Lewy Body Dementia

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Lewy Body Dementia

NINDS Dementia With Lewy Bodies Information Page

"Dementia with Lewy bodies (DLB) is one of the most common types of progressive dementia. The central feature of DLB is progressive cognitive decline, combined with three additional defining features: (1) pronounced “fluctuations” in alertness and attention, such as frequent drowsiness, lethargy, lengthy periods of time spent staring into space, or disorganized speech; (2) recurrent visual hallucinations, and (3) parkinsonian motor symptoms, such as rigidity and the loss of spontaneous movement. People may also suffer from depression. The symptoms of DLB are caused by the build-up of Lewy bodies – accumulated bits of alpha-synuclein protein -- inside the nuclei of neurons in areas of the brain that control particular aspects of memory and motor control. … The similarity of symptoms between DLB and Parkinson’s disease, and between DLB and Alzheimer’s disease, can often make it difficult for a doctor to make a definitive diagnosis. In addition, Lewy bodies are often also found in the brains of people with Parkinson's and Alzheimer’s diseases. These findings suggest that either DLB is related to these other causes of dementia or that an individual can have both diseases at the same time. DLB usually occurs sporadically, in people with no known family history of the disease."

Highlighted Article

Dementia with lewy bodies. (Semin Neurol. 2007)

"The presentation of DLB is typically one of cortical and subcortical cognitive impairments, with worse visuospatial and executive dysfunction than Alzheimer's disease. There may be relative sparing of memory especially in the early stages. Core clinical features of DLB include fluctuating attention, recurrent visual hallucinations, and parkinsonism. Suggestive features include REM sleep behavior disorder, severe neuroleptic sensitivity, and low dopamine transporter uptake in the basal ganglia on functional neuroimaging. Additional supportive features that commonly occur in DLB, but with lower specificity, include repeated falls and syncope, transient, unexplained loss of consciousness, severe autonomic dysfunction, hallucinations in other modalities, systematized delusions, depression, relative preservation of medial temporal lobe structures on structural neuroimaging, reduced occipital activity on functional neuroimaging, prominent slow wave activity on electroencephalogram, and low uptake myocardial scintigraphy."

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Lewy Body Dementia

Diagnosis, Imaging, and Screening

NEWS:

Personality Changes Predict Dementia "According to researchers who followed 290 people until they died, those who displayed passive personality changes at the beginning of the study -- like a diminished emotional response, repetitive behaviors, lack of interest in hobbies, and lack of interest in life -- were significantly more likely to be diagnosed with dementia with Lewy bodies. Overall, up to 75 percent of people with that form of dementia had exhibited passive personality changes at the beginning of the research, compared to just 45 percent of those who ended up with a diagnosis of Alzheimer's disease. These traits were often associated with visual and auditory hallucinations as well, which are hallmarks of the condition. Separating dementia with Lewy bodies patients from Alzheimer's patients is important, note the investigators, because some of the drugs prescribed to treat Alzheimer's disease can be deadly for people with dementia with Lewy bodies. Early identification of people likely to progress to this form of dementia can ensure doctors prescribe appropriate medications and can also help families begin the long process of coping with a loved one with the disease."

Rostral raphe involvement in Lewy body dementia and multiple system atrophy. (Acta Neuropathol (Berl). 2007) "Depression is a feature of both Lewy body disorders and multiple system atrophy (MSA). Since serotonergic neurons of the rostral raphe have been implicated in depression, we sought to determine whether there is a differential involvement of these neurons in cases with clinically diagnosed dementia with Lewy bodies (DLB) or MSA. … The caudal raphe groups were affected both in MSA and in DLB. There is a differential involvement of raphe neurons in DLB and MSA. Although loss of rostral raphe neurons may contribute to depression in DLB, this appears to be less likely in MSA. Factors other than the neurochemical phenotype determine neuronal vulnerability in MSA."

ARTICLES:

JOURNAL ARTICLES:

A novel locus for dementia with Lewy bodies: a clinically and genetically heterogeneous disorder. (Brain. 2007) "Dementia with Lewy bodies (DLB) represents the second most frequent type of neurodegenerative dementia in the elderly. Although most patients have sporadic DLB, a limited number of DLB families have been described, suggesting that genetic factors may contribute to DLB pathogenesis. Here, we describe a three-generation Belgian family with prominent dementia and parkinsonism, consistent with a diagnosis of DLB, that was autopsy confirmed for the index patient."

