Fast progressive memory loss in a 63-year-old man

A 63-year-old man presented to the neurology department with fast
progressive memory loss especially short term memory. For 2 weeks he
had experienced loss of orientation, judgment difficulties, and concentration
problems. A CT scan of the brain was normal.
MRI showed normal findings on T1 and T2 weighted images.. There
was no contrast enhancement. On diffusion weighted images hyperintense
signal was seen at the cortex of the right hemisphere and in the
basal ganglia. On PET regional hypometabolism of glucose correlated
with sites of increased intensity on diffusion weighted images. An electroencephalogram
showed periodic synchronous bi- or triphasic sharp
wave complexes. Laboratory findings showed a positive CSF 14-3-3
immuno-assay. Given these findings the diagnosis of sporadic
Creutzfeldt-Jacob disease was suggested. Dementia progressed rapidly
and the patient subsequently developped myoclonic jerks. The patient
died of pneumonia one month later. Brain biopsy confirmed the diagnosis
of Creutzfeldt Jacob.