[Panencephalopathy of the Creutzfeldt-Jakob disease type]

Abstract

Creutzfeldt-Jakob disease (CJD) occurring in later life has a duration of about one year. Gait disturbance, myoclonic jerks, dementia and akinetic mutism occur. Post-mortem examination of the brain shows nerve cell loss, gliosis and spongiform changes of the grey matter. Clinical and neuropathological features of a case of the panencephalopathic type of CJD, with additional extensive degeneration of the cerebral white matter, are described. The panencephalopathic type of CJD is characterized clinically by a longer duration and a biphasic course.