NEUROPHYSIOLOGICAL FOLLOW-UP IN 2 CHILDREN WITH CREUTZFELDT-JAKOB-DISEASE AFTER HUMAN GROWTH-HORMONE TREATMENT

A serial neurophysiological study has been performed of 2 children dur ing the clinical course of Creutzfeldt-Jakob disease after human growt h hormone (hGH) treatment. Evolution of the EEG pattern was typical: s low waves, periodic sharp wave complexes, then extinction. VEP compone nts were moderately altered. BAERs performed in only 1 child were norm al. The blink reflex (BR) showed an early alteration of the R1 compone nt. The ERG exhibited early and profound anomalies. Pathological chang es were obvious at the first recording, at the beginning of the second month after clinical onset: increase in peak latencies, morphological changes and important reduction of b wave amplitude (a/b amplitude ra tio greater than or equal to 0.70; normal range 0.27-0.41). The ERG wa s completely absent a few months later. These results are compared wit h the similar retinopathy described in CJD- or scrapie-infected rodent s. In the 2 children, pathological changes in ERG and in BR were obvio us several months before the development of the typical EEG pattern. T herefore, early ERG and BR recording can be helpful in the diagnosis o f CJD, especially in this ataxic form following hGH treatment.