Neuropathology of Lewy body disease: Clinicopathological crosstalk between typical and atypical cases.

Neuropathology of Lewy body disease: Clinicopathological crosstalk between typical and atypical cases.

Publication date: Sep 09, 2019

Lewy body disease (LBD) is characterized by the presence of Lewy bodies (LBs) and Lewy neurites and comprises a diagnostic spectrum that includes Parkinson’s disease (PD), PD with dementia, and dementia with LBs. LBs and Lewy neurites are insoluble aggregates composed mainly of phosphorylated α-synuclein and can be widely distributed throughout the central and peripheral nervous systems. The distribution of LBs may determine the LBD phenotype. Braak hypothesized that Lewy pathology progresses ascendingly from the peripheral nervous system to the olfactory bulbs and brainstem and then to other brain regions. Braak’s PD staging suggests that LBD is a prion-like disease. Most typical PD cases fit with Braak’s PD staging, but the scheme fails in some cases. Alzheimer’s disease, progressive supranuclear palsy, corticobasal syndrome, multiple system atrophy, frontotemporal lobar degeneration, Creutzfeldt-Jakob disease, cerebrovascular diseases, and essential tremor are common misdiagnoses for pathologically confirmed LBD. LBD exhibits considerable heterogeneity in both clinical and pathological settings, which makes clinical diagnosis challenging.

Kon, T., Tomiyama, M., and Wakabayashi, K. Neuropathology of Lewy body disease: Clinicopathological crosstalk between typical and atypical cases. 22233. 2019 Neuropathology.

Concepts Keywords
Alzheimer Multiple system atrophy
Brain Tremor
Brainstem Disease
Cerebrovascular Diseases Neurological disorders
Crosstalk Cognitive disorders
Dementia Lewy body dementia
Essential Tremor Organ systems
Heterogeneity Nervous system
Lewy Bodies Parkinson’s disease
Lewy Body Disease Psychiatric diagnosis
Multiple System Atrophy RTT
Neurites Braak staging
Neuropathology Corticobasal syndrome
Olfactory Bulbs
Parkinson
Pathology
Peripheral Nervous System
Phenotype
Phosphorylated
Prion
Progressive
Spectrum
Supranuclear Palsy

Semantics

Type Source Name
disease MESH diagnosis
disease MESH misdiagnoses
disease DOID essential tremor
disease MESH essential tremor
disease MESH cerebrovascular diseases
disease DOID Creutzfeldt-Jakob disease
disease DOID frontotemporal lobar degeneration
disease MESH frontotemporal lobar degeneration
disease DOID multiple system atrophy
disease MESH multiple system atrophy
disease DOID syndrome
disease MESH syndrome
disease DOID progressive supranuclear palsy
disease MESH progressive supranuclear palsy
disease DOID dementia
disease MESH dementia
disease DOID Lewy body disease
disease MESH Lewy body disease

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