Objective: We report a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) who achieved durable and steroid-free remission after IV cladribine.
Methods: A 25 year-old man presented with progressively worsening headaches, polydipsia, dysarthria, diplopia and vertigo, and obtundation requiring respiratory support. CSF revealed lymphocytosis, and MRI revealed a perivascular pattern of punctate enhancement involving the pons. An extensive workup for inflammatory, autoimmune, infective, and malignant explanations was unrevealing. He responded dramatically to steroids, compatible with CLIPPERS as a diagnosis of exclusion. Attempts to wean prednisone over the ensuing year resulted in 2 clinical relapses and persistent punctate enhancement. Given significant steroid side effects, steroid-sparing agents were considered.
Results: IV cladribine IV (0.0875 mg/kg adjusted body weight daily × 4 days at 0, 4, 8, and 16 months) was selected, given its favorable side effect profile including lower risks of malignancy and infertility and the potential for long-lasting effects. The only side effect was short-term fatigue at the time of infusion. At 20 months after cladribine initiation, he was able to wean-off prednisone altogether. Now at 33 months, he remains in clinical and MRI remission.
Discussion: Cladribine is a rational candidate steroid-sparing treatment for presumed neurologic autoimmune conditions such as CLIPPERS.
Classification of evidence: This study provides Class IV evidence that cladribine is a steroid-sparing treatment consideration in CLIPPERS.
Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.