ABSTRACT
Objective: To present an unusual presentation of CLIPPERS that was responsive to rituximab Background: CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a neuroinflammatory disorder typically affecting the brainstem and cerebellum with clinical and radiographic improvement with steroids. One reported case showed improvement with rituximab. We present a case of CLIPPERS with supra- and infratentorial involvement that improved with rituximab. Design/Methods: A 30-year-old male presented with several months of headaches, dizziness, and face and arm numbness, then developed diplopia and gait ataxia. Serial MRI's showed worsening punctate enhancing and T2-hyperintense lesions in the brainstem and supratentorial white matter over months. Workup showed normal CSF cell count, and negative CSF cytology, ACE, VDRL, oligoclonal bands, serologic IgG4, Coccidioidomycosis, Lupus, rheumatoid arthritis, and COVID. Vascular imaging showed no evidence of vasculitis. Biopsy showed a dense perivascular lymphocytic infiltrate including B and T cells, without evidence of vasculitis or lymphoma. He received IVIG and IV solumedrol, with symptom resolution and some improvement on imaging. Rituximab was started with subsequent resolution of the lesions on MRI. Results: NA Conclusions: CLIPPERS has a variable clinical presentation but typically includes gait ataxia and diplopia. MRI shows multiple punctate/curvilinear enhancing lesions in the brainstem and cerebellum, rarely in the spinal cord or supratentorially. Differential diagnoses include neurosarcoidosis, Behcet's disease, vasculitis, lymphoma, chronic infections, glioma, and demyelinating disease. It is characterized by responsiveness to steroids, and patients require long-term steroid or steroid-sparing agent treatment, at least until resolution of enhancement. Methotrexate, hydroxychloroquine, and cyclophosphamide are most commonly used. There was one report of treatment with rituximab with 4 years stability. Our case was unusual as he had supra and infratentorial lesions, and he had good response to rituximab. Rituximab should be considered in the treatment of CLIPPERS.