Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a rare chronic inflammatory neurological syndrome affecting multiple regions of the brain, including brainstem, cerebellum, and spinal cord, that has become increasingly recognized since its introduction in 2010 by Pittock et al.1 The hallmark neuroimaging finding for CLIPPERS consists of multiple punctate, curvilinear lesions, notably, “peppering” of the pons with spread to other parts of the cerebellum on gadolinium-enhanced magnetic resonance imaging (MRI). The clinical presentation of CLIPPERS is variable; symptoms are subacute, progressive, and episodic, making diagnosis difficult. However, once identified, it is treatable with long-term steroids, making early recognition and treatment key to reducing morbidity and mortality.1, 2, 3
A 71-year-old woman with no significant past medical history presented with 5 months of progressively worsening dizziness, intermittent diplopia, ataxia, and occasional hand and lip tingling. Vital signs were unremarkable. Physical examination revealed multidirectional nystagmus, and dysmetria with finger-to-nose and heel-to-shin testing bilaterally. Extensive work-up for autoimmune disease, infection, malignancy, and paraneoplastic neurological disease was negative. Flow cytometry did not show oligoclonal bands. Prior to admission, extensive outpatient work-up included multiple negative lumbar punctures, unremarkable for leukocytosis, lymphocytosis, protein and glucose abnormalities, and cytology. A brain MRI study had demonstrated miliary pattern enhancement in the brainstem and cerebellum, which had progressed on subsequent hospital imaging with new involvement of thalami, basal ganglia, and central cerebral white matter (Figure 1).
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-Kshipra Joshi, DO, Todd Golden, MSc, Sehem Ghazala, MD
This article originally appeared in the February issue of The American Journal of Medicine.
