Myoclonus and cerebellar ataxia associated with COVID-19: a case report and systematic review

Abstract

Background Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic in December 2019, neurological manifestations have been recognized as potential complications. Relatively rare movement disorders associated with COVID-19 are increasingly reported in case reports or case series. Here, we present a case and systematic review of myoclonus and cerebellar ataxia associated with COVID-19. Methods A systematic review was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guideline using the PubMed and Ovid MEDLINE databases, from November 1, 2019 to December 6, 2020. Results 51 cases of myoclonus or ataxia associated with COVID-19, including our case, were identified from 32 publications. The mean age was 59.6 years, ranging from 26 to 88 years, and 21.6% were female. Myoclonus was multifocal or generalized and had an acute onset, usually within 1 month of COVID-19 symptoms. Myoclonus occurred in isolation (46.7%), or with ataxia (40.0%) or cognitive changes (30.0%). Most cases improved within 2 months, and treatment included anti-epileptic medications or immunotherapy. Ataxia had an acute onset, usually within 1 month of COVID-19 symptoms, but could be an initial symptom. Concurrent neurological symptoms included cognitive changes (45.5%), myoclonus (36.4%), or a Miller Fisher syndrome variant (21.2%). Most cases improved within 2 months, either spontaneously or with immunotherapy. Conclusions This systematic review highlights myoclonus and ataxia as rare and treatable post-infectious or para-infectious, immune-mediated phenomena associated with COVID-19. The natural history is unknown and future investigation is needed to further characterize these movement disorders and COVID-19. Supplementary Information The online version contains supplementary material available at 10.1007/s00415-021-10458-0.

Symptoms of gait and coordination impairment in a patient with COVID-19 interstitial pneumonia

“…Encephalopathies, which are apparently linked to COVID-19 infection, seem to share a coordination and gait impairment, denoting a cerebellar syndrome. Poor coordination and ataxia have been extensively described as rare and treatable post-infectious or para-infectious, immune-mediated phenomena associated with COVID-19 [2]. In some cases, opsoclonus and myoclonus have also been described in association with ataxia, denoting a specific condition named opsoclonus-myoclonus-ataxia syndrome (OMAS): this is a rare disorder that is thought to be immune-mediated, with primarily paraneoplastic or para-infectious etiologies [2].…”

Post-acute COVID-19 functional movement disorder

“… 4 Recently, Chan et al reported a case and systematically reviewed the clinical features of 51 cases of myoclonus presenting within 1 month of COVID-19 symptoms with associated ataxia in 40% of patients and cognitive changes in 30% of patients. 5 The likely pathophysiology behind the myoclonus was presumed to be parainfectious or neuro-inflammatory and most cases improved with immunotherapy or spontaneously within 2 months of onset. 4 , 5 The onset of movement disorder in our patient was 2 months after initial COVID-19 symptoms without any improvement or worsening with time.…”

“… 1 COVID-19-related movement disorders include myoclonus, opsoclonus, and ataxia and are presumed to be parainfectious or post-infectious in etiology. 4 , 5 Functional movement disorders (FMDs) are also being reported after COVID-19 infection as well after COVID-19 vaccination. 6 We want to report a case of a 54-year-old man who presented to post-COVID clinic at the University of Iowa with abnormal repetitive movement of the head, which started 2 months after SARS-CoV-2 infection.…”

Neurological update: COVID-19

“…Whilst these disorders of coagulation and endothelial integrity represent potential drivers of large vessel cerebrovascular disease, they also raise the possibility that impairment of the microvasculature might be involved in some of the less clear-cut neurological presentations, such as prolonged disorders of consciousness (including akinetic-mutism states) [63,[100][101][102], myoclonus (a relatively common complication with over 50 cases reported in the literature, which is seemingly unrelated to hypoxic ischaemic brain injury [103]), or even on the long-term cognitive outcomes of those who recover. Indeed, neuroimaging studies have commonly described cerebral microhaemorrhages occurring in those with severe COVID-19, reminiscent of microangiopathic disorders such as amyloid and hypertensive angiopathies [100,104,105], a phenomenon borne out in pathology series [32,75,100,106].…”