A 70-year-old man presented with rapidly progressive cognitive impairment with ataxia
and myoclonus. Visual agnosia was noticed after blood pressure oscillations, during
immunoglobulin therapy for suspected autoimmune encephalitis. Brain magnetic resonanbce
imaging scans before and after visual agnosia are presented in [Figure 1]. Electroencephalogram disclosed periodic sharp wave complex, and cerebrospinal fluid
was positive for 14-3-3 protein. The final diagnosis was probable Creutzfeldt-Jakob
disease (CJD) associated with posterior reversible encephalopathy syndrome (PRES).
Blood pressure and immunoglobulin therapy may explain PRES[1]. It seems that some types of CJD prion proteins could induce the activation of microglia
and dysfunction of vasoconstrictors upregulation, leading to vasospasm, ischemia and
PRES[1],[2]
Figure 1 Magnetic resonance imaging features of sporadic Creutzfeld-Jakob disease and posterior
reversible encephalopathy syndrome. (A, B, C and D) Initial diffusion-weighted images
revealing bilateral hyperintensities in parietal areas, mostly on the right, and hyperintensities
in cingulate gyrus. The same findings apply albeit to a lesser extent in the right
basal ganglia (caudate and putamen, white arrows). (E, F, G and H) T2WI and T2-fluid-attenuated
inversion-recovery reveals new cortico-subcortical hyperintensities in parietal and
occipital areas and cerebellum, compatible with vasogenic edema, highly suggestive
of PRES (thick white arrows). Previously described alterations remain the same. T1W1
does not reveal contrast enhancement.
