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Lewy body

Etiology: - Parkinson's disease - diffuse Lewy body disease - Lewy bodies have also been seen in: - ataxia telangiectasia - corticobasal degeneration - Downs syndrome - familial early onset Alzheimer's disease - Hallervorden Spatz disease - motor neuron disease - multiple system atrophy - neuroaxonal dystrophy - progressive supranuclear palsy - subacute sclerosing panencephalitis Pathology: - round eosinophilic inclusions within cytoplasm of selectively vulnerable neurons - lewy bodies in the brainstem & basal forebrain are generally > 15 um in diameter with a spherical, dense hyaline core & a clear hal - lewy bodies in the cerebral cortex are smaller & lack a distinct core - cortical lewy bodies are most often seen in layers V & VI of temporal, insular & cingulate cortex & best identified with anti-ubiquitin or anti-alpha synuclein staining - the mechanism of formation of Lewy bodies & the role of Lewy bodies in the pathogenesis of Parkinson's disease are unknown * see histopathology (Aaushi.info) Structure: - components of Lewy bodies include: a) primary constituents 1] neurofilaments 2] ubiquitin, UBQLN1 3] alpha synuclein 4] clusterin 5] synphilin-1 (alpha synuclein interacting protein) 6] synaptotagmin-11 7] PACRG b) proteins representing a cellular response to Lewy bodies 1] kinases 2] phosphatases c) proteins that become entrapped during Lewy body formation - RNF19A

Related

alpha-synuclein; non-A beta component of AD amyloid; non-A4 component of amyloid; NACP (SNCA, NACP, PARK1) Lewy body dementia neurofilament protein Parkinson's disease (paralysis agitans, PD) ubiquitin (UBCEP2, UBB, UBC)

General

abnormal morphologic structure (malformation)

Properties

PATHOLOGY: Parkinson's disease Lewy body dementia

References

  1. Lang AE & Lazano AM Parkinson's disease. First of two parts. N Engl J Med. 1998 Oct 8;339(15):1044-53. PMID: 9761807
  2. Spillantini MG et al. Alpha-synuclein in Lewy bodies. Nature 388:839-40, 1997 PMID: 9278044
  3. Mezey E et al. Alpha synuclein in neurodegenerative disorders: murderer or accomplice? Nature Medicine 4:755-7, 1998 PMID: 9662355
  4. Hansen L., Dementia with Lewy bodies, In: Alzheimer's Disease, 2nd ed, Terry RD et al ed, Lippincott, Williams & Wilkins, Philadelphia, 1999

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