Objective: The current study sought to determine whether autoimmunity can account for some cases of IDCA.

Background: Idiopathic cerebellar ataxia (IDCA) is the clinical-based term for sporadic cerebellar ataxia with insidious onset and a slowly progressive course. Despite its heterogeneous pathogenesis, diagnostic criteria for IDCA were recently proposed, based on criteria for sporadic adult-onset cerebellar ataxia of unknown etiology with slight modifications.

Method: Using tissue-based immunofluorescence assay, we examined the expression of anti-cerebellar antibodies (ACAs) in serum samples from 47 patients who met the IDCA diagnostic criteria and control subjects, including 20 patients with multiple system atrophy, 13 with hereditary ataxia, and 17 healthy subjects. Clinical and imaging features were compared between ACA-positive and ACA-negative IDCA patients.

Results: ACAs were detected in serum samples of 34% patients with IDCA. This ACA prevalence was significantly higher than that among patients with multiple system atrophy (10%, P = 0.037), hereditary ataxia (0%, P = 0.010), or healthy subjects (6%, P = 0.016). ACA-positive IDCA patients frequently showed asymmetrical cerebellar hypoperfusion on single-photon emission computed tomography (SPECT) and tended to show pure cerebellar ataxia. The degree of disability in IDCA patients with a neuropil staining pattern of the molecular layer was more severe than that of patients with an intracellular staining pattern of Purkinje cells.

Conclusion: We detected ACAs in 34% of IDCA patients. Autoimmunity can account for some cases of IDCA. Characteristic clinical features of ACA-positive IDCA patients included asymmetrical cerebellar hypoperfusion on SPECT and pure cerebellar ataxia.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-and-imaging-features-of-idiopathic-cerebellar-ataxia-with-anti-cerebellar-antibodies/