Dementia with Lewy bodies

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Dementia with Lewy bodies
ICD-10	G31.8
ICD-9	331.82
DiseasesDB	3800
MeSH	D020961

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Overview

Dementia with Lewy bodies is the second most frequent cause of hospitalization for dementia, after Alzheimer's disease. Current estimates are that about 60-to-75% of diagnosed dementias are of the Alzheimer's and mixed (Alzheimer's and vascular dementia) type, 10-to-15% are Lewy Bodies type, with the remaining types being of an entire spectrum of dementias including frontotemporal lobar degeneration, alcoholic dementia, pure vascular dementia, etc.

Historical Perspective

• Lewy body dementia (LBD) was named after Frederich Heinrich Lewy, a German-American neurologist who discovered the characteristic intracytoplasmic inclusions in 1912.
• In 1961, Okazaki suggested that the presence of cortical Lewy bodies with associated with the development of dementia.
• In 1984, Kosaka and colleagues hypothesized that the presence of Lewy bodies may correspond to a new disease entity, which was eventually named "diffuse Lewy body disease". The disease name was then changed in 1996 at the First International Workshop of the Consortium on Dementia with Lewy Bodies (DLB) to become "dementia with Lewy bodies".
• DLB was not considered a diagnosis of dementia in the first 4 versions of the Diagnostic and Statistical Manual (DSM) for Mental Disorders. In 2013, DSM-5 finally incorporated DLB as a diagnosis for dementia.

Presentation

Dementia with Lewy Bodies (DLB) exhibits clinical overlap between Alzheimer's disease and Parkinson's disease. Pathologically, it is characterized by development of abnormal proteinaceous (alpha-synuclein) cytoplasmic inclusions, called Lewy bodies, throughout the brain. These inclusions have similar structural features to "classical" Lewy Bodies seen subcortically in Parkinson's disease.

Additionally, there is a loss of dopamine-producing neurons (in the substantia nigra) similar to that seen in Parkinson's disease, and a loss of acetylcholine-producing neurons (in the basal nucleus of Meynert and elsewhere) similar to that seen in Alzheimer's disease. Cerebral atrophy (or shrinkage) also occurs as the cerebral cortex degenerates. Autopsy series have revealed that the pathology of DLB is often concomitant with the pathology of Alzheimer's disease. That is, when Lewy Body inclusions are found in the cortex, they often co-occur with Alzheimer's disease pathology found primarily in the hippocampus, including: neurofibrillary tangles (abnormally phosphorylated tau protein), senile plaques (deposited beta-amyloid protein), and granulovacuolar degeneration.

Within DLB, the loss of cholinergic (acetylcholine-producing) neurons is thought to account for the degradation of cognitive and emotional functioning as in Alzheimer's disease, while the loss of dopaminergic (dopamine-producing) neurons is thought to account for the degradation of motor control as in Parkinson's disease. Thus, DLB is similar in some ways to both the dementia resulting from Alzheimer's disease and Parkinson's disease. In fact, it is often confused in its early stages with Alzheimer's disease and/or vascular dementia (multi-infarct dementia). The overlap of neuropathologies and presenting symptoms (cognitive, emotional, and motor) may make an accurate differential diagnosis difficult to reach.

Differential Diagnosis

• Major or mild neurocognitive disorder due to Parkinson’s disease

Epidemiology and Demographics

Prevalence

The prevalence of major or mild neurocognitive disorder is:

5,000 per 100,000 (5%) of the general elderly population

17,000-30,500 per 100,000 (1.7%-30.5%) of all dementia cases

Risk Factors

• Genetic predisposition

Diagnosis

Clinical features

Core features include fluctuating cognition with variations in attention and alertness, recurrent visual hallucinations (typically early in the disease), and motor features of parkinsonism. DLB patients also often experience repeated falls, syncope (fainting), transient loss of consciousness, and hypersentivity to neuroleptic medications. Generally, DLB is diagnosed when cognitive symptoms develop within a year or two of movement disorder/Parkinsonian symptoms. Recent research suggests that presence of sleep disturbance may also be useful in differentiating DLB from other forms of dementia.

Diagnostic Criteria

DSM-V Diagnostic Criteria for Major or Mild Neurocognitive Disorder With Lewy Bodies

“

A.The criteria are met for major or mild neurocognitive disorder. AND B.The disorder has an insidious onset and gradual progression. AND C.The disorder meets a combination of core diagnostic features and suggestive diagnostic features for either probable or possible neurocognitive disorder with Lewy bodies. For probable major or mild neurocognitive disorder with Lewy bodies, the individual has two core features, or one suggestive feature with one or more core features. For possible major or mild neurocognitive disorder with Lewy bodies, the individual has only one core feature, or one or more suggestive features. 1.Core diagnostic features: a.Fluctuating cognition with pronounced variations in attention and alertness. b.Recurrent visual hallucinations that are well formed and detailed. c.Spontaneous features of parkinsonism, with onset subsequent to the development of cognitive decline. 2.Suggestive diagnostic features; a.Meets criteria for rapid eye movement sleep behavior disorder. b.Severe neuroleptic sensitivity. AND D.The disturbance is not better explained by cerebrovascular disease, another neurodegenerative disease, the effects of a substance, or another mental, neurological, or systemic disorder.

”

Treatment

The treatment of DLB, as with Parkinson's disease, involves striking a balance between treating the motor and emotive/cognitive symptoms. Treatment of the movement portion of the disease can typically result in worsening hallucinations and psychosis, while treatment of the hallucinations and psychosis can result in worsening movement symptoms. The use of cholinesterase inhibitors represents the treatment of choice. This improves symptoms, but does not cure the disease. The use of memantine may be recommended, and may represent a means to slow or prevent the decline of cognitive function, although strong evidence to support or disprove this is lacking.

Nomenclature

Dementia with Lewy bodies (DLB) is also known under a variety of other names including, Lewy Body dementia (LBD), Diffuse Lewy Body disease (DLBD), Cortical Lewy Body disease (CLBD), and Senile dementia of Lewy type. All incorporate the name Lewy, as Dr. Frederic Lewy (1885-1950) was first to discover the abnormal protein deposits ("Lewy Body inclusions") in the early 1900s.