Introduction:

Opsoclonus Myoclonus Ataxia syndrome (OMA) in children is an under recognised disorder and literature on its clinical profile and outcome are sparse.

Aim:

To study the clinical profile and outcome of OMA in children.

Materials and methods:

Retrospective chart review of children with OMA diagnosed from March 2006 to October 2013.

Results and discussion:

Clinical data of 11 children with OMA were analyzed. Mean age at onset of symptoms was 26 months (±7.3). Nine children (81.8%) had post infectious cause and 2 (18.2%) children had paraneoplastic etiology. Clinical symptoms at presentation were recurrent falls, refusal to stand, tremulousness, myoclonus, eye movement abnormalities and irritability. Mean delay in diagnosis was 6 months (varying from 1 to 20 months) and it was more for post infectious etiology when compared to paraneoplastic etiology. Younger age group, extreme irritability, early eye movement abnormalities and male sex predilection were found in paraneoplastic group. In paraneoplastic group, one had well differentiated neuroblastoma in suprarenal region and the other had poorly differentiated neuroblastoma in retroperitoneal area.  Post infectious group sustained good remission with immunotherapy for prolonged periods of time. Paraneoplastic group did poorly with recurrent relapses and increased severity of symptoms in our series, although diagnosed early.

Conclusion:

Paraneoplastic OMA had increased severity of symptoms and early eye movement abnormalities. Relapses were more common and severe in paraneoplastic group.