Creutzfeldt-Jakob disease is a prion disease clinically characterised by rapidly progressing dementia, cerebellar and extrapyramidal signs, and myoclonus. Cerebral imaging procedures are considered to be of little value for definite premortem diagnosis, which still depends on brain biopsy. Corresponding to cognitive deficits neuropathological changes mostly affect the cerebral cortex, and less severely other grey matter areas such as the caudate, putamen, and thalamus.1 The following case report suggests that MRI using a fluid attenuated inversion recovery (FLAIR) sequence might detect pathological changes in the cerebral cortex.

A 45 year old man presented with a three month history of progressive memory deficit, listlessness, and loss of speech. He repeatedly lost his orientation in the forest where he had worked as a wood cutter for many years. His history was remarkable for bulbectomy of the right eye at the age of 15 months, probably due to retinoblastoma. On examination he complied with simple requests, only. He spoke very little with multiple perseverations. Severe deficits of memory and orientation were obvious. Pronounced irritability with bursts of aggressiveness made neuroleptic therapy necessary. Deep tendon reflexes were brisk but plantar responses were flexor. Rigidity affecting all limbs and hypomimia indicated involvement of …