[Juvenile spongiform encephalopathy with unusual pathomorphologic findings]

A 27-year-old man presented disturbances of gait and language, quickly followed by intellectual deterioration, tetraplegia, anarthria and myoclonus. Histological examination of a cerebral biopsy showed not only cortical changes consistent with the diagnosis of Creutzfeldt-Jakob disease, but also many amyloid-plaques with variable morphology. The diagnostic interpretation of the case particularly as to concern his relationship to s.c. Gerstmann-Strüssler syndrome is discussed.