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Treatment‐responsive primary autoimmune cerebellar ataxia in a patient with IgG and IgM anticerebellar antibodies
Author(s) -
Jaffe Stephen L.,
Carlson Noel G.,
Peterson Lisa K.,
Greenlee John E.
Publication year - 2021
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/ene.14659
Subject(s) - antibody , medicine , immunology , cerebellar ataxia , cerebellum , antigen , autoantibody , pathogenesis , ataxia , immunoglobulin m , intracellular , autoimmune disease , pathology , immunoglobulin g , biology , endocrinology , microbiology and biotechnology , psychiatry
Background and purpose Primary autoimmune cerebellar ataxia (PACA) in the absence of another triggering disease represents an emerging category of neurological illness. We report such a case whose ataxia was markedly responsive to plasma exchange. We analyzed patient serum for the presence of IgM or IgG anticerebellar neuronal antibodies. Methods Case presentation: rat cerebellar slice cultures incubated with patient sera were studied for IgG and IgM antibody uptake, intracellular binding, and neuronal death. Patient serum was evaluated for anti‐myelin associated glycoprotein (anti‐MAG) and associated anti‐glycolipid antibodies. Results Antibodies were taken up by viable cerebellar neurons and bound to intracellular antigens. Uptake and predominantly nuclear binding of IgG were seen in granule cells whereas cytoplasmic binding of IgM was observed predominantly in Purkinje cells. Intracellular antibody accumulation was not accompanied by neuronal death, consistent with the patient’s excellent clinical response to plasma exchange. Anti‐MAG or other associated anti‐glycolipid antibodies were not detected. Conclusions PACA may be associated with both IgG and IgM antibodies reactive with cerebellar neuronal antigens. Our patient’s response to plasma exchange supports a role for antineuronal antibodies in disease pathogenesis and emphasizes the need for rapid diagnosis and treatment.