Paraneoplastic cerebellar degeneration with a circulating antibody against neurons and non-neuronal cells.
We describe a woman with paraneoplastic cerebellar degeneration associated with para-ovarian adenocarcinoma, who had a circulating antibody with a corresponding antigen not only in cerebellar Purkinje cells but also in neurons located in the molecular layer of the human and rat cerebellum. The antigen was also present in neurons in the cerebral cortex, brain stem, anterior horn cells, dorsal root ganglia, intestinal autonomic neurons, retinal ganglion cells, Schwann cells of the peripheral nerve and epithelial cells of the renal glomerulus in rats. Immunoelectron microscopy revealed immunoprecipitates in the smooth and rough endoplasmic reticulum and polyribosomes in human and rat cerebellar Purkinje cells and other neuronal cell bodies as well as Schwann cells of the peripheral nerve. Even though patients with this disorder manifest primarily with cerebellar and some extracerebellar signs, the antigen also exists in many neurons other than cerebellar Purkinje cells and even in non-neuronal cells. The clinicopathologic significance of the observed immunologic reaction in diverse neurons remains to be determined.
Tomimoto
H
H
Department of Neurology, Mayo Clinic, Rochester, MN 55905.
Brengman
J M
JM
Yanagihara
T
T
D000230:Adenocarcinoma / Q000150:complications / Q000473:pathology / Q000556:secondary; D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D000912:Antibodies, Neoplasm / Q000032:analysis / Q000276:immunology*; D015153:Blotting, Western; D002526:Cerebellar Diseases / Q000150:complications / Q000473:pathology*; D002528:Cerebellar Neoplasms / Q000150:complications / Q000473:pathology / Q000556:secondary; D002531:Cerebellum / Q000648:ultrastructure; D002540:Cerebral Cortex / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology / Q000378:metabolism; D009474:Neurons / Q000276:immunology* / Q000648:ultrastructure; D010051:Ovarian Neoplasms / Q000473:pathology; D010257:Paraneoplastic Syndromes / Q000276:immunology / Q000473:pathology*; D011689:Purkinje Cells / Q000276:immunology / Q000648:ultrastructure; D051381:Rats; D017208:Rats, Wistar; D013116:Spinal Cord / Q000473:pathology; D013347:Subcellular Fractions / Q000378:metabolism
D000912:Antibodies, Neoplasm; D009419:Nerve Tissue Proteins