Objective: To investigate whether the encephalopathy associated with the opsioclonus myoclonus syndrome in COVID-19 is likely to be parainfections.

Background: Adult onset opsoclonus myoclonus syndrome (OMS) is rare and is usually paraneoplastic in aetiology. However, para-infectious and idiopathic cases have been well documented. OMS associated encephalopathy is said to occur in the paraneoplastic cases. Emerging literature suggests that OMS may be part of the spectrum of neurological syndromes associated with COVID-19. A recent series of 7 patients with COVID-19 associated OMS have highlighted the absence of encephalopathy, although COVID-19 associated encephalopathy with hyposmia, hyposomnia and myoclonus has also been reported in one series.

Method: We studied the clinical features, investigation results and outcomes of two patients who presented with the opsoclonus myoclnus syndrome following testing positive for covid-19.

Results: We report two patients in whom SARS-CoV-2 infection was followed within two weeks by a severe opsoclonus myoclonus syndrome with marked encephalopathy and ataxia. MRI head scan and CSF examination were unremarkable in both and metabolic and drug aetiologies for the encephalopathy were not found. Extensive investigations for associated malignancy were also negative. Both cases responded well to a combination of immunotherapy and symptomatic treatment.

Conclusion: We conclude that COVID-19 can be associated with OMS, and that this may be associated with a severe encephalopathy. The mechanism by which SARS-CoV-2 may trigger OMS is uncertain, although the time course of the syndrome in relation to the viral illness would support a parainfectious aetiology. Further systematic case descriptions and prospective surveillance studied are required to characterise these syndromes in more detail.

References: 1. Klaas JP, Ahlskog JE, Pittock SJ, et al. Adult-Onset Opsoclonus-Myoclonus Syndrome. Arch Neurol. 2012;69(12):1598–1607. 2. Rábano-Suárez P, Bermejo-Guerrero L, Méndez-Guerrero A, ParraSerrano J, Toledo-Alfocea D, Sánchez-Tejerina D, SantosFernández T, Folgueira-López MD, Gutiérrez Gutiérrez J, AyusoGarcía B (2020) Generalized myoclonus in COVID-19. Neurol. 3. Emamikhah, M., Babadi, M., Mehrabani, M. et al. Opsoclonus-myoclonus syndrome, a post-infectious neurologic complication of COVID-19: case series and review of literature. J. Neurovirol. 27, 26–34 (2021). 4. Ellul MA, Benjamin L, Singh B, Lant S, Michael BD, Easton A, Kneen R, Defres S, Sejvar J, Solomon T. Neurological associations of COVID-19. Lancet Neurol. 2020 Sep;19(9):767-783. 5. Ellul M, Varatharaj A, Nicholson TR, Pollak TA, Thomas N, Easton A, Zandi MS, Manji H, Solomon T, Carson A, Turner MR, Kneen R, Galea I, Pett S, Thomas RH, Michael BD; CoroNerve Steering Committee. Defining causality in COVID-19 and neurological disorders. J Neurol Neurosurg Psychiatry. 2020 Aug;91(8):811-812.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/covid-19-associated-opsoclonus-myoclonus-syndrome-with-encephalopathy/