Neuropathology and pathogenesis of dementia with lewy bodies

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Abstract

Dementia with Lewy bodies (DLB) is a clinical syndrome characterized by mid-to-late life dementia associated with visual hallucinations, parkinsonism and a fluctuating clinical course (McKeith et al, 2004). In this discussion, the term Lewy body disease (LBD) is used to refer to the pathological process associated with neuronal cytoplasmic inclusions first described by Lewy (Dickson, 2002a). As is true for all clinical neurological syndromes, the pathological substrate of DLB is not exclusively a single disease process. For example, parkinsonism, which is the clinical syndrome characterized by bradykinesia, rigidity and resting tremor, has a number of distinct pathological substrates, including LBD, multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) (Dickson, 2002b). These disorders share progressive neuronal loss and gliosis in the dopaminergic neurons of the pars compacta of the substantia nigra, which leads to the striatal dopaminergic deficiencies that cause the extrapyramidal parkinsonian syndrome (Fig. 11.1c). Given that one of the cardinal clinical features of DLB is parkinsonism, it can be predicted that the pathological substrate for this aspect of DLB might be heterogeneous. Indeed, autopsy studies of prospectively diagnosed patients with DLB occasionally have identified neurofibrillary tangle pathology, consistent with PSP, in the substantia nigra rather than Lewy bodies (McKeith et al, 2000).

Original languageEnglish (US)
Title of host publicationDementia with Lewy Bodies
Subtitle of host publicationand Parkinson's Disease Dementia
PublisherCRC Press
Pages149-166
Number of pages18
ISBN (Electronic)9780203313909
ISBN (Print)1841843954, 9781841843957
StatePublished - Jan 1 2005

ASJC Scopus subject areas

  • Medicine(all)

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    Dickson, D. W. (2005). Neuropathology and pathogenesis of dementia with lewy bodies. In Dementia with Lewy Bodies: and Parkinson's Disease Dementia (pp. 149-166). CRC Press.