Neuropathology of Creutzfeldt-Jakob disease in Japan. With special reference to the panencephalopathic type

Abstract

Neuropathology of Creutzfeldt-Jakob disease (CJD), particularly of the panencephalopathic type and its additional findings, was described. The panencephalopathic type of CJD was characterized by extensive degeneration of the cerebral white matter which is diffuse in the deep and circumscribed and spongy in the digital white matter. This white matter lesion could not be explained simply as secondary to cortical deterioration. Most cases of the panencephalopathic type disclosed involvement of anatomically-interrelated systems in various combinations (inferior olivary nucleus-pontine nucleus-cerebellar cortex, globus pallidus - subthalamus - substantia nigra, optic tract-lateral geniculate body - optic radiation, and primary thalamic degeneration). These associated findings which could be paralleled as those of combined multisystemic degenerations have been found not only in the panencephalopathic type but also in other subtypes of CJD including Japanese cases of spongiform encephalopathy with multiple kuru plaques. They cannot be considered as coincidental to CJD.