Alexandra Rincones Alfonso1, Osama Elzaafarany2, Sepideh Mokhtari2, David Iacono2, Muhammad Jaffer2, Edwin Peguero2
1University of South Florida, 2Moffitt Cancer Center
Objective:
To expand on an elusive disorder through report of unique MRI findings.
Background:
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory CNS disorder. CLIPPERS is currently characterized by enhancement of perivascular punctate and/or curvilinear lesions in the pons and cerebellum on MRI, lymphocytic pleocytosis with T-cell predominance on CSF analysis and clinical improvement after steroid use.
Design/Methods:
We report a case of a 60-year-old man that presented with symptoms of progressively worsening fatigue, balance and gait difficulty and an enhancing cerebellar lesion on brain MRI. Biopsy, which was completed after dexamethasone use, revealed an atypical lymphoid infiltrate with positive T-cell rearrangement. CSF was negative for malignancy. Chemotherapy with methotrexate and maintenance temozolomide were started for presumptive diagnosis of primary CNS lymphoma. Symptoms improved initially after five MTX cycles with partial response. He was started on temozolomide which was discontinued as the initial diagnosis was no longer most favorable. Symptoms returned about a month after completion of chemotherapy and continued to worsen the next three months. Subsequent MRI scans showed progression with extensive lesions in the pons, cerebellum, frontal, mesial temporal and occipital lobes, basal ganglia, thalamus, hypothalamus, and upper cervical spinal cord. Three additional CSF studies were negative for malignancy. CSF showed elevated lymphocytes, oligoclonal bands, myelin basic protein, IgG index and interleukin-2 (IL-2). In the setting of worsening disease, an additional biopsy was held and five days of high dose IVMP were started.
Results:
A dramatic improvement of symptoms and significant resolution of most lesions ensued after initiation of immunosuppressive therapy. Symptoms have remained stable on a moderate dose of oral prednisone.
Conclusions:
We shed light on the possibility that this rare disorder is being commonly misdiagnosed. Our case contributes to the expanding literature regarding the atypical involvement of the supratentorium and spinal cord in CLIPPERS.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.