Visceral involvement in juvenile amaurotic idiocy

  title={Visceral involvement in juvenile amaurotic idiocy},
  author={Krister Kristensson and Simon Rayner and Patrick Sourander},
  journal={Acta Neuropathologica},
The involvement of visceral organs was studied in three typical clinical and pathological cases of juvenile amaurotic idiocy (Batten-Spielmeyer-Vogt disease). Abundant intracellular deposits of a substance showing the same staining reactions and primary yellow fluorescence as that previously described in the nerve cells was found particularly in the myocardium, liver, kidneys, spleen and lymphnodes. The resemblance between this substance and lipofuscin and its different chemical composition as… CONTINUE READING