Dementia with Lewy Bodies

  • Anne-Catherine Vijverman
  • Carmela Tartaglia
  • Susan FoxEmail author


DLB is a progressive multisystem neurodegenerative disorder with widespread alpha-synuclein (αSyn) deposits in the central and peripheral nervous system as well as within the autonomic nervous system. In addition to dementia, its distinctive clinical features are visual hallucinations, parkinsonism, cognitive fluctuations, dysautonomia, sleep disorders, and neuroleptic sensitivity. The disease has an insidious onset and progresses to death with variable disease duration of on average 6.4 years. “Dementia with Lewy bodies” (DLB) is the clinical syndrome and “Lewy body disease” (LBD) refers to the pathological disease. The neuropathological hallmark of LBD is the presence of αSyn-positive neuronal inclusions in the form of Lewy bodies and Lewy neuritis. Neurochemically, DLB is associated with alterations in several neurotransmitter systems, the main changes occurring in the cholinergic, dopaminergic, and serotoninergic systems.

Currently no disease-modifying therapy is available, and management is therefore focused on symptomatic relief, but the combination of extrapyramidal symptoms, neuropsychiatric symptoms, and neuroleptic sensitivity makes the pharmacological treatment of DLB challenging. The mainstay of treatment includes levodopa for the parkinsonism and cholinesterase inhibitors for the cognitive deficits. Non-pharmacological, behavioral strategies aimed at modifying stressors in the environment should be employed whenever possible.


Dementia with Lewy bodies (DLB) Lewy body disease (LBD) Lewy bodies (LB) Movement disorders Dementia Neuroleptic sensitivity Alpha-synuclein (αSyn) Hallucinations Cognitive fluctuations 



Serotonin (5-hydroxytryptamine)


Alzheimer’s disease


Apolipoprotein E ε 4 allele


Amyloid β


Cerebrospinal fluid


Dopamine transporter SPECT


Dementia with Lewy bodies




Extrapyramidal signs


Glucocerebrosidase gene


Lewy bodies


Lewy body disease


Lewy neurites


123 I-meta-iodobenzylguanidine


Magnetic resonance imaging


Multiple system atrophy


Neurofibrillary tangle


Parkinson’s disease


Parkinson’s disease with dementia


Positron-emission tomography


11C-Pittsburgh compound B-PET


Postural instability and gait difficulties


REM sleep behavior disorder


Rapid eye movement


Sporadic Creutzfeldt-Jakob disease


Senile Plaque


Single photon emission computerized tomography


Selective serotonin reuptake inhibitors


Tremor dominant


Visual hallucinations




Total tau


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Copyright information

© Springer-Verlag London 2014

Authors and Affiliations

  • Anne-Catherine Vijverman
    • 1
  • Carmela Tartaglia
    • 2
  • Susan Fox
    • 3
    Email author
  1. 1.Department of Movement Disorders (Neurology)Toronto Western HospitalTorontoCanada
  2. 2.Memory Clinic (Neurology)Toronto Western HospitalTorontoCanada
  3. 3.Movement Disorders (Neurology)Toronto Western HospitalTorontoCanada

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