In 1912, Frederick Lewy first described large eosinophilic spherical or kidney-shaped inclusions (now known as Lewy bodies) in neuronal cell bodies in the substantia nigra of patients with Parkinson’s disease (PD). Cortical Lewy bodies in association with dementia were first reported only in the 1960s. Unlike the classic Lewy bodies of PD found in pigmented brainstem nuclei, cortical Lewy bodies are only faintly eosinophilic, are not sharply demarcated by a surrounding halo and do not show a radial filamentous substructure [1]. All these features made these cortical lesions difficult to identify until the 1980s when ubiquitin and α-synuclein immunostains became available. Ubiquitin and α-synuclein staining revealed that Lewy bodies were a common neuropathological finding in dementia. By the mid-1990s, cortical Lewy body disease was recognised as a relatively common cause of dementia. Using new immunohistochemical techniques significant numbers of cortical Lewy bodies could be found in as many as 15–25% of patients with dementia, although many of these patients also showed Alzheimer-type neuropathological changes [1].