LAUSR

Literature reported on ERGs and VEPs changes in patients with prion disease (CJD). To characterize the extent of ERG and VEPs involvement in confirmed CJD; to assess ERGs and VEPs diagnostic usefulness in the workout of suspected CJD. White flash ERG (a/b-wave, 30-Hz flicker, oscillatory potentials) and VEP (P1, N1 and P2 components) were recorded in 122 consecutive unselected CJD patients (57 females, 65 males, mean age: 61.98 years) and compared with: (a) normal and control non-CJD (sensitivity and specificity), and (b) EEG, MRI and laboratory tests. ERG changes were correlated with pathology of the retina performed in several cases. ERG/VEPs were abnormal in just over 60% of CJD, compared with 76% MRI and 66% EEG abnormal rate; ERG changes revealed definite correlation with the codon129, VEPs did not; giant VEPs was recorded in the MM subtype; the VV patients had the most severe retinal changes. Twenty-two patients recorded over a period of six months showed ERG deteriorative pattern, VEPs did not, for it remained stable. ERGs/VEPs revealed abnormalities in CJD patients whose EEG and/or MRI did not show typical changes; although nonspecific they contribute to propose a diagnostic workout of patients suspected of CJD.