[Creutzfeldt-Jakob disease: alterations in an isolated cortical signal in diffusion magnetic resonance imaging]

Neuroimaging is playing an increasingly important role in the diagnosis of Creutzfeldt-Jakob disease (CJD), as shown by the fact that it is giving rise to the exclusion of other processes and providing invaluable diagnostic data -above all alterations in the signal in basal ganglia and in the cerebral cortex.

Case 1: A 66-year-old male who had symptoms of rapidly progressing cognitive impairment that began in the occipital region, with ataxia and myoclonias. The electroencephalogram (EEG) recordings revealed the presence of periodic biphasic sharp waves, and determination of protein 14-3-3 in cerebrospinal fluid (CSF) was negative. In the second cranial magnetic resonance (MR) scan that was performed, a signal increase at the level of the cortex, and predominantly in the right hemisphere, was observed in the diffusion sequences. Case 2: An 80-year-old male with a subacute clinical picture of cognitive and motor impairment that progressed in a matter of weeks to akinetic mutism accompanied by myoclonias. EEG recordings showed diffuse slowing, and the determination of protein 14-3-3 in CSF was positive. In both cranial MR scans carried out, we observed an isolated increase in the cortical signal in FLAIR sequences, and in the second MR scan there was also an increase in the cortical signal in the diffusion sequences. Neuropathology findings confirmed the diagnosis of sporadic CJD in both patients.

Cranial MR imaging in patients with CJD can be used to complement the other diagnostic tests and MR with diffusion sequences appears to offer greater diagnostic sensitivity, above all for the detection of a signal increase on a cortical level.