Vietnamese Journal of Neurology

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Tổng quan Issue: Tạp chí thần kinh học số 38 Thần kinh miễn dịch

Immune-Mediated Cerebellar Ataxia: Clinical Diagnosis and Treatment Based on Immune Mechanisms and Pathophysiology

Published: September 26, 2023
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Abstract

This review summarizes the history, classification, and pathophysiology of immune-mediated cerebellar ataxias (IMCA), including gluten ataxia, post-infectious cerebellitis (PIC), opsoclonus-myoclonus syndrome (OMS), paraneoplastic cerebellar degeneration (PCD), anti-GAD ataxia, and primary autoimmune cerebellar ataxia (PACA). The cerebellum retains notable capacity for compensation and recovery after injury, making early immunotherapy — before an irreversible "threshold" is crossed — critical for prognosis. Specific antibodies (anti-Yo, Hu, CV2, Ri, Ma2, GAD65) help identify underlying etiologies. Treatment involves removing the triggering factor (gluten-free diet, tumor resection) combined with immunotherapy (IVIG, corticosteroids, plasma exchange, rituximab). Acetyl-DL-leucine is discussed as an adjunctive therapy for ataxic symptoms.

Keywords
immune-mediated cerebellar ataxia gluten ataxia paraneoplastic cerebellar degeneration anti-GAD ataxia opsoclonus-myoclonus syndrome post-infectious cerebellitis onconeural antibodies acetyl-DL-leucine

References

1.
Hiroshi Mitoma, Mario Manto và Marios Hadjivassiliou, “Immune-Mediated Cerebellar Ataxias: Clinical Diagnosis and Treatment Based on Immunological and Physiological Mechanism”, Journal of Movement Disorders, 14(1): 10-28, 2021.

Authors

Nguyễn Hải Anh Bệnh viện Bạch Mai; Đại học Y Dược Đại học Quốc gia Hà Nội
Immune-Mediated Cerebellar Ataxia: Clinical Diagnosis and Treatment Based on Immune Mechanisms and Pathophysiology

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Section Tổng quan
Category Thần kinh miễn dịch
Pages 69-79
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