Adult neuronal ceroid-lipofuscinosis--personal observations

A workshop on the diagnostic criteria of adult neuronal ceroid-lipofuscinosis (ANCL) or Kufs' disease represented an unique opportunity to make a critical review of the ANCL material from the files of the Born-Bunge Foundation. Our review included the clinical data when available, the light microscopic sections and the electron micrographs. When the postmortem material had not been immediately fixed in glutaraldehyde, we sampled formalin-fixed material, post-fixed it and used classical electron microscopic techniques to better define the ultrastructure of the stored products. Only one family fulfilled the criteria for ANCL Neurons and vascular smooth muscle cells contained membrane-bound inclusions with curvilinear, rectilinear and fingerprint profiles.