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Opsoclonus-myoclonus syndrome — Clinical Guidelines

Overview

Opsoclonus-myoclonus syndrome (OMS) is characterized by rapid, involuntary eye movements (opsoclonus), myoclonus, cerebellar ataxia, and often associated with neuroblastoma in children or parainfectious triggers. 1 2

Diagnosis

Presence of opsoclonus and myoclonus movements

Cerebellar ataxia

Elevated serum autoantibodies against neural antigens, particularly targeting cerebellar Purkinje cells 2

Imaging or neuropathological evidence of CNS involvement 3

Management

First-line treatments: Immunosuppressive therapy (e.g., corticosteroids, intravenous immunoglobulin) 2

Adjunctive treatments: Propranolol (2 mg/kg/24 hr) for movement control 5

Anesthetic caution: Avoid agents that may exacerbate involuntary movements; monitor effects of atropine 1

Special Populations

Pediatrics: Often associated with neuroblastoma; response to immunosuppressive therapy is common 2

Comorbidities: No specific management noted for elderly or pregnant populations in provided abstracts

Key Recommendations

Initiate immunosuppressive therapy for symptomatic management in patients with OMS, particularly those with identifiable autoantibodies [Evidence: Strong 2]

Consider propanolol for controlling involuntary movements in pediatric patients with OMS [Evidence: Moderate 5]

Exercise caution with atropine and other agents that may worsen involuntary movements in OMS patients [Evidence: Weak 1]

References

1 Miyoshi H, Nakamura R, Yamaga A, Haraki T, Yasuda T, Hamada H et al.. Transient symptomatic worsening by atropine in opsoclonus-myoclonus syndrome. Pediatrics international : official journal of the Japan Pediatric Society 2017. link 2 Connolly AM, Pestronk A, Mehta S, Pranzatelli MR, Noetzel MJ. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues. The Journal of pediatrics 1997. link70272-5) 3 Mani J, Oak J, Chawla KP. Opsoclonus. The Journal of the Association of Physicians of India 1993. link 4 Sogg RL, Steinman L, Rathjen B, Tharp BR, O'Brien JS, Kenyon KR. Cherry-red spot-myoclonus syndrome. Ophthalmology 1979. link35336-2) 5 Fowler GW. Propanolol treatment of infantile polymyoclonia. Neuropadiatrie 1976. link

Opsoclonus-myoclonus syndrome