Panencephalopathic type of Creutzfeldt-Jakob disease with neuropathologic features similar to Pick's disease

The panencephalopathic type of Creutzfeldt-Jakob disease is characterized by a serious degeneration of the white matter in addition to the other pathological features of the classic Creutzfeldt-Jakob disease. The clinical and neuropathological findings of a new case are described in a woman aged 62, who died after a year of illness. The brain appeared seriously affected by atrophy and white matter degeneration. Microscopically, it showed a marked cortical spongiosis, with gemistocytic astrogliosis and degeneration of the white matter of both hemispheres. Although a serious loss of nerve cells was evident, some residual neurons with a ballooned aspect were found in the fronto-temporal cortex. Other neurons presented argyrophilic inclusions similar to Pick bodies. By means of immunohistochemical techniques and monoclonal antineurofilaments antibodies some neurons with swollen cytoplasm and enlargement of the first tract of the neurites were detected in the basal layers of the frontal and temporal cortex. These abnormal features were due to the accumulation of phosphorylated 200 Kd neurofilaments. The relations between Creutzfeldt-Jakob and Pick's diseases are analyzed on the basis of the neuropathological findings. There is evidence from the immunohistochemical data of an interference in the axonal transport of neurofilaments.