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

Structure

• components of Lewy bodies include:
  • primary constituents
    • neurofilaments
    • ubiquitin, UBQLN1
    • alpha synuclein
    • clusterin
    • synphilin-1 (alpha synuclein interacting protein)
    • synaptotagmin-11
    • PACRG
  • proteins representing a cellular response to Lewy bodies
    • kinases
    • phosphatases
  • proteins that become entrapped during Lewy body formation
    • RNF19A

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)

More general terms

• abnormal morphologic structure (malformation)

Additional terms

• 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)