Multifocal Motor Neuropathy

Multifocal motor neuropathy (MMN) is a neuromuscular disease that usually presents with asymmetric weakness in a limb. The clinical pattern is that of mononeuropathy multiplex. The condition is treatable.
 

Clinical features:

  • Asymmetric weakness, usually upper limbs, spares cranial nerves, reflexes may be preserved, fasciculations may occur
  • Note this is a form of mononeuropathy multiplex

Investigations:

  • Serum Anti-GM1 IgM positive, titre >1:350
  • Nerve conduction studies NCS:
    • Multifocal motor demyelination
    • Conduction block
    • Normal sensory response

Pathology/Nerve biopsy:

  • In some cases evidence of demyelination & remylination
  • In some cases: inflammatory cells

Treatment options:

  • Intravenous immunoglobulin (IVIg)
  • In refractory cases Cyclophosphamide intravenously may be considered
  • There appears to be no benefit from Mycophenolate mofetil.

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