Objective: To describe a rare manifestation of a case of CLIPPERS with video-based documentation of treatment responsiveness.

Background: CLIPPERS is a rare inflammatory disease of central nervous system (CNS) creating punctate gadolinium enhancing lesions on MRI that are pontine predominant and markedly responsive to corticosteroid therapy. Pathological findings can be characteristic however given the rarity of this disorder, inconsistent patterns in cerebrospinal fluid (CSF), presence of mimics including malignancies and limitations on obtaining biopsy; obtaining a definitive diagnosis is challenging.

Method: Case report.

Results: 64-year-old female initially presented with 2 years of slowly progressive postural tremors in bilateral upper extremities, 1.5 years of progressive cognitive impairment and 6 months of frequent falls. She had been previously diagnosed with Parkinson’s disease and failed a levodopa trial. On neurological exam she was found to have bradykinesia, hypomimia, shuffling gait and postural instability. An extensive work up including CSF studies, paraneoplastic panel (including Ma-2 antibody), positron emission tomography (PET) scan, and computed tomography (CT) scan of the chest, abdomen, and pelvis was unremarkable. Brain MRI revealed T2 hyperintensities in the pons, left basal ganglia, and corona radiata with patchy contrast enhancement. Given the lack of active signs of malignancy as an underlying cause and all other testing negative, the patient was treated with high dose steroids for 3 days with subsequent symptomatic and MRI improvement and was sustained on maintenance immunosuppressive therapy with continued pursuit of tissue for a definitive diagnosis. Her parkinsonian features responded favorably to a levodopa trial and she continues to show improvement at her 5 month follow up.

Conclusion: CLIPPERS is a rare CNS disease, difficult to diagnose given its heterogeneous presentations and lack of distinct bio-markers. It is a diagnosis of exclusion which highlights the importance of considering rare manifestations while performing extensive evaluation to rule out other causes of disease. While immunosuppressive therapy remains the mainstay of primary immunologic treatment,  understanding the phenomenology of the presentation and addressing the parkinsonism helped in the clinical management of our patient.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/progressive-parkinsonism-and-cognitive-changes-a-rare-presentation-of-chronic-lymphocytic-inflammation-with-pontine-perivascular-enhancement-responsive-to-steroids-clippers/