Corticobasal degeneration syndrome: a case of Lewy body variant of Alzheimer?s disease

Abstract

Corticobasal degeneration syndrome (CBDS) is a well-described entity, although there are some cases in which clinical manifestations do not link with pathological findings. We present a 60-year-old man with a clinical course of CBDS. Postmortem examination demonstrated the features of the Lewy body variant of Alzheimer disease (LBVA). Different neurological evaluations showed a progressive motor disorder with alien hand and parkinsonism affecting mainly the left side. His neuropsychological examination was comparable with biparietal dysfunction, especially apraxias and signs of Gertsmann's syndrome. MRI and SPECT imaging revealed parietal, temporal and occipital involvement. We conclude that the CBDS is a heterogeneous pathological entity.