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Paracentral acute middle maculopathy in Susac syndrome after dual exposure to SARS-CoV-2 antigen
Fernando Korn Malerbi1, Vinicius Andreoli Schoeps2, Kimble T F Matos3
1Department of Ophthalmology and Visual Sciences, Federal University of Sao Paulo, Sao Paulo, Brazil.
Abstract:
We report a case of Susac syndrome after SARS-CoV-2 infection and subsequent vaccination that presented with meningitis and retinal microembolisation in the form of paracentral acute middle maculopathy (PAMM). After presenting with headache, fever and myalgia followed by scotomata, a woman in her 50s was hospitalised for meningitis; she had had mild COVID-19 infection 2 months prior to admission, having received the first vaccine dose 1 month prior to the neurological manifestation. Eye fundus examination and optical coherence tomography were suggestive of PAMM. D-dimer levels and erythrocyte sedimentation rate were elevated. Before infectious investigation results were available, she was started on empirical antibiotic and antiviral treatment. Having ruled out infectious causes, she was started on high-dose prednisolone. After 1 month, there was partial resolution of retinal lesions. This case highlights that exposure to SARS-CoV-2 antigen may be related to this rare syndrome; treatment with steroids may improve central and retinal impairment.
Insights
Susac syndrome, a rare condition, can occur after SARS-CoV-2 infection and vaccination, presenting with meningitis and vision loss. Steroid treatment may help improve neurological and retinal damage in these cases.
Area of Science:
- Neurology
- Ophthalmology
- Immunology
Background:
- Susac syndrome is a rare neurological disorder characterized by encephalopathy, retinopathy, and hearing loss.
- The association between SARS-CoV-2 infection/vaccination and neurological conditions is an emerging area of research.
Observation:
- A woman in her 50s developed meningitis and paracentral acute middle maculopathy (PAMM) after SARS-CoV-2 infection and vaccination.
- Clinical presentation included headache, fever, myalgia, and visual disturbances (scotomata).
- Elevated D-dimer and erythrocyte sedimentation rate were noted, with initial empirical antibiotic and antiviral treatment.
Findings:
- Ocular findings suggestive of PAMM were confirmed by fundus examination and optical coherence tomography.
- Infectious causes were ruled out, leading to high-dose prednisolone treatment.
- Partial resolution of retinal lesions was observed after one month of steroid therapy.
Implications:
- This case suggests a potential link between SARS-CoV-2 antigen exposure and the development of Susac syndrome.
- Early diagnosis and treatment with corticosteroids may be crucial for managing neurological and visual impairments.
- Further research is warranted to understand the pathophysiology and optimize treatment strategies for post-SARS-CoV-2 Susac syndrome.
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