CLIPPERS-mimics (n=13) | CLIPPERS (n=29) | |
Clinical red flags | ||
Cortical signs. Polyneuropathy. Clinical steroid resistance. | 1 PACNS. 1 GFAP. 3 (2 CNS-LYG, 1 PCNSL). | 0 0 0 |
MRI red flags |
| |
| 4 (1 MOG-opathy, 1 PACNS, 1 CNS-LYG, 1 PCNSL). 2 (1 CNS-LYG, 1 PCNSL). 2 (1 PACNS, 1 CNS-LYG). 1 CNS-LYG. 1PACNS. 1 PACNS. 1 GFAP. 2 (1 PACNS, 1 CNS-LYG). 3 (2 CNS-LYG, 1 PCNSL). 3 (2 CNS-LYG, 1 PCNSL). 6 (1 MOG-opathy, 1 GFAP, 2 PACNS, 2 CNS-LYG). | 0 0 0 0 0 0 0 0 0 0 0 |
Additional discriminating features | ||
Relapse when daily dose of glucocorticoids 30 mg. Hypointense lesions on T2*WI. Vascular stenosis on MRA (confirmed on DSA). Diffusion restriction on ADC map. | 5 (1 PACNS, 2 CNS-LYG, 2 PCNSL). 5 (1 MOG-opathy, 3 PACNS, 1 Hodgkin’s lymphoma). 2 PACNS. 2 PACNS. | 0 0 0 0 |
Biopsy of PCGd+ with histological CLIPPERS features. Biopsy of MRI red flags with alternative diagnosis. | 3 (1 CNS-LYG, 2 PCNSL). 7 (1 PACNS, 4 CNS-LYG, 2 PCNSL). | 12 0 |
Red flags only at the second attack. Red flags only after the second attack. Number of red flags 2 at the end of follow-up. | 4 (MOG-opathy, 1 CNS-LYG, 2 PCNSL) 0 12 (all except Hodgkin’s lymphoma). | 0 0 0 |
ADC, apparent coefficient diffusion map; CLIPPERS, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; CNS, central nervous system; CNS-LYG, CNS lymphomatoid granulomatosis; DSA, digital subtraction angiography; GFAP, glial fibrillar acidic protein; Gd+, gadolinium enhancement; MOG, myelin oligodendrocyte glycoprotein; MRA, magnetic resonance angiography; PACNS, primary angiitis of the CNS; PCGd+, punctate and curvilinear gadolinium enhancement; PCNSL, primary CNS lymphoma; T2WI, T2-weighted image; T2*WI, T2*-weighted gradient-echo image.