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Light chain deposition disease restricted to the brain: The first case report

Mara Popović1, Rok Tavćar, Damjan Glavac

  • 1Institute of Pathology, Faculty of Medicine, University of Ljubljana, SI-1000 Ljubljana, Slovenia. mara.popovic@mf.uni-lj.si

Human Pathology
|October 25, 2006
PubMed

Insights

This study reports the first case of light chain deposition disease exclusively affecting the brain. The condition involved lambda light chain deposits in cerebral vessels, leading to neurological symptoms and death.

Area of Science:

  • Neuropathology
  • Immunopathology

Background:

  • The patient presented with symptoms consistent with paranoid schizophrenia, treated for 13 years.
  • In the final year of life, severe dysphagia, muscle weakness, and cognitive decline emerged.

Observation:

  • Cerebral examination revealed periventricular lesions with thickened vessels.
  • Microscopy identified eosinophilic, PAS-positive, Thioflavin T-positive deposits within vessel walls, associated with hypoxic encephalopathy.
  • Immunohistochemistry confirmed lambda light chains as the primary component of these deposits.

Findings:

  • Ultrastructural analysis showed amorphous electron-dense material in vessel walls.
  • Perivascular B-cell proliferation with monoclonal heavy chain Ig gene rearrangement was observed.
  • No systemic organ involvement or deposits were detected outside the brain.

Implications:

  • This case represents the first documented instance of light chain deposition disease (LCDD) localized solely to the brain.
  • The findings suggest a potential link between B-cell clonality and localized cerebral vascular deposition.
  • This unique presentation expands the understanding of LCDD spectrum and its potential neurological manifestations.

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