Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) after COVID-19

ABSTRACT

Background and aims: SARS-CoV-2 infection is now known to be associated with a wide spectrum of neurological autoimmune syndromes, in some cases responding to immunotherapies, arising during or after the infection. Whether molecular mimicry or other immune stimulation may induce an aberrant delayed autoimmune response is still to be established. Methods: Case report. Results: A 71 year-old man with no previous medical history apart from mild COVID-19 pneumonia three month earlier, sought medical attention for a subacute onset of diplopia in left gaze, general malaise and fatigue. MRI was characterized by bilateral FLAIR hyperintensities with punctate, perivascular and confluent post-gadolinium enhancement in the pons, mesencephalon, hypothalamus, internal capsules and right hippocampus. Repeated cerebrospinal fluid analysis were normal (2 cells/μL), with no evidence of oligoclonal bands or atypical cells. Screening panel for autoimmune and infectious aetiologies was negative. Whole-body contrast-enhanced CT was unremarkable. Stereotactic temporal lobe brain biopsy showed aspecific chronic lymphocytic perivascular inflammation. Partial spontaneous remission of symptoms occurred within few weeks. He was then treated with intravenous high-dose methylprednisolone with almost complete enhancement regression on MRI. Collected data were suggestive of CLIPPERS with diffuse bilateral sovratentorial involvement. The patient started daily oral steroid tapering and monthly cycles of intravenous cyclophosphamide with persistent clinical and neuroradiological stability. Conclusion: CLIPPERS is a rare diagnosis and to the best of our knowledge, this is the first time it was reported after COVID-19 disease. Even though a case report is not enough to suggest a causal link, future reports could support this possibility. (Figure Presented).