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. 2019 Apr 8;6(3):e560. doi: 10.1212/NXI.0000000000000560

Figure 1. Imaging and histopathologic characteristics of patients with CNS-FHL.

Figure 1

FLAIR (row A) and post-contrast T1 (row B) MRI findings for patients before initial diagnosis or at relapse. Note the varied lesion appearance with small multifocal, confluent, and tumefactive lesions. Punctate and curvilinear enhancement resembling CLIPPERS is common to 3 of the 4 patients early in disease. Patient 4 had multifocal small lesions with a single area of enhancement. Avid enhancement was a common feature of new or active lesions. Over time, lesions became confluent, and diffuse atrophy evolved in patients 1 and 2. Representative histopathology (row C) images from prediagnosis or relapse biopsies demonstrating a diffuse mixed chronic inflammatory infiltrate in patient 1, foci of perivascular lymphocytes (arrow) extending into the parenchyma for patient 2 prediagnosis, perivascular (arrow) and parenchymal mixed chronic inflammatory infiltrate with small vessel vasculitis in patient 3, and destructive perivascular (arrow) and parenchymal lymphohistiocytic infiltrate in patient 2 at relapse (magnification: ×40 objective). Post-HCT FLAIR (row D) and post-contrast T1 (row E) MRI images demonstrate resolved enhancement and no development of new lesions for patients 1, 3, and 4 after HCT #1 and for patient 2 after HCT #2. CLIPPERS = chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; FLAIR = fluid-attenuated inversion recovery; HCT = hematopoietic cell transplantation.