Abstract
Background/Objectives Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system (CNS) inflammatory syndrome. Whilst ‘pontine’ is specified in the title, other CNS regions are often involved, including the spinal cord in approximately 50% of cases. We report a diagnostically challenging case of CLIPPERS with prominent spinal cord involvement, to raise awareness of the phenotypic spectrum.
Methods Single case report.
Results A 24-year-old Nepalese man developed progressive ataxia and lower limb heaviness. Bilateral upper and lower limb hyperreflexia, sustained ankle clonus and extensor plantar reflexes were found on examination five months after symptom onset. Magnetic resonance imaging demonstrated innumerable contrast-enhancing foci, predominantly in the cervical and thoracic spine, but also brain and brainstem. Cerebrospinal fluid findings included mildly raised protein, lymphocytic pleocytosis with 20 cells per microlitre and no oligoclonal bands. A whole body positron emission tomography scan was unremarkable. The condition responded to intravenous methylprednisolone followed by oral prednisone. He moved overseas where he was also given mycophenolate, but relapsed eight months later in the context of down-titrating prednisone. Brain biopsy was then performed, demonstrating perivascular lymphocytic inflammation consistent with CLIPPERS. Prednisone dose was increased and plasmapheresis, followed by rituximab, were administered. There was marked improvement in function and MRI appearance on follow up five months later.
Conclusion Our case emphasises the potential for CLIPPERS to present with prominent spinal cord involvement. CLIPPERS should be considered in the differential diagnosis for CNS punctate and curvilinear contrast-enhancing lesions extending beyond the hindbrain.
