ABSTRACT
The sporadic form of this disease typically presents in middle age, but it may occur in young adults. In humans, transmission has been through corneal transplants, depth electrodes and human growth hormone injections. It is transmitted by a prion protein. After a latency period of 20-30 years (sometimes less), a rapidly progressive dementia ensues, culminating in death in less than a year. The EEG evolves into a characteristic pattern (Figure 4.38) and MRI may show abnormal high signal in the basal ganglia on T2-weighted images (Figure 4.39). CSF may be positive for the 14-3-3 brain protein but false positive and negative results may occur. The histopathological features are unique (Figure 4.40).
