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Diffuse Lewy Body Disease (diffuse + lewy_body_disease)

Distribution by Scientific Domains

Medical Sciences	100%

Distribution within Medical Sciences

Pathology	77%
Neurology	22%

Selected Abstracts

Iron metabolism in Parkinsonian syndromes

MOVEMENT DISORDERS, Issue 9 2006
Daniela Berg MD
Abstract Growing evidence suggests an involvement of iron in the pathophysiology of neurodegenerative diseases. Several of the diseases are associated with parkinsonian syndromes, induced by degeneration of basal ganglia regions that contain the highest amount of iron within the brain. The group of neurodegenerative disorders associated with parkinsonian syndromes with increased brain iron content can be devided into two groups: (1) parkinsonian syndromes associated with brain iron accumulation, including Parkinson's disease, diffuse Lewy body disease, parkinsonian type of multiple system atrophy, progressive supranuclear palsy, corticobasal ganglionic degeneration, and Westphal variant of Huntington's disease; and (2) monogenetically caused disturbances of brain iron metabolism associated with parkinsonian syndromes, including aceruloplasminemia, hereditary ferritinopathies affecting the basal ganglia, and panthotenate kinase associated neurodegeneration type 2. Although it is still a matter of debate whether iron accumulation is a primary cause or secondary event in the first group, there is no doubt that iron-induced oxidative stress contributes to neurodegeneration. Parallels concerning pathophysiological as well as clinical aspects can be drawn between disorders of both groups. Results from animal models and reduction of iron overload combined with at least partial relief of symptoms by application of iron chelators in patients of the second group give hope that targeting the iron overload might be one possibility to slow down the neurodegenerative cascade also in the first group of inevitably progressive neurodegenerative disorders. © 2006 Movement Disorder Society [source]

The first autopsied case of diffuse Lewy body disease (DLBD): re-examination by recent immunostaining methods

NEUROPATHOLOGY, Issue 5 2010
The 50th Anniversary of Japanese Society of Neuropathology
Materials from our first autopsied case of diffuse Lewy body disease (DLBD), that was originally reported in 1976, were re-examined using recent immunohistochemical methods. Lewy pathology consisting of Lewy bodies and Lewy neurites appeared much more marked with alpha-synuclein immunostaining than had been detected with classical stainings. This case and our other similar cases prompted us to propose the terms "Lewy body disease" in 1980 and "diffuse Lewy body disease" in 1984. We also reported in 1990 that DLBD was classified into two forms: a pure form and a common form. Based on these studies the term "dementia with Lewy bodies (DLB)" was proposed in 1996. Since 1980, we have insisted that DLB, Parkinson disease (PD), and PD with dementia (PDD) should be understood within the spectrum of Lewy body disease. This insistence has been recently accepted by the International Workshop and the International Working Group on DLB and PDD in 2005 and in 2006, respectively. [source]

Chaperone and anti-chaperone: Two-faced synuclein as stimulator of synaptic evolution

NEUROPATHOLOGY, Issue 5 2006
Masayo Fujita
Previous studies have shown that ,-synuclein (,-syn), the homologue of ,-syn, inhibited ,-syn aggregation and stabilized Akt cell survival signaling molecule, suggesting that ,-syn was protective against ,-syn-related neurodegenerative disorders, such as Parkinson's disease and diffuse Lewy body disease. However, emerging evidence argues that the situation may be not so simple. Two missense mutations of ,-syn were identified in familial and sporadic diffuse Lewy body disease, and wild type ,-syn was induced to form fibril structures in vitro, while, ,-syn was shown to be protective against neurodegeneration caused by deletion of cysteine-string protein-,, the presynaptic cochaperone to Hsc70 in mice. Collectively, ,- and ,-syn are both, but in varying degrees, featured with two opposite properties, namely normal chaperone and anti-chaperone. By reviewing recent progress in syn biology with a particular focus on ,-syn, this manuscript refers to the intriguing possibility that the dual syn proteins might have acquired a driving force for synaptic evolution. Hypothetically, the anti-chaperone syn may provoke stress-induced diverse responses, whereas, the chaperone syn may provide buffering for them, allowing accumulation of nonlethal phenotypic variations in synapses. Consequently, dual syn proteins may cope with forth-coming stresses in the brain by stimulating adaptive evolution. In this context, failure to regulate this process due to various causes, such as gene mutations and environmental risk factors, may result in imperfect adaptability against stresses, leading to neurodegenerative disorders. [source]

