What is the pathophysiology of dementia with Lewy bodies (DLB)?

Updated: Sep 24, 2018
  • Author: Stephen M Bloomfield, MD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Answer

No familial disposition for DLB has been reported. Some have proposed that DLB represents an extended form of Parkinson disease. However, other authors view this as a clinically distinct entity.

The common thread to DLB and Parkinson disease is the presence of Lewy bodies. These are rounded inclusion bodies containing ubiquitin as the main component. In Parkinson disease, they are mainly observed in the substantia nigra. In contrast, in DLB they are scattered throughout the cerebral cortex and also are seen in the nigra and other subcortical regions. In contrast to Alzheimer disease and CBD, cortical atrophy is not prominent. Two distinct forms of DLB have been described. The pure form occurs with Lewy bodies in the cortical and subcortical structures, whereas the common form has Lewy bodies accompanied by plaques and tangles.


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