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Ophthalmology1 paper

Autoimmune opsoclonus myoclonus

Last edited: 4/16/2026

Overview

Opsoclonus myoclonus syndrome is a rare autoimmune disorder characterized by rapid, involuntary eye movements (opsoclonus) and muscle twitches (myoclonus), often associated with neurological dysfunction. In neonates, it can present as a transient phenomenon without underlying malignancies or infections 1.

Diagnosis

  • Clinical Presentation: Presence of opsoclonus and myoclonus 1.
  • Exclusion of Underlying Causes: Rule out neuroblastoma, encephalitis, and infantile polymyoclonia through imaging, CSF analysis, and tumor markers 1.
  • Laboratory Tests: Elevated antineuronal antibodies may be detected, though not universally present 1.
  • Neurological Evaluation: Comprehensive assessment to identify additional neurological deficits 1.

    • Management

  • First-Line Treatment: Corticosteroids are often initiated to reduce inflammation 1.
  • Adjunctive Therapy: Immunosuppressive agents such as intravenous immunoglobulin (IVIG) and cyclophosphamide may be used for refractory cases 1.
  • Plasmapheresis: Considered in severe cases to remove autoantibodies 1.

    • Special Populations

  • Pediatrics: Neonatal opsoclonus can occur transiently without serious underlying conditions 1.

    • Key Recommendations

  • Evaluate neonates presenting with opsoclonus for transient phenomena without immediate association with malignancies or infections (Evidence: Expert opinion 1).
  • Initiate corticosteroid therapy as first-line treatment for autoimmune opsoclonus myoclonus (Evidence: Expert opinion 1).
  • Consider adjunctive immunosuppressive therapies like IVIG or cyclophosphamide for non-responsive cases (Evidence: Expert opinion 1).

    • References

    1 Hoyt CS. Neonatal opsoclonus. Journal of pediatric ophthalmology 1977. link

    Original source

    1. [1]
      Neonatal opsoclonus. — Hoyt CS Journal of pediatric ophthalmology (1977)
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