Cerebellar encephalitis and peripheral neuropathy with an atypical clinical and neuroimaging signature following Covid-19 vaccine: a report of two cases Authors

• Published in: Journal of neurology
• Main Authors: Sicard, Marin, Shor, Natalia, Davy, Vincent, Rouby, Jean-Jacques, Oquendo, Bruno, Maisonobe, Thierry, Puybasset, Louis, Lehericy, Stephane, Lecarpentier, Amandine, Donadio, Cristiano, Oasi, Christel, Belmin, Joël, Lubetzki, Catherine, Corvol, Jean-Christophe, Grabli, David, Saracino, Dario
• Format: Journal Article
• Language: English
• Published: Springer Verlag 04.05.2024
• Subjects: Life Sciences; Covid-19; SARS-COV-2; plasmapheresis; Vaccination; neuropathy; cerebellar encephalitis
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-024-12390-5

Background. Immune-mediated neurological syndromes may occur following SARS-CoV-2 infection or vaccination. Their presentation can be extremely heterogeneous and there are no established guidelines for treatment.Methods. We report the clinical and instrumental features of two patients presenting neurological syndromes started two weeks after Covid-19 vaccine, with infection co-occurring in one case, describe their common neuroimaging profile and illustrate their response to immunosuppressive treatment.Results. Both patients displayed simultaneous central and peripheral nervous system involvement. Cerebellar ataxia and predominantly sensory neuropathy/neuronopathy were present in one case, whereas rapidly evolving quadriparesis, sensory level, bulbar deficits and altered vigilance characterized the other. Electrophysiological studies were in favor of both central and peripheral conduction deficits. Brain MRI displayed inflammatory changes with contrast enhancement in superior cerebellar peduncles in both cases. Intrathecal IgG synthesis was present, but no known autoantibodies were found in plasma and CSF. Immunosuppressive treatments, namely plasma exchanges and high-dose corticosteroids, had a partially favorable impact, at least on central involvement.Conclusions. We report two cases of cerebellar encephalitis following Covid-19 exposure with an atypical neuroimaging signature involving superior cerebellar peduncles. This neuroinflammatory pattern, already identified in patients exposed to SARS-CoV-2 vaccine, suggests that cerebellar encephalitis may be considered a rare but severe adverse event of RNA vaccine against Covid-19. We also provide evidence concerning the potential benefit of intensive immunosuppressive strategies in such cases.