Abstract Details

Title Clinical and imaging features of idiopathic cerebellar ataxia with anti-cerebellar antibodies

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 073

Objective The purpose of this study was to determine whether autoimmunity can account for some cases of idiopathic cerebellar ataxia (IDCA).

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

Design/Methods Using serum samples from 47 patients who met IDCA diagnostic criteria and control subjects, including 20 patients with multiple system atrophy (MSA), 13 with hereditary ataxia (HA), and 17 healthy subjects, we examined expression of anti-cerebellar antibodies (ACAs) by tissue-based immunofluorescence assay (TBA). Clinical and imaging features were compared between ACAs-positive and -negative IDCA patients. Immunoreactive patterns of ACAs-positive IDCA patient sera were examined in the cerebellar cortex and classified into subgroups by cluster analysis, dependent on immunoreactivity distribution.

Results ACAs were detected in serum samples of 16/47 (34%) patients with IDCA by TBA. This prevalence of ACAs was significantly higher compared with those of patients with MSA (10%, P = 0.037), HA (0%, P = 0.010), and healthy subjects (6%, P = 0.016). ACAs-positive IDCA patients frequently showed asymmetrical cerebellar hypoperfusion on single photon emission computed tomography (SPECT) and tended to show pure cerebellar ataxia. Cluster analysis of the distribution pattern of immunoreactivity of ACAs-positive IDCA sera identified three expression patterns: neuropil of the molecular layer, mainly intracellular regions of the granular and Purkinje cell layers, and other expression.

Conclusions

We detected ACAs in 34% of serum samples from patients with IDCA. Autoimmunity can account for some cases of IDCA. Characteristic clinical features of ACAs-positive IDCA patients involved asymmetrical cerebellar hypoperfusion on SPECT and pure cerebellar ataxia.

Authors/Disclosures
Akira Takekoshi PRESENTER	Akira Takekoshi has nothing to disclose.
Akio Kimura, MD (Gifu University Graduate School of Medicine)	Dr. Kimura has nothing to disclose.
Nobuaki Yoshikura, MD, PhD (Gifu univesity graduate school of medicine department of neurology)	Dr. Yoshikura has nothing to disclose.
Takayoshi Shimohata, MD, FAAN (Department of Neurology, Gifu University)	Dr. Shimohata has nothing to disclose.

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