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CLINICO-EPIDEMIOLOGICAL PROFILE OF CHILDREN WITH OPSOCLONUS MYOCLONUS ATAXIA SYNDROME: A DECADE’S EXPERIENCE FROM A TERTIARY CARE CENTRE IN NORTH INDIA

Objectives: Opsoclonus Myoclonus Ataxia Syndrome (OMAS) is a potentially treatable neuro-inflammatory disorder. The objective is to study the clinico-epidemiological profile and treatment strategies of children with OMAS.

Methods: A retrospective review of case records of children presenting with OMAS (January 2012 to March 2022) to a tertiary centre was performed.

Results: A total of 40 children (23 females, 17 males) were identified. The median age of symptom onset was 21.5 months (IQR :16.3- 24.5). 17/40 (42.5%) were non ambulatory at presentation. Underlying neuroblastoma was present in 19/40 (47.5%). In the non-tumour group (n=21), 4/21(19%) were parainfectious and 17/21 (80.9%) were idiopathic. Antineuronal antibodies (2 for anti-Hu and 1 for anti PNMA2) were detected in 7.5 % (3/40) of children and they showed early response to immunotherapy. 42.5% received IVIG+ACTH, 40% received ACTH alone,7.5% received IVIG+IV methylprednisolone pulse and 10% received only steroids as the first line therapy. Irritability (58%) was the first symptom to improve. 15 children (37.5%) showed relapse on first line therapy (median time :5 months). Relapse was treated with IVIG+ACTH in 7/13 (53.8%) ,6/13 (46%) required rituximab (375mg/m2-1.12mg/m2) and 8/13(61.5%) required long term immunosuppression. Children in the tumour group had an earlier age of onset, attained delayed remission and had more relapses in comparison to non-tumour group.

Conclusion: Early diagnosis, periodic tumour surveillance and aggressive combined immunotherapy is the key in improving outcomes.
Keywords: Opsoclonus Myoclonus Ataxia Syndrome, Anti-neuronal antibodies, IVIG, ACTH