Cerebral and cerebellar atrophy on serial magnetic resonance imaging in an initially symptom free subject at risk of familial prion disease

Abstract
A 42 year old woman from a family with histologically confirmed prion disease entered a study of symptom free subjects. She had no complaints of physical or cognitive problems and her lack of symptoms was confirmed by her family. Neurological examination and neuropsychometry gave normal results. She scored 29/30 on the mini-mental state examination. Her magnetic resonance scan was interpreted, by a neuroradiologist (JMS) blind to clinical details, as showing widening of cortical sulci, particularly those of the parietal lobes. In T2 weighted images there were no obvious abnormalities.