Objective: To evaluate for rare causes of Opsoclonus Myoclonus Ataxia Syndrome.

Background: Opsoclonus Myoclonus is a rare acute onset neurological disorder characterized by associated ocular, motor and behavioral symptoms. It is more common is children.. In adults it is  generally associated with a paraneoplastic etiology.

Method: We present two rare causes of Opsoclonus Myoclonus Ataxia Syndrome with their clinical presentation and evaluation in the form of two case reports.

Results: Case 1: A 63-year-old lady presented with history of fever following which she developed dysarthria with gait disturbance and jerky movements of her limbs. There was a progressive deterioration in her gait and cognition. Associated drowsiness was present. Opsoclonus with myoclonic jerks and limb ataxia was present. She was extensively evaluated for the cause of her symptoms. Autoimmune, paraneoplastic workup was negative. Whole body PET and MRI brain was normal. Serum and CSF IgM antibody testing for Dengue virus was positive. She was diagnosed to have Dengue encephalitis and started on intravenous immunoglobulins 400mg/kg/day for 5 days. She recovered completely in 3 weeks. She has been followed up for 18 months since discharge and is neurologically normal.Case 2: A 26 years old lady presented with jerky movements of trunk and limbs with oscillopsia. On examination opsoclonus, myoclonus and ataxia was present. MRI brain and CSF study was normal. NMDA antibody test was positive. In view of rapid deterioration in neurological status, she was started on intravenous immunoglobulins. Whole body PET scan revealed right ovarian teratoma. Laparoscopic excision of teratoma was done. She was kept on oral steroids and after 3 months she developed recurrence of symptoms on tapering steroids. Repeat PET scan did not reveal any recurrence of teratoma or any new lesions. She was started on injection Rituximab following which she had complete resolution of symptoms. She has been on follow up for the past one year and is neurologically normal.

Conclusion: We report two rare causes of Opsoclonus Myoclonus syndrome. Both Dengue encephalitis and NMDAR encephalitis presenting as Opsoclonus Myoclonus are extremely rare. Both patients responded well to immunomodulation and had complete resolution of neurological symptoms.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/rare-causes-of-opsoclonus-myoclonus-ataxia-syndrome/