ICNC Abstracts, ICNC 2018

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Opsoclonus myoclonus ataxia syndrome: A study from a tertiary care center in Western India
leeha Singh, Shilpa Kulkarni, Anaita Hegde, K. N. Shah

Last modified: 2018-09-09

Abstract


INTRODUCTION: The opsoclonus-myoclonus ataxia syndrome (OMAS) also called “Kinsbourne syndrome” or “dancing eye syndrome” is a rare but serious disorder characterized by opsoclonus, myoclonus and ataxia along with extreme irritability and behavioral changes. It can be idiopathic, parainfectious or paraneoplastic with tumors of the neural crest. Indian data on its epidemiology, clinical features and outcome is limited. The aim of our study was to investigate the epidemiology, clinical features, etiological aspects, treatment and outcome of OMAS in Indian children.

METHODS: Data on clinical, etiological and treatment response in children diagnosed with OMAS over a period of 2 years (2015-2017) were analyzed.

RESULTS: Total 15 patients (9 girls  and 6 boys) were included. Median age of onset was 15 months (range: 3 months – 3.5 years). Time to diagnosis ranged between 10 days to 12 months. Median time lag between onset and treatment was 3 months. Median follow-up period was 2 years (range: 2 months - 6.5 years). Acute ataxia was the preponderant inaugural feature. Neural crest tumors were the most common tumors (n=11).  In "No tumor group" (n=4), parainfectious (n=1) and idiopathic (n=3) OMS were identified. All but one patient received immunomodulatory treatment. Complete recovery of OMS symptoms was obtained in 8 (61%) children. 2 patients had relapse of symptoms. There was no correlation between grade of tumor and response to treatment.

CONCLUSION: With early initiation of immunomodulatory treatment in the course of illness better outcome is noted. Idiopathic OMAS has poor outcome.


Keywords


Opsoclonus myoclonus ataxia, outcome

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