CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement and steroid response) with longitudinally extensive spinal cord involvement. An atypical presentation

Abstract

CLIPPERS (Chronic Lymphocytic Inflammation with Perivascular Pontine Enhancement and Steroid Response) is a recently described neuroinflammatory disorder in which symptoms involving the brainstem predominate, with variable pattern on neuroi- maging and a perivascular infiltrate of T lymphocytes on brain biopsy. We present a case of a 50-year-old male who presented symptoms of asthenia, adynamia, weakness in lower limbs extremities, sphincter disorder and ataxia. Patient was extensively stu- died to rule out infectious, neoplastic, autoimmune, and demyelinating conditions. Diagnosis was made taking into account signifi- cant clinical-imaging elements, remarking the spinal cord involvement in this patient. Treatment with steroids and steroid-sparing agents, such as methotrexate, was found to be effective. Very few cases in the literature to date describe CLIPPERS-associated spinal cord involvement.