Lewy Body Dementia
Significance of Brain Lesions in Parkinson Disease Dementia and Lewy Body DementiaJellinger K.A.
Institute of Clinical Neurobiology, Vienna, Austria
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Dementia is increasingly recognized as a common feature in patients with Parkinson disease (PD)and dementia with Lewy bodies (DLB), both sharing many clinical and morphological features andbelieved to form a continuum within the spectrum of Lewy body diseases. Based on a large autopsyseries of parkinsonism (31–37% with dementia) and review of the recent literature, the pathologicalchanges underlying cognitive impairment in PD with dementia (PDD) and DLB are discussed. PDcases with Lewy body stages 3–5, i.e. only mild to moderate cortical -synuclein (Syn) depositions,and no additional pathologies, are rarely associated with cognitive impairment, which is frequentlyseen in PD and DLB cases with considerable cortical and limbic Syn load (increasing Lewy bodydensities) and/or associated widespread Alzheimer-type pathology. Clinicopathological studiesshow a negative relation between cognitive impairment and both cortical Lewy body pathology andAlzheimer type changes, suggesting that these either alone or in combination are major causes ofcognitive dysfunction, while others related them to presynaptic Syn aggregates. The neuropathologyof PDD and DLB is similar, without significant differences between cortical and subcortical Lewybodies and the pattern of synuclein pathology in the brainstem, but there are topographic differencesin nigral lesions, more frequent affection of the hippocampal CA 2/3 subareas and more severediffuse amyloid plaque load in the striatum of DLB. In conclusion, the pathology underlying cognitiveimpairment in PDD and DLB is heterogeneous, but there are some differences in the topographyand severity of lesions between both phenotypes that need further evaluation.
© 2009 S. Karger AG, Basel
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