A case of CLIPPERS with persistent CSF OCBs: where do the immunoglobulins come from?

Abstract Introduction: CLIPPERS is a rare inflammatory disorder of central nervous system characterized by significant involvement of the subtentorial region. There is a growing body of evidence suggesting a potential association between CLIPPERS and malignancies, specifically lymphoma. Some studies have proposed a role for Epstein Barr Virus in the pathogenesis of CLIPPERS, with the presence of CSF oligoclonal bands observed in certain cases. Case presentation: A 39-year-old man presented with gait instability and cognitive impairment lasting proximately half a year following a respiratory tract infection. Pathogenic analysis identified the presence of Epstein-Barr virus in CSF. MRI revealed hyperintense lesions in the subcortical white matter, pontine and cerebellar regions, accompanied by perivascular contrast enhancement resembling a curvilinear, pepper-like pattern. Persistent oligoclonal bands (OCBs) in CSF have also been a notable characteristic. Biopsy results indicated a perivascular infiltration of T lymphocyte, ruiling out granulomas, vasculitis, or neoplasia. After excluding other potential diagnosis, the patient was ultimately diagnosed with CLIPPERS. Long term follow-up revealed persistent cerebrospinal fluid restricted oligoclonal bands and elevated protein. Conclusion: The presence of persistent of OCBs in patients with CLIPPERS may suggest a heightened B cell activation milieu and a history of prior infections. Given the elevated risk of malignant transformation, long-term monitoring is imperative for CLIPPERS patients, especially those with Epstein-Barr virus infection.