Dementia with Lewy Bodies

Jared Hinkle , Gregory Pontone, M.D.
Dementia with Lewy Bodies is a topic covered in the Johns Hopkins Psychiatry Guide.

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DEFINITION

  • Dementia with Lewy bodies (DLB) is a primary neurodegenerative disorder affecting cognitive function and movement.
    • Early symptoms include prominent deficits in attention, as well as visuospatial and executive dysfunction.
    • Parkinsonism, hallucinations, cognitive fluctuations, and REM sleep behavior disorder are commonly present.
  • DLB is one of the three major α-synucleinopathies, along with Parkinson disease (PD) and multiple system atrophy but is more likely to be misdiagnosed as Alzheimer disease (AD).
    • α-synuclein protein aggregation in cytoplasmic neuronal inclusions (Lewy bodies) is a pathological hallmark of both DLB and PD.
    • Classically, DLB pathology features heavy neocortical Lewy body burden with low AD pathology Aβ or tau pathology, but co-pathology is frequent and may contribute to heterogeneity of disease [1][2][3].
  • Compared with AD, DLB has a shorter mean survival time after diagnosis (~4 years vs. ~6 years) [4] and generally places more significant financial burdens on patients and their families/caretakers [5].
  • Cognitive impairment due to Lewy bodies is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5) [6].

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DEFINITION

  • Dementia with Lewy bodies (DLB) is a primary neurodegenerative disorder affecting cognitive function and movement.
    • Early symptoms include prominent deficits in attention, as well as visuospatial and executive dysfunction.
    • Parkinsonism, hallucinations, cognitive fluctuations, and REM sleep behavior disorder are commonly present.
  • DLB is one of the three major α-synucleinopathies, along with Parkinson disease (PD) and multiple system atrophy but is more likely to be misdiagnosed as Alzheimer disease (AD).
    • α-synuclein protein aggregation in cytoplasmic neuronal inclusions (Lewy bodies) is a pathological hallmark of both DLB and PD.
    • Classically, DLB pathology features heavy neocortical Lewy body burden with low AD pathology Aβ or tau pathology, but co-pathology is frequent and may contribute to heterogeneity of disease [1][2][3].
  • Compared with AD, DLB has a shorter mean survival time after diagnosis (~4 years vs. ~6 years) [4] and generally places more significant financial burdens on patients and their families/caretakers [5].
  • Cognitive impairment due to Lewy bodies is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5) [6].

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Last updated: April 3, 2022