title: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids with a Significant Elevation of β-2 Microglobulin Levels.
abstract: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a relapsing-remitting disorder for which steroid administration is a key to control the progression. CLIPPERS can exhibit radiological features similar to malignant lymphoma, whose diagnosis is confounded by prior steroid administration. We report a case of CLIPPERS accompanied by abnormal elevation of β-2 microglobulin in the cerebrospinal fluid (CSF). A 62-year-old man started to experience numbness in all fingers of his left hand one year ago, which gradually extended to his body trunk and legs on both sides. Magnetic resonance imaging demonstrated numerous small enhancing spots scattered in his brain and spinal cord. CSF levels of β-2 microglobulin were elevated; although this often indicates central nervous system involvement in leukemia and lymphoma, the lesions were diagnosed as CLIPPERS based on the pathological findings from a biopsy specimen. We emphasize the importance of biopsy to differentiate between CLIPPERS and malignant lymphoma because the temporary radiological response to steroid might be the same in both diseases but the treatment strategies regarding the use of steroid are quite different.
authors.affiliation: Department of Neurosurgery, Saitama Medical Center, Saitama Medical University, Saitama, Tokyo, Japan.
authors.forename: Naoaki
authors.initials: N
authors.lastname: Fujisawa
authors.affiliation: Department of Neurosurgery, Saitama Medical Center, Saitama Medical University, Saitama, Tokyo, Japan.
authors.forename: Soichi
authors.initials: S
authors.lastname: Oya
authors.affiliation: Department of Radiology, Graduate School of Medicine and Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: Harushi
authors.initials: H
authors.lastname: Mori
authors.affiliation: Department of Neurosurgery, Saitama Medical Center, Saitama Medical University, Saitama, Tokyo, Japan.
authors.forename: Toru
authors.initials: T
authors.lastname: Matsui
keywords: Biopsy; Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; Diagnosis; malignant lymphoma; β-2 microglobulin