Guillain-Barré syndrome: The first documented COVID-19-triggered autoimmune neurologic disease: More to come with myositis in the offing

Neurol Neuroimmunol Neuroinflamm. 2020 Jun 9;7(5):e781. doi: 10.1212/NXI.0000000000000781. Print 2020 Sep.

Abstract

Objective: To present the COVID-19-associated GBS, the prototypic viral-triggered autoimmune disease, in the context of other emerging COVID-19-triggered autoimmunities, and discuss potential concerns with ongoing neuroimmunotherapies.

Methods: Eleven GBS cases in four key COVID-19 hotspots are discussed regarding presenting symptoms, response to therapies and cross-reactivity of COVID spike proteins with nerve glycolipids. Emerging cases of COVID-19-triggered autoimmune necrotizing myositis (NAM) and encephalopathies are also reviewed in the context of viral invasion, autoimmunity and ongoing immunotherapies.

Results: Collective data indicate that in this pandemic any patient presenting with an acute paralytic disease-like GBS, encephalomyelitis or myositis-even without systemic symptoms, may represent the first manifestation of COVID-19. Anosmia, ageusia, other cranial neuropathies and lymphocytopenia are red flags enhancing early diagnostic suspicion. In Miller-Fisher Syndrome, ganglioside antibodies against GD1b, instead of QG1b, were found; because the COVID-19 spike protein also binds to sialic acid-containing glycoproteins for cell-entry and anti-GD1b antibodies typically cause ataxic neuropathy, cross-reactivity between COVID-19-bearing gangliosides and peripheral nerve glycolipids was addressed. Elevated Creatine Kinase (>10,000) is reported in 10% of COVID-19-infected patients; two such patients presented with painful muscle weakness responding to IVIg indicating that COVID-19-triggered NAM is an overlooked entity. Cases of acute necrotizing brainstem encephalitis, cranial neuropathies with leptomeningeal enhancement, and tumefactive postgadolinium-enhanced demyelinating lesions are now emerging with the need to explore neuroinvasion and autoimmunity. Concerns for modifications-if any-of chronic immunotherapies with steroids, mycophenolate, azathioprine, IVIg, and anti-B-cell agents were addressed; the role of complement in innate immunity to viral responses and anti-complement therapeutics (i.e. eculizumab) were reviewed.

Conclusions: Emerging data indicate that COVID-19 can trigger not only GBS but other autoimmune neurological diseases necessitating vigilance for early diagnosis and therapy initiation. Although COVID-19 infection, like most other viruses, can potentially worsen patients with pre-existing autoimmunity, there is no evidence that patients with autoimmune neurological diseases stable on common immunotherapies are facing increased risks of infection.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases of the Nervous System / diagnosis
  • Autoimmune Diseases of the Nervous System / etiology
  • Autoimmune Diseases of the Nervous System / immunology
  • Betacoronavirus* / immunology
  • COVID-19
  • Coronavirus Infections / complications*
  • Coronavirus Infections / diagnosis*
  • Coronavirus Infections / immunology
  • Female
  • Guillain-Barre Syndrome / diagnosis*
  • Guillain-Barre Syndrome / etiology*
  • Guillain-Barre Syndrome / immunology
  • Humans
  • Male
  • Myositis / diagnosis*
  • Myositis / etiology*
  • Myositis / immunology
  • Pandemics
  • Pneumonia, Viral / complications*
  • Pneumonia, Viral / diagnosis*
  • Pneumonia, Viral / immunology
  • SARS-CoV-2