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Dementia with Lewy Bodies

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

Abstract

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.

Keywords

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

Abbreviations

5-HT

Serotonin (5-hydroxytryptamine)

AD

Alzheimer’s disease

ApoE4

Apolipoprotein E ε 4 allele

42

Amyloid β

CSF

Cerebrospinal fluid

DaTSCAN

Dopamine transporter SPECT

DLB

Dementia with Lewy bodies

EEG

Electroencephalography

EPS

Extrapyramidal signs

GBA1

Glucocerebrosidase gene

LB

Lewy bodies

LBD

Lewy body disease

LN

Lewy neurites

MIBG

123 I-meta-iodobenzylguanidine

MRI

Magnetic resonance imaging

MSA

Multiple system atrophy

NFT

Neurofibrillary tangle

PD

Parkinson’s disease

PDD

Parkinson’s disease with dementia

PET

Positron-emission tomography

PIB-PET

11C-Pittsburgh compound B-PET

PIGD

Postural instability and gait difficulties

RBD

REM sleep behavior disorder

REM

Rapid eye movement

sCJD

Sporadic Creutzfeldt-Jakob disease

SP

Senile Plaque

SPECT

Single photon emission computerized tomography

SSRI’s

Selective serotonin reuptake inhibitors

TD

Tremor dominant

VH

Visual hallucinations

αSyn

alpha-synuclein

τ

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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