Objective:

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a distinct inflammatory disorder of the central nervous system characterized by specific clinical, radiologic, and histopathologic findings, and notable responsiveness to corticosteroid therapy. The exact pathogenesis remains unclear, and there is ongoing debate as to whether CLIPPERS represents a unique disease entity or a syndrome encompassing heterogeneous etiologies. 

Background:

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Design/Methods:

Case Presentation: We present a complex case of a 59-year-old female who sustained a neck injury while lifting a heavy object approximately two years prior. The following day, she developed progressive leg weakness, difficulty rising from a chair, inability to raise her neck up, and electric shock–like sensations radiating into the arms and legs. These symptoms appeared concurrently, accompanied by unintentional weight loss of 40 lbs in over 2 years. The patient was initially treated by rheumatology for presumed seronegative rheumatoid arthritis with mycophenolate mofetil and corticosteroids, which resulted in partial improvement. Brain MRI demonstrated scattered punctate diffusion-restricting foci with associated T2 hyperintensities, while cervical spine MRI revealed central canal dilation with subtle ependymal enhancement. The etiology of these findings was initially uncertain. However, discontinuation of steroids resulted in disease relapse with worsening MRI findings. An extensive workup, including cerebrospinal fluid analysis and evaluation for autoimmune, infectious, inflammatory, and neoplastic processes, was inconclusive. With CLIPPERS being a diagnosis of exclusion, other potential causes were reasonably ruled out through this evaluation. Based on the inflammatory CSF, steroid responsiveness and classic appearance on Brain MRi the diagnosis of CLIPPERS was made.

Results:

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Conclusions:

This case highlights the heterogenous presentation and the importance of early recognition of CLIPPERS as a rare yet treatable inflammatory CNS disorder. Prompt diagnosis and corticosteroid therapy are crucial to prevent disease progression, neurological impairment, and irreversible damage, emphasizing the need for heightened clinical awareness of this entity.