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Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia

Gomperts, Stephen N. MD, PhD

CONTINUUM: Lifelong Learning in Neurology: April 2016 - Volume 22 - Issue 2, Dementia - p 435–463
doi: 10.1212/CON.0000000000000309
Review Articles

ABSTRACT Purpose of Review: This article provides an overview of the clinical features, neuropathologic findings, diagnostic criteria, and management of dementia with Lewy bodies (DLB) and Parkinson disease dementia (PDD), together known as the Lewy body dementias.

Recent Findings: DLB and PDD are common, clinically similar syndromes that share characteristic neuropathologic changes, including deposition of α-synuclein in Lewy bodies and neurites and loss of tegmental dopamine cell populations and basal forebrain cholinergic populations, often with a variable degree of coexisting Alzheimer pathology. The clinical constellations of DLB and PDD include progressive cognitive impairment associated with parkinsonism, visual hallucinations, and fluctuations of attention and wakefulness. Current clinical diagnostic criteria emphasize these features and also weigh evidence for dopamine cell loss measured with single-photon emission computed tomography (SPECT) imaging and for rapid eye movement (REM) sleep behavior disorder, a risk factor for the synucleinopathies. The timing of dementia relative to parkinsonism is the major clinical distinction between DLB and PDD, with dementia arising in the setting of well-established idiopathic Parkinson disease (after at least 1 year of motor symptoms) denoting PDD, while earlier cognitive impairment relative to parkinsonism denotes DLB. The distinction between these syndromes continues to be an active research question. Treatment for these illnesses remains symptomatic and relies on both pharmacologic and nonpharmacologic strategies.

Summary: DLB and PDD are important and common dementia syndromes that overlap in their clinical features, neuropathology, and management. They are believed to exist on a spectrum of Lewy body disease, and some controversy persists in their differentiation. Given the need to optimize cognition, extrapyramidal function, and psychiatric health, management can be complex and should be systematic.

Address correspondence to Dr Stephen N. Gomperts, 114 16th St, Massachusetts General Hospital, Charlestown, MA 02129, sgomperts@partners.org.

Relationship Disclosure: Dr Gomperts receives grant support from the National Institutes of Health as principal investigator of study 1-R21-NS-090243–01 and receives research support as principle investigator from the National Parkinson Foundation.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Gomperts reports no disclosure.

© 2016 American Academy of Neurology
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