Summary

A 71-year-old Caucasian woman presented with a 3 week history of progressive ataxia followed by rapid cognitive decline. Examination on admission showed cerebellar ataxia. This was followed by multifocal dementia with cortical, subcortical and brainstem involvements during her hospital stay. Protein 14-3-3 was identified in the cerebrospinal fluid (CSF) as well as a markedly increased S100b. The patient was reviewed by the National CJD Surveillance Unit and the National Prion Unit who supported the pre-mortem diagnosis of probable Sporadic Creutzfeldt-Jakob disease. The patient deteriorated rapidly, became cortically blind, bed bound and died in May 2009, 4 months after the onset of symptoms.