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Background and aim <p>Anti-myelin-associated glycoprotein (anti-MAG) neuropathy is typically a chronic, progressive, predominantly sensory distal and demyelinating neuropathy, with ataxia and postural tremor</p> Methods and results <p>Herein we describe an atypical case of anti-MAG neuropathy, characterized by acute lower limb weakness and severe ataxia with difficulty in stance and walking, resembling a Guillain-Barrè Syndrome. The presence of disproportionate distal nerve conduction slowing, and an IgM k monoclonal component have arisen the suspect of anti-MAG neuropathy, confirmed by high titer anti-MAG antibody. Rituximab treatment was started, and patient experienced a dramatic clinical worsening which was rescued by Plasma Exchange.</p> Interpretation <p>We described an atypical case of anti-MAG neuropathy that was challenging in diagnosis and therapeutic management.</p>

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Acute onset anti-MAG neuropathy and paradoxical worsening to rituximab: a challenging case

  • 期刊论文

  • 发表日期：

    2025年3月7日

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  • Emanuele Cassano,
  • Rosa Iodice,
  • Isabella Di Sarno,
  • Roberta Bencivenga,
  • Fiore Manganelli,
  • Stefano Tozza

摘要

Background and aim

Anti-myelin-associated glycoprotein (anti-MAG) neuropathy is typically a chronic, progressive, predominantly sensory distal and demyelinating neuropathy, with ataxia and postural tremor

Methods and results

Herein we describe an atypical case of anti-MAG neuropathy, characterized by acute lower limb weakness and severe ataxia with difficulty in stance and walking, resembling a Guillain-Barrè Syndrome. The presence of disproportionate distal nerve conduction slowing, and an IgM k monoclonal component have arisen the suspect of anti-MAG neuropathy, confirmed by high titer anti-MAG antibody. Rituximab treatment was started, and patient experienced a dramatic clinical worsening which was rescued by Plasma Exchange.

Interpretation

We described an atypical case of anti-MAG neuropathy that was challenging in diagnosis and therapeutic management.