Neurology and Neuroscience

The Essentials in Lewy Body Dementia

Horacio Enrique Sacristán,

Horacio Enrique Sacristán

1) Assistant Professor of Neurology, Faculty of Medicine, University of Buenos Aires (UBA), Buenos Aires, Argentina. 2) Full Professor of Neurology, Higher School of Health Sciences, University of Morón (UM), Buenos Aires, Argentina. 3) Adventist Clinical Neurology Service, Buenos Aires, Argentina.

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Falco Muñiz S,

Falco Muñiz S

Teaching Unit, Prof. Bernardo Houssay Hospital, School of Medicine, University of Buenos Aires, Buenos Aires, Argentina.

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Mauriño A,

Mauriño A

Department of Therapeutic Trials, Institute of Cognitive Neurology (INECO), Buenos Aires, Argentina.

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Pereira Pedreira V

Pereira Pedreira V

School of Health Sciences, University of Morón, Buenos Aires, Argentina.

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  10.33425/2692-7918.1126
Lewy body dementia (LBD) is an umbrella term describing two closely related conditions: Parkinson disease dementia (PDD) and dementia with Lewy bodies (DLB). LBD is the second most common cause of neurodegenerative dementia after Alzheimer's disease (AD), often underrecognized in clinical practice and accounting for approximately 4-7.5% of clinically diagnosed dementia cases but up to 20% of autopsy-confirmed dementia cases [1]. Neuropathologically, it is characterized by the accumulation of misfolded α-synuclein aggregates in neurons of the central and autonomic nervous systems and belongs to the group of synucleinopathies [2]. Core clinical features include progressive cognitive decline with marked fluctuations, recurrent visual hallucinations, REM sleep behavior disorder (RBD), and parkinsonism. Supportive features include severe neuroleptic sensitivity, autonomic dysfunction, postural instability, and recurrent falls [3]. The clinical presentation is heterogeneous and can overlap with AD and Parkinson's disease dementia (PDD), complicating differential diagnosis. In addition to Lewy bodies (LB), Alzheimer's disease co-pathology (beta-amyloid and tau deposits) is frequently present, contributing to diagnostic and clinical complexity [4]. The diagnosis of LBD is based on clinical criteria that include clinical manifestations and biomarkers, such as functional neuroimaging (PET or SPECT with dopamine transporter imaging), polysomnography (PSG), and ¹²³I-MIBG myocardial scintigraphy [5]. LBD exhibits genetic heterogeneity, with mutations in genes such as SNCA and GBA, and common risk factors such as APOE [6]. This condition typically manifests after age 60, although early-onset cases exist, posing a diagnostic challenge [7]. This review covers the key epidemiological, neuropathological, cognitive, behavioral, and biomarker characteristics of LBD and analyzes current treatment options.
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