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Autosomal dominant diffuse Lewy body disease

K Wakabayashi1, S Hayashi, A Ishikawa

  • 1Brain Disease Research Center, Brain Research Institute, Niigata University, Japan. koichi@bri.niigata-u.ac.jp

Acta Neuropathologica
|August 15, 1998
PubMed
Summary

This study identifies a Japanese family with autosomal dominant diffuse Lewy body disease (DLBD), characterized by early-onset parkinsonism and dementia. Pathological findings reveal widespread Lewy bodies and neuronal loss, suggesting a genetic basis for DLBD.

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Area of Science:

  • Neurology
  • Genetics
  • Pathology

Background:

  • Parkinsonism and dementia are complex neurological disorders.
  • Lewy body disease (LBD) is a common finding in neurodegenerative conditions.
  • Autosomal dominant inheritance patterns are crucial for understanding familial neurological diseases.

Observation:

  • A Japanese family presented with a unique combination of parkinsonism and later-onset dementia.
  • Pathological examination revealed significant neuronal loss with Lewy bodies (LBs) in the brainstem and cortex.
  • Amyloid plaques were also noted in the proband, alongside ubiquitin-positive hippocampal neurites.

Findings:

  • Two generations exhibited parkinsonism and dementia, with onset in one son as early as 28 years.
  • Autosomal dominant inheritance was suggested by the family's presentation.

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  • Apolipoprotein E genotypes varied (ε3/4 and ε4/4), but did not preclude the diagnosis.

    • Implications:

    • This family's condition strongly suggests autosomal dominant diffuse Lewy body disease (DLBD).
    • Understanding the genetic underpinnings of DLBD can aid in diagnosis and therapeutic strategies.
    • Further research into the genetic factors contributing to DLBD in this family is warranted.