Myoclonus-Ataxia Syndrome Associated with COVID-19

Abstract

Neurological manifestations of coronavirus disease (COVID-19) have increasingly been reported since the onset of the pandemic. Herein, we report a relatively new presentation. A patient in the convalescence period following a febrile illness with lower respiratory tract infection (fever, myalgia, nonproductive cough) presented with generalized disabling myoclonus, which is phenotypically suggestive of brainstem origin, along with additional truncal cerebellar ataxia. His neurology work-ups, such as brain MRI, electroencephalography, serum autoimmune and paraneoplastic antibody testing, were normal. His CT chest scan revealed right lower lung infiltrates, and serological and other laboratory testing did not show evidence of active infection. COVID-19 titers turned out to be strongly positive, suggestive of post-COVID-19 lung sequelae. He responded partially to antimyoclonic drugs and fully to a course of steroids, suggesting a para- or postinfectious immune-mediated pathophysiology. Myoclonusataxia syndrome appears to be a neurological manifestation of COVID-19 infection, and knowledge regarding this phenomenon should be increased among clinicians for better patient care in a pandemic situation.

Temporal Changes in Brain Perfusion in a Patient with Myoclonus and Ataxia Syndrome Associated with COVID-19

“…This is the first report of COVID-19-related myoclonus and ataxia in Japan and also the first to illustrate brain perfusion findings on SPECT in a patient with COVID-19- As in our case, no previous case reports of myoclonus and ataxia with or without opsoclonus have shown CSF pleocytosis, and PCR tests for SARS-CoV-2 in the CSF were negative in all cases tested (2,3,(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). Furthermore, brain MRI in previous case reports showed unremarkable results in almost all patients (2,3,5,(7)(8)(9)(11)(12)(13)(14)(15)(16)(17)(18)(19), except for one case that showed diffuse pachymeningeal enhancement (10). All previous cases were assumed to be associated with postinfectious or parainfectious complications of COVID-19 (2,3,(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)…”

“…Furthermore, brain MRI in previous case reports showed unremarkable results in almost all patients (2,3,5,(7)(8)(9)(11)(12)(13)(14)(15)(16)(17)(18)(19), except for one case that showed diffuse pachymeningeal enhancement (10). All previous cases were assumed to be associated with postinfectious or parainfectious complications of COVID-19 (2,3,(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19).…”

Post‐infectious cerebellar ataxia following COVID‐19 in a patient with epilepsy

“…The MRI did not show any abnormal signals, although various abnormal neuroimaging changes in SARS‐CoV‐2 have been described in the literature, possibly due to cytokine‐induced immune‐mediated tissue damage. 13 The cause of ataxia without any abnormal MRI signal in our patient is likely a post‐infective phenomenon, causing a delayed humoral or cell‐mediated immune response due to cross‐reactive cerebellar antigens with viral antibodies and/or T cells. However, other possibilities remain.…”

Autoimmune mediated hyperkinetic movement disorders in SARS-CoV-2 infection — a systematic review

“…Thus, the cerebellum remains a structure frequently affected by the pathology of autoimmune processes [68], and its participation in the pathogenesis of cortical myoclonus has been reported [69]. However, it has not yet been demonstrated that myoclonus and ataxia occur in patients with a more severe course of COVID-19 [45]. The obtained data indicates that hyperkinetic movement disorders in the course of COVID-19 are an early complication.…”

“…In the analysed case reports, myoclonus was characterised by a different course: they appeared both in generalised and isolated form, action and resting, and there were also differences in their severity. Unfortunately, few studies have analysed the exact genesis of the emerging myoclonus, and those that have done so indicated both their cortical and subcortical sources [45,55,57]. Furthermore, in some cases, the EEG record remained without clinically significant changes [24,34].…”