A patient with Creutzfeldt-Jakob disease who showed numbness of the right hand as the initial symptom was reported. A 51-year-old man, a dairy farmer, complained of numbness of the right hand. All sensations, except for cortical senses, were reduced in the right hand. Nerve conduction velocities and cervical spine X-ray were normal. During the next three weeks, ataxic hemiparesis, cortical blindness and dementia appeared. He died eight months after the onset. Neuropathological findings revealed patchy and localized spongy states in the subcortical white matter in addition to severe neuronal loss with status spongiosus in the cerebral cortex (panencephalopathic type).