CJD is a neurodegenerative disease with a rapid onset characterized by progressive dementia, myoclonus and also cerebellar, pyramidal and extrapyramidal signs. It is caused by an increased loss of neurons due to the abnormal conformation of a membrane protein.

A 69 year-old woman was admitted to Psychiatry with a clinical picture characterized by psychomotor agitation, disorientation, confusion, confabulation, false ricognition, circumstantiality, perseveration, logorrhea and dysphoria. She presented no alterations of sense-perception. Her psychiatric history was silent. The neurological examination showed no focal neurological deficits and the first EEG was nonspecific. She became progressively more confused, with subsequent drowsiness with psychomotor agitation, aimless buste, disorganization and visual hallucinations. She started treatment with quetiapine (200 mg/die) and lorazepam (4 mg/die) without benefit. The second EEG was characterized by triphasic waves with diffuse projection. MRI angiography revealed the presence of diffuse cortical ribbon hyperintensity of gray matter. CSF examination showed positivity of 14/3/3 protein and a TAU protein value greater than 16000 pq/ml allowing diagnosis of CJD.

We emphasize the importance of a correct differential diagnosis in patients with psychiatric symptoms and rapidly progressive dementia unresponsive to therapy.