Objective: Opsoclonus myoclonus ataxia (OMA) is a rare neurological syndrome characterized with with autoimmune background. However the exact pathophysiology is not clear. Most common underlying causes are malignancy and paraneoplastic syndromes. Since it is more common in children with neuroblastoma, in particular, and can be rarely seen in adults, we presented this adult patient with the triad of opsoclonus, myoclonus and ataxia which responded well to topiramate.

Background: A-34 year old young man presented to our hospital with sudden-onset ataxia and involuntary movements in his eyes. He was suffering from blurred vision and sleep problems. He was extremely anxious.

Methods: His initial neurological examination revealed involuntary unpredictable, multidirectional fast eye movements without any intersaccadic intervals which were consistent with opsoclonus. There were involuntary jerks in his neck and body which was diagnosed as myoclonus, and he was unable to sit without support due to these involuntary movements. He was diagnosed as OMA. Routine laboratory investigations, markers of vasculitis and malignancy, serological investigations and cranial, cervical, toracal magnetic resonance imagings, abdominal and genitourinary computerized tomography, paraneoplastic biomarkers and PET scan were normal.

Results: Intravenous metilprednisolone (IVMP) 1 grams/day was administered but no recovery was seen at the end of 5 days. Treatment was prolonged to 10 days and topiramate with the dose of 25 milligrams/day (mg/d) was added, and tapered to 200 mg/d at the 3rd day. The patient began to walk with support, myoclonus resolved and restricted to neck at the 3rd day. 0.4 gr/day intravenous immunoglobulin (IVIG) was administered for 5 days. He was discharged without myoclonus, minimal opsoclonus, and ataxia with brief intersakkadic intervals at the end of 20 days.

Conclusions: OMA is a rare neurological syndrome with frustrating triad of symptoms including opsoclonus, myoclonus, and ataxia leading to disability. It has a rarer prevalence in adults.We presented this case to emphasize the positive effect of topiramate as an antiepileptic agent which is thought to be effective through the inhibition of glutamate mediated neurotransmission, as well as enhancing the gamma amino-butyric acid pathway in OMA syndrome.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/topiramate-responsive-adult-onset-opsoclonus-myoclonus-ataxia-syndrome/