We report the case of a 56 year old male, presenting approximately 6 weeks after his first symptoms of Sporadic Creutzfeldt-Jakob Disease. The patient demonstrated classical magnetic resonance imaging (MRI) and electroencephalographic (EEG) changes reflecting clinical progression.

Creutzfeldt-Jakob Disease was suspected and later confirmed by positive biochemical markers approximately 10 days after presentation; including positive protein 14-3-3, S-100b and positive rtQUIC assay.

Serial MRI and EEG findings correlated with clinical symptoms witnessed on presentation (nystagmus, mild myoclonus and lability of mood) and disease progression through to coma before death

MRI changes were seen as initial localised cortical ribboning progressing to more widespread ribboning and areas of high signal in the basal ganglia (striatum). This correlated with the findings on his earliest EEG of periodic frontal and right side dominant sharp waves, to the last EEG showing more widespread periodic sharp waves.

MRI and EEG findings are discussed as well as differentials that may appear with similar MRI findings.

The presentation provides an accessible learning case for neurologist, neuro-radiologists and neurophysiologists at all levels.