Lewy body

Lewy bodies are inclusion bodies – abnormal aggregations of protein – that develop inside neurons affected by Parkinson's disease, the Lewy body dementias (Parkinson's disease dementia and dementia with Lewy bodies (DLB)), and in several other disorders such as multiple system atrophy.[1] The defining proteinaceous component of Lewy bodies is alpha-synuclein (α-synuclein), which aggregates to form Lewy bodies within neuronal cell bodies, and Lewy neurites in neuronal processes (axons or dendrites). In some disorders, alpha-synuclein also forms aggregates in glial cells that are referred to as 'glial cytoplasmic inclusions'; together, diseases involving Lewy bodies, Lewy neurites and glial cytoplasmic inclusions are called 'synucleinopathies'.[2]

Lewy bodies appear as spherical masses in the neuronal cytoplasm that can displace other cellular components such as the nucleus and neuromelanin.[3] A given neuron may contain one or more Lewy bodies.[4] There are two main kinds of Lewy bodies – classical (brainstem-type) and cortical-type.[5] Classical Lewy bodies occur most commonly in pigmented neurons of the brainstem, such as the substantia nigra and locus coeruleus, although they are not restricted to pigmented neurons. They are strongly eosinophilic structures ranging from 8-30 microns in diameter, and when viewed with a light microscope they are seen to consist of a dense core that is often surrounded by a pale shell.[5] Electron microscopic analyses found that the core consists of a compact mass of haphazard filaments and various particles surrounded by a diffuse corona of radiating filaments.[4][3] In contrast, cortical-type Lewy bodies are smaller, only faintly eosinophilic, and devoid of a surrounding halo with radial filaments.[5][6] Cortical-type Lewy bodies occur in multiple regions of the cortex and in the amygdala.[5] Cortical Lewy bodies are a distinguishing feature of dementia with Lewy bodies, but they may occasionally be seen in ballooned neurons characteristic of behavioural variant frontotemporal dementia and corticobasal degeneration,[7] as well as in patients with other tauopathies.[8]

  1. ^ Jellinger KA (September 2007). "More frequent Lewy bodies but less frequent Alzheimer-type lesions in multiple system atrophy as compared to age-matched control brains". Acta Neuropathologica. 114 (3): 299–303. doi:10.1007/s00401-007-0227-4. PMID 17476513. S2CID 32406286.
  2. ^ Koga S, Sekiya H, Kondru N, Ross OA, Dickson DW (2021). "Neuropathology and molecular diagnosis of synucleinopathies". Molecular Neurodegeneration. 16 (1) 83. doi:10.1186/s13024-021-00501-z. PMC 8684287. PMID 34922583.
  3. ^ a b Revesz, Tamas; Gray, Francoise; Scaravilli, Francesco (2001). "Chapter 9: Parkinson's Disease". In Duckett, Serge; De La Torre, JC (eds.). Pathology of the Aging Human Nervous System. Oxford University Press. pp. 264–308. ISBN 0-19-513069-3.
  4. ^ a b Duchen, LW (1984). "Chapter 1: General Pathology of Neurons and Neuroglia". In Adams, J Hume; Corsellis, JAN; Duchen, LW (eds.). Greenfield's Pathology (Fourth ed.). John Wiley and Sons. pp. 1–52. ISBN 0-471-82307-4.
  5. ^ a b c d Vonsattel, Jean Paul G; Hedley-White, E Tessa (2001). "Chapter 7: Dementia". In Duckett, Serge; De La Torre, JC (eds.). Pathology of the Aging Human Nervous System. Oxford University Press. pp. 156–206. ISBN 0-19-513069-3.
  6. ^ Mrak, Robert E.; Griffin, W Sue T (October 2007). "Dementia with Lewy bodies: Definition, diagnosis, and pathogenic relationship to Alzheimer's disease". Neuropsychiatric Disease and Treatment. 3 (5): 619–625. PMC 2656298. PMID 19300591.
  7. ^ Dickson DW, Feany MB, Yen SH, Mattiace LA, Davies P (1996). "Cytoskeletal pathology in non-Alzheimer degenerative dementia: New lesions in Diffuse Lewy body disease, Pick's disease, and Corticobasal Degeneration". New Trends in the Diagnosis and Therapy of Non-Alzheimer's Dementia. Journal of Neural Transmission Supplement. Vol. 47. pp. 31–46. doi:10.1007/978-3-7091-6892-9_2. ISBN 978-3-211-82823-6. PMID 8841955.
  8. ^ Popescu A, Lippa CF, Lee VM, Trojanowski JQ (December 2004). "Lewy bodies in the amygdala: increase of alpha-synuclein aggregates in neurodegenerative diseases with tau-based inclusions". Archives of Neurology. 61 (12): 1915–1919. doi:10.1001/archneur.61.12.1915. PMID 15596612.
This article is issued from Wikipedia. The text is available under Creative Commons Attribution-Share Alike 4.0 unless otherwise noted. Additional terms may apply for the media files.