Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids: An Acute Presentation

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare disease with an unknown etiology which most commonly results in subacute diplopia and ataxia. Diagnosis is achieved through a triad of the following findings: lymphocytic pleocytosis with increased CD4+ T cells on cerebrospinal fluid (CSF) analysis; perivascular punctate and curvilinear hemorrhages in the pons, medulla, or cerebellum on magnetic resonance imaging (MRI) with contrast; and the cessation of symptoms after the initiation of corticosteroids. Here, we report the case of a 23-year-old male who presented with non-specific signs and symptoms, including diffuse weakness in all limbs, ataxia, and slurred speech. The diagnosis was achieved through a contrast MRI of the brain, suggestive of brainstem encephalitis, and a CSF analysis, which revealed elevated glucose and protein levels. Intravenous methylprednisolone was administered for five days and resulted in acute improvement of the patient's clinical status. Repeat CSF analysis and MRI of the brain with contrast two weeks later showed resolution of previous findings. CLIPPERS syndrome is a newly identified disease thought to cause a predominantly inflammatory reaction in the pons, medulla, cerebellum, and supratentorial region. MRI with contrast tends to reveal a "salt and pepper appearance" in a punctate and curvilinear fashion. The hallmark of treatment is corticosteroid therapy, and discontinuation of therapy should be done with caution as relapse of the syndrome with corticosteroid withdrawal has been documented.