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Table 4 First-line immunotherapy for the main subtypes of immune-mediated cerebellar ataxia

From: Immune-mediated cerebellar ataxias: from bench to bedside

Gluten ataxia

 Induction and maintenance therapies: strict gluten-free diet

 Immunosuppressants or IVIg for patients who show no improvement or are negative for gluten-related antibodies

Paraneoplastic cerebellar degeneration

 Early removal of neoplasm is the first objective of treatment, followed by induction therapy (mPSL, IVIg, immunosuppressants, or/and plasma exchange). Discussion according to associated Abs. Long-term oral PSL, IVIg, immunosuppressants for maintenance therapy

Anti-GAD65 Ab-associated cerebellar ataxia

 Induction therapy: mPSL, IVIg, immunosuppressants, plasma exchange, or/and rituximab

  Maintenance therapy: continuous oral PSL, IVIg, immunosuppressants, or/and rituximab

  1. Modified from Mitoma and Manto (2016) [14]
  2. Abbreviations: Abs antibodies, mPSL intravenous methylprednisolone, oral PSL oral prednisolone, IVIg intravenous immunoglobulins