported to the "German National Reference Center for the Surveillance of Transmissible Spongiform Encephalopathies" (NRC). The patient's medical history comprised allergic asthma, hyperlipidemia, nasal sinus surgery, and pulmonary embolism. The family's medical history comprised allergies, asthma, and psoriasis, cerebrovascular disease, colon cancer, but no early onset dementia. The combination of rapidly progressive dementia, psychosyndrome, ataxia, visual disturbances, and the Pulvinar Sign revealed by MRI as well as the exclusion of encephalitis or many other possible causes of that symptom constellation made the involved physicians consider a prion disease, namely variant Creutzfeldt-Jakob disease, as a differential diagnosis. Rapidly progressive dementia, the "Hockey Sticks" and the exclusion of encephalitis led to the diagnosis of variant Creutzfeldt-Jakob disease (vCJD) in the first place. Wernicke's disease was not as strongly considered initially since the patient was non-alcoholic.