Immunocytochemical study of ballooned neurons in cortical degeneration with neuronal achromasia.
We studied the immunocytochemical characteristics of the ballooned neurons (BN) in three patients with cortical degeneration with neuronal achromasia (CDNA) using antibodies to phosphorylated neurofilaments (PNF), tau, Alz-50, ubiquitin, beta (A4) amyloid, and glial fibrillary acidic protein. All BN exhibited intense perikaryal staining for PNF protein. Most BN and some normal-appearing neurons also stained for ubiquitin and Alz-50. The BN did not immunostain for tau protein, and none of the cases had tau-reactive neocortical neurofibrillary tangles or Pick bodies. One case had occasional senile plaques that stained for beta amyloid; no case had amyloid angiopathy. Our findings suggest that the pathophysiologic basis of the cortical degeneration in CDNA involves an alteration of neuronal cytoskeletal metabolism affecting neurofilament and possibly microtubular proteins in conjunction with activation of the ubiquitin proteolytic system.
Smith
T W
TW
Department of Pathology (Neuropathology), University of Massachusetts Medical Center, Worcester 01655.
Lippa
C F
CF
de Girolami
U
U
D000368:Aged; D016229:Amyloid beta-Peptides / Q000032:analysis; D000941:Antigens / Q000032:analysis; D001921:Brain / Q000473:pathology; D004258:DNA Polymerase III; D003704:Dementia / Q000473:pathology*; D003937:Diagnosis, Differential; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D009410:Nerve Degeneration / Q000502:physiology*; D009474:Neurons / Q000473:pathology*; D009562:Nissl Bodies / Q000473:pathology*; D014157:Transcription Factors / Q000032:analysis; D014452:Ubiquitins / Q000032:analysis
C056167:Alzheimer's disease antigen; D016229:Amyloid beta-Peptides; D000941:Antigens; D005904:Glial Fibrillary Acidic Protein; D014157:Transcription Factors; D014452:Ubiquitins; C041273:transcription factor tau; D004258:DNA Polymerase III