Scholarlink

<p>We present the case of a&#xa0;46-year-old woman with a&#xa0;2-year history of a&#xa0;chronic ataxic neuropathy characterized by progressive sensory deficits, gait ataxia, and ultimately ophthalmoplegia. Diagnostic work-up revealed an immunoglobulin&#xa0;M (IgM) monoclonal gammopathy and high titers of anti-GD2-IgM antibodies, leading to the diagnosis of CANOMAD syndrome (chronic ataxic neuropathy with ophthalmoplegia, monoclonal gammopathy, cold agglutinins, and disialosyl antibodies). Initial therapy with intravenous immunoglobulin (IVIG) resulted in transient improvement of ataxia and muscle strength, but the effect waned within weeks. Subsequent switch to subcutaneous immunoglobulin was unsuccessful in maintaining clinical stability. The patient’s condition was eventually stabilized with a&#xa0;combination of B‑cell depleting therapy (rituximab) and periodic IVIG during relapses. This case highlights the key points in CANOMAD management—from first presentation through evolving symptoms and delayed diagnosis to therapeutic challenges and current evidence-based strategies. Timely immunotherapy can improve neurological outcomes and prevent irreversible disability.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

CANOMAD – eine seltene immunvermittelte Neuropathie

  • 期刊论文

  • 发表日期：

    2025年10月30日

查看全文
  • Barbara Castek

摘要

We present the case of a 46-year-old woman with a 2-year history of a chronic ataxic neuropathy characterized by progressive sensory deficits, gait ataxia, and ultimately ophthalmoplegia. Diagnostic work-up revealed an immunoglobulin M (IgM) monoclonal gammopathy and high titers of anti-GD2-IgM antibodies, leading to the diagnosis of CANOMAD syndrome (chronic ataxic neuropathy with ophthalmoplegia, monoclonal gammopathy, cold agglutinins, and disialosyl antibodies). Initial therapy with intravenous immunoglobulin (IVIG) resulted in transient improvement of ataxia and muscle strength, but the effect waned within weeks. Subsequent switch to subcutaneous immunoglobulin was unsuccessful in maintaining clinical stability. The patient’s condition was eventually stabilized with a combination of B‑cell depleting therapy (rituximab) and periodic IVIG during relapses. This case highlights the key points in CANOMAD management—from first presentation through evolving symptoms and delayed diagnosis to therapeutic challenges and current evidence-based strategies. Timely immunotherapy can improve neurological outcomes and prevent irreversible disability.