Steroid-responsive multifocal motor neuropathy with cranial manifestations - a case report

Acta Neurol Taiwan. 2024 Mar 30:33(1):28-35.

Abstract

The typical presentation of multifocal motor neuropathy (MMN) is progressive asymmetric limb weakness. Cranial neuropathy is rare. We report a 28-year-old woman with cranial and bulbar palsies but with typical electrophysiological features of MMN by multifocal motor conduction blocks and serological markers of anti-ganglioside GM1 antibodies. The previous consensus on the treatment of MMN is intravenous immunoglobulins, but our patient responded to oral steroids and had clinical and electrophysiological improvement under continuous low-dose prednisolone treatment. In summary, MMN is a treatable chronic inflammatory disease of peripheral nerves. Cranial neuropathies can be its initial presentations. Electromyography studies are crucial for MMN diagnosis and helpful in monitoring disease activity and treatment responses. Although the previous guideline did not suggest using steroids for MMN, with careful patient selection, low-dose oral steroids can be an effective treatment in patients with relatively minor symptoms. Keywords: Multifocal motor neuropathy, conduction block, bulbar palsy, cranial nerve, cranial neuropathy.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bulbar Palsy, Progressive* / drug therapy
  • Cranial Nerve Diseases* / drug therapy
  • Cranial Nerve Diseases* / etiology
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Motor Neuron Disease* / diagnosis
  • Motor Neuron Disease* / drug therapy
  • Neural Conduction / physiology
  • Polyneuropathies* / drug therapy
  • Steroids / therapeutic use

Substances

  • Immunoglobulins, Intravenous
  • Steroids