CREUTZFELDT-JAKOB-DISEASE IN A CADAVER-DE RIVED HUMAN GROWTH-HORMONE RECIPIENT - A CLINICOPATHOLOGICAL STUDY

This report describes the pathological changes observed in the brain o f an 18-year-old patient who died with Creutzfeldt-Jakob disease (CJD) of the ataxic and panencephalopathic type. Clinically, the disease be gan at age 17 with a rapidly progressive cerebellar syndrome, associat ed with myoclonus and mental deterioration. Cranial CT scan and MRI sh owed minor abnormalities. EEG demonstrated non specific changes. The p atient became progressively demented, bedridden and died 10 months aft er the onset. Nine years earlier, he had been operated upon for cranio pharyngioma and subsequently treated with cadaver-derived human growth hormone. Post-mortem examination of the brain revealed mild diffuse a trophy. Histology showed congophilic amyloid plaques found in both gra y and while matters of the cerebrum and cerebellum, associated with sp ongiosis, mainly in the basal ganglia and cerebellum, and gliosis. The y were immunostained with antiprion protein antibody using the immunop eroxydase method on paraffin embedded sections. The clinical findings in this case were similar to those of other cases of human growth horm one-associated CJD. However, this case is unusual because of the great number of amyloid plaques, which have been only rarely found in previ ous reports.