Acute disseminated encephalomyelitis

The frequency of CNS neuroinflammatory events reported in the literature after both SARS-CoV-2 infection and COVID-19 vaccination is still largely unknown. While these conditions can occur spontaneously, they have also been reported following vaccinations, including those for influenza, hepatitis B, human papillomavirus, and COVID-19. Viruses and their vaccines may unmask CNS neuroinflammatory conditions. 

A 47-year-old man with a history of psoriasis and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection presented with 3 months of subacute lower extremity weakness, erectile dysfunction, and gait instability with falls. His symptoms started 2 weeks after receiving the second of his 2-shot primary mRNA vaccine series for coronavirus disease 2019 (COVID-19) vaccination, which he received 3 months after a mild case of COVID-19. He had no other relevant medical or family history and no recent travel. After evaluation by a urologist, he was prescribed a course of cefdinir for presumed prostatitis. His weakness and gait dysfunction worsened over the ensuing 3 months despite this treatment and prompted his presentation to our hospital.

The multifocal, perivascular enhancing lesions in the brain and spinal cord were consistent with relapsing steroid-responsive encephalomyelitis after SARS-CoV-2 infection and subsequent COVID-19 vaccination. This is the first reported case of relapsing steroid-responsive encephalomyelitis after COVID-19 vaccination. 

The patient was diagnosed with immune-mediated encephalomyelitis. The primary cause was felt to be immune-mediated given the relapsing nature of the condition, steroid responsiveness, and absence of other identifiable causes to date, in addition to objective findings of inflammation, including the enhancing MRI lesions, and pleocytosis on CSF testing.

The patient was treated with 5 days of high-dose IV methylprednisolone (1,000 mg), with drastic improvement in gait, strength, and fatigue. He was subsequently discharged on a course of oral prednisone at 80 mg daily and slowly tapered down over several weeks. Two weeks later, his neurologic examination demonstrated near resolution of his neurologic deficits, and repeat MRI of the brain and complete spinal cord also demonstrated resolution of the enhancement.

Another case involves 31-year-old female presented with gradual progression of right-sided weakness and numbness during a three-week period that started one week after the single-dose SARS-CoV-2 vaccine Ad26.COV2.S. Neuroimaging revealed five large juxtacortical T2 FLAIR hyperintense lesions with incomplete contrast enhancement on post-contrast T1 images located supratentorial: one in the right cerebral hemisphere and four in left cerebral hemisphere. The patient was followed up for four months. Symptom debut, clinical picture and MRI were typical for ADEM and the patient completely recovered after high dose intravenous methylprednisolone treatment.

Acute disseminated encephalomyelitis is a rapidly progressive autoimmune process that is characterized by demyelination in the brain and spinal cord as a result of inflammation that occurs in response to a preceding infection or immunization. ADEM ypically presents with encephalopathy and multifocal neurological symptoms. Transverse myelitis is a similar condition that involves inflammation of the spinal cord, leading to weakness, sensory deficits, and bowel/bladder dysfunction. Optic neuritis is an inflammation of the optic nerve that can cause vision loss and pain with eye movement. 

About 200 cases of ADEM, either transverse myelitis or optic neuritis following various COVID-19 vaccines, including the vaccines from AstraZenica, Pfizer, Sputnik V, SinoVac, Moderna, Sinopharm and J&J have been described in the literature. 


REFERENCES


Roy S, Barreras P, Pardo CA, Graves JS, Zamvil SS, Newsome SD. Relapsing Encephalomyelitis After COVID-19 Infection and Vaccination: From the National MS Society Case Conference Proceedings. Neurol Neuroimmunol Neuroinflamm. 2023 Apr 4;10(3):e200112. doi: 10.1212/NXI.0000000000200112. PMID: 37015826; PMCID: PMC10074377.

Gustavsen S, Nordling MM, Weglewski A. Acute disseminated encephalomyelitis following the COVID-19 vaccine Ad26. COV2. S, a case report. Bulletin of the National Research Centre. 2023 Dec;47(1):1-5. PMID: 36643729 doi: 10.1186/s42269-023-00981-7


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