H. Tomimoto et al., PARANEOPLASTIC CEREBELLAR DEGENERATION WITH A CIRCULATING ANTIBODY AGAINST NEURONS AND NONNEURONAL CELLS, Acta Neuropathologica, 86(2), 1993, pp. 206-211
We describe a woman with paraneoplastic cerebellar degeneration associ
ated 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 c
erebellum. The antigen was also present in neurons in the cerebral cor
tex, brain stem, anterior horn cells, dorsal root ganglia, intestinal
autonomic neurons, retinal ganglion cells, Schwann cells of the periph
eral nerve and epithelial cells of the renal glomerulus in rats. Immun
oelectron microscopy revealed immunoprecipitates in the smooth and rou
gh endoplasmic reticulum and polyribosomes in human and rat cerebellar
Purkinje cells and other neuronal cell bodies as well as Schwann cell
s of the peripheral nerve. Even though patients with this disorder man
ifest primarily with cerebellar and some extracerebellar signs, the an
tigen 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 de
termined.