Adult-onset Opsoclonus-Myoclonus Syndrome Associated With Ganglionic Acetylcholine Receptor Autoantibody

Neurologist. 2016 Nov;21(6):99-100. doi: 10.1097/NRL.0000000000000098.


Introduction: Opsoclonus-myoclonus syndrome (OMS) may have a toxin induced, parainfectious, or paraneoplastic etiology. Several autoantibodies have been associated with adult-onset OMS, most commonly antineuronal nuclear antibody 2 (Ri), and it is most frequently associated with breast or small cell lung cancer. The nicotinic ganglionic acetylcholine receptor autoantibody (α3-AChR Ab) has not been described in association.

Case report: A 46-year-old woman was evaluated for symptoms of oscillopsia, tremor, gait imbalance, and mild cognitive deficits that began 6 weeks prior. Neurological examination demonstrated opsoclonus, myoclonus, and mild gait ataxia. Laboratory evaluation revealed an elevated α3-AChR Ab at 0.27 nmol/L (normal ≤0.02 nmol/L) with no other autoantibodies or infectious etiology detected. Thorough screening revealed no evidence of associated malignancy. Immunotherapy with weekly methylprednisolone led to significant improvement.

Conclusions: This first reported case of α3-AChR Ab positivity in the setting of adult-onset OMS expands the spectrum of associated autoantibodies. The mechanism of disease may be linked to cholinergic nuclei within the brainstem. This case suggests including α3-AChR Ab in the evaluation of adult-onset OMS, and highlights the importance of further understanding α3-AChR within the brain.

Publication types

  • Case Reports

MeSH terms

  • Age of Onset
  • Antibodies, Antinuclear / immunology
  • Autoantibodies / immunology*
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Opsoclonus-Myoclonus Syndrome / drug therapy
  • Opsoclonus-Myoclonus Syndrome / immunology*
  • Receptors, Nicotinic / immunology*


  • Antibodies, Antinuclear
  • Autoantibodies
  • Glucocorticoids
  • Receptors, Nicotinic
  • nicotinic receptor subunit alpha3
  • Methylprednisolone