Opsoclonus Myoclonus Ataxia Syndrome (OMAS) is a constellation of symptoms encompassing opsoclonus, myoclonus and ataxia. 1 Here we present, an adult onset OMAS following dengue viral fever.
A 39‐year‐old male patient with no comorbidities presented with acute onset high grade fever and generalized body ache for last two weeks. He tested positive for dengue non‐structural protein 1 (NS1) on the 5th day of his illness. Fever subsided on the 9th day of illness. Two days after subsidence of fever, he noted acute onset tremulousness, while approaching an object with either hand. There was a progressive worsening of tremulousness and subsequently he developed swaying to either side while walking. Over the next few days, he was unable to stand or sit by himself due to severe truncal imbalance; and also developed head titubation along with ataxic dysarthria. Abnormal intermittent involuntary jerks affecting all the four limbs, trunk and face were noticed thereafter. Relatives mentioned that his eyes were jerky and he failed to open his eyes due to these jerky movement. There was no history of any tonic clonic limb movements or loss of consciousness.
On examination he was conscious, oriented with a GCS of E4V5M6. Ocular examination revealed rapid, repetitive, chaotic, multidirectional eye movements which were non‐suppressible with fixation (Segment 1 of Video 1). Additionally, abnormal intermittent multifocal myoclonus involving distal limbs and face, which was aggravated by posture or action (Segment 1 of Video 1) were noted on examination. Cerebellar examination showed limb ataxia, dysarthria, titubation and truncal ataxia (Segment 1 of Video 1). Other cranial nerves, motor and sensory examination were unremarkable.
Video 1.
In segment 1, opsoclonus as rapid, repetitive, chaotic, multidirectional eye movements, along with severe myoclonus and ataxia at admission and segment 2 showing significant improvement after treatment.
The dengue infection was further confirmed by a dengue IgM test. His serial blood counts showed a recovering trend from the thrombocytopenia with no apparent renal or hepatic impairment. MRI brain was also within normal limits. CSF showed lymphocytic pleocytosis with 15 cells with normal sugar and proteins. Inflammatory markers. CRP (149 mg/dl) and ESR (55 mm/h) were raised. Other investigations were unremarkable.
He was initially started on intravenous (IV) methyl prednisolone (1gm/day) for 5 days. Subsequently, IV immunoglobulin (2gm/kg total dose divided in 5 days) was administered. There was significant improvement in his myoclonus and opsoclonus, with the patient being able to sit and walk without support (Segment 2 of Video 1).
As per Centres for Disease control and Preventions (CDC), worldwide each year 400 million people get infected with dengue and out of them, 100 million become sick from infection and 40,000 die from severe dengue. Dengue viral infection can have varied clinical manifestations ranging from asymptomatic infection to dengue shock syndrome. However, neurological manifestations are rare and include encephalitis, myelitis, myositis, hypokalemic paralysis, acute disseminated encephalomyelitis, Guillain‐Barré syndrome and opsoclonus myoclonus syndrome. 2
The causes of OMAS may be divided into paraneoplastic, para infectious and autoimmune conditions. In children, the most common cause of OMAS is paraneoplastic secondary to neuroblastoma, while in adults it is idiopathic and para‐infectious causes predominate. 3 The pathogenesis of OMAS is possibly immune mediated. The immune mediated nature is suggested by the clinical response to corticosteroids, IVIG, plasmapheresis and rituximab. To our best knowledge there are only six case reports available to date, all reporting a good prognosis. 4 , 5 Early identification and prompt treatment will reduce morbidity and can have good clinical outcome similar to the current case.
Author Roles
Research project: (1) A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript: A. Writing of the first draft, B. Review and Critique.
A.K.: 1A, 1B, 1C, 2A, 3A
I.H.: 2B, 3A
V.S.: 2C, 3C, 3B
D.J.:1B, 2B, 3C
Disclosures
Ethical Compliance Statement: The authors confirm that the approval of an institutional review board was not required for this work. We have taken the consent of the patient for the publication of the video. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.
Funding Sources and Conflicts of Interest: The authors report no sources of funding and no conflicts of interest.
Financial Disclosures for Previous 12 Months: The authors declare that there are no disclosures to report.
References
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