Four patients could not be classified according to the CDC diagnostic criteria at the time of presentation owing to only one clinical domain involvement apart from dementia at the time of presentation but otherwise had clinical, EEG and imaging characteristics consistent with the diagnosis of CJD with the alternative diagnosis ruled out by relevant diagnostic investigations (autoimmune, paraneoplastic panel, anti-TPO, and CSF analysis were normal). Here, TPO is linked to Creutzfeldt Jacob disease.