Background Rapidly progressive dementia can be caused by several factors like neurodegenerative, toxic/metabolic, infectious, autoimmune or neoplastic disorders. Here I present a case of this type and the diagnostic process leading to diagnosis. Material and methods This case describes a woman in her sixties admitted to hospital with dementia and anxiety as initial symptoWithin 2 month progression of these symptoms and appearance of apraxia, visual affection and cerebellar gait. The diagnostic process is illustrated. including consecutive EEGs, PET, MRi, CSF markers. Results Initial MRi was considered normal and first EEG with normal posterior dominant rhythm and bi-fronto-temporal 2–3 Hz activity (leftsided dominance) and no paroxystic activity. Repeated tests revealed classic changes seen in sporadic CJD on MRi in the form of cortical ribbon sign and adynamic periodic paroxyms and low frequency activity (2–3 Hz) lateralized in the EEG. CSF with normal count of protein and normal count of White blood cells and elevated count of total tau 1200 (normal range Conclusions Even though a definite diagnosis of Creutzfeldt-Jacob disease (CJD) can only be manifest by brain autopsy or biopsy post mortem a possible or likely diagnosis can be established combining CSF markers, brain imaging and EEG. This case illustrates the importance of EEG and especially consecutive EEGs in the diagnostic process of rapidly progressive dementia.
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