Behavioral and psychological symptoms in dementia with Lewy-bodies (DLB): Frequency and relationship with disease severity and motor impairment (Archives of Gerontology and Geriatrics 2007) "Clinical criteria for DLB have been more and more accurate over time, and they had focused on psychotic symptoms for their high frequency. Recent literature suggests that behavioral and psychological symptoms of dementia (BPSD) are frequently associated with DLB, beyond the presence of psychosis. Notwithstanding, the occurrence of BPSD in DLB is under-investigated, and no data are available yet in the different stages. Aim of the present study was to evaluate BPSD pattern in the different stages of DLB, and characterize the relationship with both cognitive deficits and Parkinsonian signs."

Boston naming performance distinguishes between Lewy body and Alzheimer's dementias (Archives of Clinical Neuropsychology 2007) "Recent research has suggested that persons with dementia with Lewy bodies (DLB) have more visuospatial/visuoperceptual dysfunction than those with Alzheimer's disease (AD), which may impact their ability to correctly perceive and name objects. … In terms of error types, DLB patients made significantly more visuoperceptual errors (p < .05) while AD patients made significantly more semantic errors (p < .001)."

Classification of Psychotic Symptoms in Dementia With Lewy Bodies (Am J Geriatr Psychiatry 2007) "Conclusion: This study suggested that hallucinations, misidentifications, and delusions should be separately considered in understanding of underlying pathophysiology or psychopathology of DLB."

Comparison of Regional Brain Volume and Glucose Metabolism Between Patients with Mild Dementia with Lewy Bodies and Those with Mild Alzheimer's Disease. (J Nucl Med. 2007) "CONCLUSION: Comparison of very mild DLB and AD revealed different morphologic and metabolic changes occurring in the medial temporal lobes and the occipital lobe, demonstrating characteristic pathophysiologic differences between these 2 diseases."

Deep gray matter hyperperfusion with occipital hypoperfusion in dementia with Lewy bodies. (Eur J Neurol. 2007) "Our results suggest that the addition of deep gray matter hyperperfusion to occipital hypoperfusion may be useful in the clinical differentiation of DLB and AD."

Dementia with lewy bodies. (Semin Neurol. 2007) "The presentation of DLB is typically one of cortical and subcortical cognitive impairments, with worse visuospatial and executive dysfunction than Alzheimer's disease. There may be relative sparing of memory especially in the early stages. Core clinical features of DLB include fluctuating attention, recurrent visual hallucinations, and parkinsonism. Suggestive features include REM sleep behavior disorder, severe neuroleptic sensitivity, and low dopamine transporter uptake in the basal ganglia on functional neuroimaging. Additional supportive features that commonly occur in DLB, but with lower specificity, include repeated falls and syncope, transient, unexplained loss of consciousness, severe autonomic dysfunction, hallucinations in other modalities, systematized delusions, depression, relative preservation of medial temporal lobe structures on structural neuroimaging, reduced occipital activity on functional neuroimaging, prominent slow wave activity on electroencephalogram, and low uptake myocardial scintigraphy."

Dementia with lewy bodies: A comparison of clinical diagnosis, FP-CIT SPECT imaging and autopsy. (J Neurol Neurosurg Psychiatry. 2007) "Interpretation: FP-CIT SPET scans substantially enhanced the accuracy of diagnosis of DLB by comparison with clinical criteria alone."

Distinguishing Lewy body dementias from Alzheimer's disease. (Expert Rev Neurother. 2007) "Lewy body dementia (LBD) is the second most common dementia after Alzheimer's disease (AD). LBD is characterized clinically by visual hallucinations, extrapyramidal symptoms, cognitive fluctuations and neuroleptic sensitivity. LBD and AD share many common features in pathology, genetics and biochemical alterations; however, correct clinical distinction between these disorders has prognostic and therapeutic implications."

DLB and PDD boundary issues (NEUROLOGY 2007)

Focal atrophy in dementia with Lewy bodies on MRI: a distinct pattern from Alzheimer's disease. (Brain. 2007) "These findings support recent pathological studies showing an ascending pattern of Lewy body progression from brainstem to basal areas of the brain. Damage to this network of structures in DLB may affect a number of different neurotransmitter systems which in turn may contribute to a number of the core clinical features of DLB."

Incident dementia in women is preceded by weight loss by at least a decade (NEUROLOGY 2007) "Conclusions: Even accounting for delays in diagnosis, weight loss precedes the diagnosis of dementia in women but not in men by several years. This loss may relate to predementia apathy, loss of initiative, and reduced olfactory function."

Personality traits distinguishing dementia with Lewy bodies from Alzheimer disease (NEUROLOGY 2007)

Visual recognition memory differentiates dementia with Lewy bodies and Parkinson's disease dementia. (J Neurol Neurosurg Psychiatry. 2007)

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