Clinical and neuropathological correlates of Lewy body disease

Excerpt

In a recent clinicopathological study of 30 brains of patients with Lewy body (LB) disease clinically presenting as Parkinson's disease (PD), dementia with Lewy bodies (DLB) or pure autonomic failure (PAF) and morphologically classified as neocortical, limbic and brain-stem predominant types, Hishikawa et al. [8] stated that both the clinical features and the morphological changes, in particular the immunopathological features and distribution of neurological and glial inclusions, were similar. All neocortical cases (n=8, mean age at death 70.2 years, mean duration 8 years) showed parkinsonism and dementia within or more than 1 year after onset of symptoms; limbic type (n=17, mean age 74.7 years, mean duration 9.9 years) had parkinsonism but only 70.6% developed dementia, occasionally with fluctuating cognition, while the brain stem-predominant type either showed parkinsonism (n=1) or PAF (n=2). Because neuritic Alzheimer's disease (AD) pathology in all cases corresponded to Braak stages I or II, none fitted to accepted criteria of additional AD or its LB variant (LBV/AD). Since DLB, PD with and without dementia and PAF shared several clinical and neuropathological feature, the authors concluded that these conditions are part of a unique "LB disease" spectrum. …