Right parietal lobe syndrome as a clinical variant of Creutzfeldt-Jakob disease

We report a 57-year-old patient with Creutzfeldt-Jakob disease that initially presented with right parietal lobe syndrome. He featured early dystonic and hemiparetic posture of the left upper limb, finger agnosia, geographic disorientation and acalculia and developed myoclonus from the left upper extremity and right-sided periodic synchronized discharges. He died 2 years and 3 months later. Brain autopsy revealed a marked reduction in brain weight, extensive cortical spongiform changes and white matter degeneration. Immunolabeled prion proteins were deposited diffusely in the neuropil. Accentuation of the cortical change was unclear but white matter degeneration was more severe in the right parietal lobe than in the left parietal lobe. Right parietal lobe syndrome is rarely reported as an initial symptom of Creutzfeldt-Jakob disease and can be a clinical variant of the disease.