Abstract
Autoimmune-mediated ataxia has been associated with paraneoplastic disease, gluten enteropathy, Hashimoto thyroiditis as well as autoimmune disorders without a known associated disease. There have been relatively few reports describing the neuropathology of these conditions. This review is an attempt to consolidate those reports and determine the ways in which autoimmune ataxias can be neuropathologically differentiated from hereditary or other sporadic ataxias. In most instances, particularly in paraneoplastic forms, the presence of inflammatory infiltrates is a strong indicator of autoimmune disease, but it was not a consistent finding in all reported cases. Therefore, clinical and laboratory findings are important for assessing an autoimmune mechanism. Such factors as rapid rate of clinical progression, presence of known autoantibodies or the presence of a malignant neoplasm or other autoimmune disease processes need to be considered, particularly in cases where inflammatory changes are minimal or absent and the pathology is largely confined to the cerebellum and its connections, where the disease can mimic hereditary or other sporadic ataxias.
Highlights
The role of autoimmunity in the pathogenesis of ataxia is multifaceted
An eighth patient with small-cell carcinoma and paraneoplastic ataxia was negative for anti-Hu, but positive for anti-CV2, an antibody that recognizes the collapse in response mediator protein 5 (CRMP5) and had extensive Purkinje cell loss in the vermis with mild lymphocytic perivascular cuffing in the dentate, brainstem and spinal cord without obvious neuronal loss
There are numerous reports of clinical and laboratory studies of autoimmune ataxias, the neuropathological literature is limited to the relatively few investigations summarized in this review
Summary
The role of autoimmunity in the pathogenesis of ataxia is multifaceted. There are classical autoimmune disorders that involve the nervous system, such as multiple sclerosis [1], acute disseminated encephalomyelitis [2], Behcet disease [3], and collagen-vascular disorders [4] which, when localized to cerebellar pathways, will induce ataxic symptoms. The emphasis will be on conditions in which the cerebellar system appears to be a major, but not always an exclusive, target of the autoimmune process. The causes of autoimmune ataxias are complex and, despite the characterization of many autoantibodies associated with those conditions, the role of those antibodies as exclusive pathogenetic mediators is still uncertain, despite the tendency to categorize the diseases based on their presence. There are cases included in this review, from earlier reports, in which the diagnosis of an autoimmune ataxia was based on an association with either an underlying malignancy or autoimmune disorder, but no autoantibodies were identified. There is the possibility that some of these patients, those in whom there were no inflammatory changes in the brain, may have had a coincidental occurrence of a hereditary or other form of sporadic ataxia in conjunction with a malignant tumor or other autoimmune disorder [5]
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