Case Report Highlights Diagnosis of Antibody-Positive NMOSD After Second COVID-19 Vaccination


A rare case report illustrated an 80-year-old man with aquaporin-4 antibody-positive NMOSD following a second dose of the Pfizer-BioNTech COVID-19 mRNA BNT162b2 vaccine.

In a recent case report study, an 80-year-old man was diagnosed with seropositive neuromyelitis optica spectrum disorder (NMOSD) after receiving a second dose of Pfizer-BioNTech’s mRNA BNT162b2 COVID-19 vaccine. The case report demonstrated and raised awareness that there is a risk of a possible severe adverse event in older adults after the COVID-19 vaccination.

The MRI of the spine revealed a longitudinally extensive transverse myelitis from T3–T4 to T9–T10. Additionally, testing for serum antibody revealed positive aquaporin-4 (AQP4) antibodies that remained positive on repeated testing after 2 weeks.

The individual completed five sessions of plasma exchange (PLEX) in which following, his lower extremity power improved to 5/5 on the right and 4 + /5 on the left. He was treated with prednisone 40 mg daily and then was given mycophenolate mofetil with a slow steroid wean. By the third month follow-up visit, the participant denied having any new symptoms and reported improvement overall.

Lead author Stephanie Kuntz, MD, neurology resident in the department of medicine at St. Michael’s Hospital and University of Toronto, and colleagues wrote, “This case report adds to the existing literature and suggests that COVID-19 vaccinations may trigger de novo NMOSD or NMOSD relapses in some individuals. Although rare, our patient presented with new-onset NMOSD in his 80s following COVID-19 vaccination.”1

The participant, a South Asian man in his early 80s, had no history of neurological symptoms and no baseline disability prior to his second vaccination for COVID-19. The first dose was given in the spring of 2021 with no complications and then the second dose was given in early summer 2021. After 2 days of receiving his second dose, he attended the St. Michael’s Hospital in Toronto because of worsening of weakness and numbness, which led to multiple falls. At the hospital, he was diagnosed with seropositive NMOSD and treated with intravenous methylprednisolone.

“As such, it is relevant to consider AQP4 testing in those presenting with a post-vaccination myelitis, regardless of age. Ongoing vaccine surveillance and research are needed to understand the risk of NMOSD post-COVID-19 vaccinations further,” Kuntz et al noted.1

Visual Field Defect Higher in Patients with NMOSD-ON Than Idiopathic ON

Results from a prospective study demonstrated a higher visual field defect in patients with neuromyelitis optica spectrum disorder-optic neuritis compared with those who had idiopathic optic neuritis.

Although current evidence suggest in some cases vaccinations may be implicated, triggers for central nervous system inflammatory disease such as NMOSD need more research to be conducted in as it is not well understood. For instance, one study demonstrated that vaccinations were estimated to carry an overall risk of 0.1% for triggering central nervous system inflammatory diseases.2 Immunizations for influenza, for example, have been associated with NMOSD onset or relapses and the risk of an NMOSD relapse after vaccination shows to be most observed in patients who are not on preventative immunotherapy.3

AQP4 antibodies may be observed long before NMOSD onset according to prior evidence, which suggests that AQP4 antibody carriers can be asymptomatic for longer time periods.4 “Theoretically, these individuals may be particularly susceptible to developing clinical symptoms when faced with a possible trigger,” Kuntz and colleagues wrote.1

Additionally, the risk of central nervous system disease post-vaccination is lower in comparison with rates following infections that vaccines were aimed to protect against. Vaccination may trigger disease activity with an individual who has underlying susceptibility although this case report cannot prove causality.

Kuntz et al noted, “In the era of the COVID-19 pandemic with increasing vaccination rates worldwide, vaccine safety remains at the forefront of discussion. Neurological symptoms which have been reported in the Centers for Disease Control Vaccine Adverse Event Reporting System include dizziness, headache, pain, muscle spasms, myalgia, and paresthesias as well as rare cases of tremor, diplopia, tinnitus, dysphonia, seizures, and reactivation of herpes zoster. With regards to NMOSD, there is very limited data linking COVID-19 vaccinations with disease onset.”1

1. Kuntz S, Saab G, Schneider R. Antibody-Positive Neuromyelitis Optica Spectrum Disorder After Second COVID-19 Vaccination: a Case Report. SN Compr Clin Med. 2022;4(1):130. doi:10.1007/s42399-022-01213-1
2. Karussis D, Petrou P. The spectrum of post-vaccination inflammatory CNS demyelinating syndromes. Autoimmun Rev. 2014;13(3):215-224. doi:10.1016/j.autrev.2013.10.003
3. Mealy MA, Cook LJ, Pache F, et al. Vaccines and the association with relapses in patients with neuromyelitis optica spectrum disorder. Mult Scler Relat Disord. 2018;23:78-82. doi:10.1016/j.msard.2018.05.003
4. Nishiyama S, Ito T, Misu T, et al. A case of NMO seropositive for aquaporin-4 antibody more than 10 years before onset. Neurology. 2009;72(22):1960-1961. doi:10.1212/WNL.0b013e3181a82621
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