CLIPPERS: a recognizable image of a treatable disease

• Published in: Arquivos de neuro-psiquiatria Vol. 77; no. 12; pp. 903 - 904
• Main Authors: Mello, Denise Fabri Rezende Engracia, Queiroz, Rodolfo Mendes, Miyake, Cecilia, Fábio, Soraia Ramos Cabette, Abud, Lucas Giansante
• Format: Journal Article
• Language: English
• Published: Brazil Arquivos de Neuro-Psiquiatria 01-12-2019; Academia Brasileira de Neurologia (ABNEURO)
• Subjects: Adrenal Cortex Hormones - therapeutic use; Aged, 80 and over; Biopsy; Brain Stem - diagnostic imaging; Brain Stem - pathology; Chronic Disease; Corticosteroids; Diagnosis, Differential; Differential diagnosis; Encephalomyelitis - diagnostic imaging; Encephalomyelitis - drug therapy; Encephalomyelitis - pathology; Eyelid; Glymphatic System - diagnostic imaging; Humans; Lymphoma; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; Male; Neuroimaging; Neurosyphilis; Steroid hormones
• ISSN: 0004-282X; 1678-4227
• DOI: 10.1590/0004-282X20190170

An 80-year-old man with a history of stroke presented with eyelid ptosis of three years' duration. Magnetic resonance imaging (MRI) of the brain revealed findings consistent with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). As limitations precluded anatomopathological examination and the imaging pattern was characteristic enough to support treatment without biopsy, the decision was made to prescribe long-term corticosteroids. At the two-year follow-up, MRI findings had regressed and the patient had improved clinically, meeting criteria for treatment response. Biopsy is indicated when symptoms and MRI findings are unresponsive to treatment. The differential diagnosis should include CNS lymphoma, neurosyphilis, and vasculitides.