Clinicopathological studies on diffuse Lewy body disease

NEUROPATHOLOGY, Issue 1 2000
Kenji Kosaka
With reports of dementia cases with numerous cortical Lewy bodies and our proposal that this be named ,diffuse Lewy body disease' (DLBD), this condition has received a great deal of attention, first in Japan and subsequently in Europe and North America. In the early 1990s, similar types of nomenclature were considered, and at the First International Workshop in 1995, it was proposed that ,dementia with Lewy bodies' be used as a generic term for Lewy body dementia, including the DLBD form. We review our previous clinicopathological findings and describe our recent immunohistochemical studies on DLBD. [source]

Selective PrP-like protein, doppel immunoreactivity in dystrophic neurites of senile plaques in Alzheimer's disease

NEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 4 2004
I. Ferrer
Doppel (Dpl) is a prion-like protein encoded by the gene PRND, which has been found downstream of the prion gene PRNP in several species. The present study examines by immunohistochemistry Dpl expression in brain samples from 10 patients with Alzheimer's disease (AD), three patients with Pick's disease, four patients with Parkinson's disease, eight patients with diffuse Lewy body disease (DLBD), six patients with sporadic Creutzfeldt,Jakob disease (CJD) methionine/methionine at the codon 129, two patients with sporadic CJD methionine/valine at the codon 129 and numerous kuru plaques in the cerebellum, one patient with fatal familial insomnia (FFI), and 10 age-matched controls. In the adult human brain, Dpl immunoreactivity was restricted to scattered granule cells of the cerebellum and scattered small granules in the cerebral cortex. Dpl immunoreactivity was seen around ,A4 amyloid deposits in neuritic plaques, but not in diffuse plaques, AD and the common form of DLBD. Neurofibrillary tangles, Pick bodies and Lewy bodies were not stained with anti-Dpl antibodies. No modifications in Dpl immunoreactivity were observed in CJD excepting those associated with accompanying senile plaques. No Dpl-positive deposits were seen in FFI. Whether Dpl in neuritic plaques may attenuate amyloid-induced oxidative stress and participate in the glial response around amyloid cores is discussed in light of the few available data on Dpl functions. [source]

Alzheimer's disease versus dementia with Lewy bodies: Cerebral metabolic distinction with autopsy confirmation

ANNALS OF NEUROLOGY, Issue 3 2001
Satoshi Minoshima MD
Seeking antemortem markers to distinguish Dementia with Lewy bodies (DLB) and Alzheimer's disease (AD), we examined brain glucose metabolism of DLB and AD. Eleven DLB patients (7 Lewy body variant of AD [LBVAD] and 4 pure diffuse Lewy body disease [DLBD]) who had antemortem position emission tomography imaging and autopsy confirmation were compared to 10 autopsy-confirmed pure AD patients. In addition, 53 patients with clinically-diagnosed probable AD, 13 of whom later fulfilled clinical diagnoses of DLB, were examined. Autopsy-confirmed AD and DLB patients showed significant metabolic reductions involving parietotemporal association, posterior cingulate, and frontal association cortices. Only DLB patients showed significant metabolic reductions in the occipital cortex, particularly in the primary visual cortex (LBVAD ,23% and DLBD ,29% vs AD ,8%), which distinguished DLB versus AD with 90% sensitivity and 80% specificity. Multivariate analysis revealed that occipital metabolic changes in DLB were independent from those in the adjacent parietotemporal cortices. Analysis of clinically-diagnosed probable AD patients showed a significantly higher frequency of primary visual metabolic reduction among patients who fulfilled later clinical criteria for DLB. In these patients, occipital hypometabolism preceded some clinical features of DLB. Occipital hypometabolism is a potential antemortem marker to distinguish DLB versus AD. [source]