title: [Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with swelling in the brainstem: a case report].
abstract: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system inflammatory disease characterized by the punctate gadolinium enhancement peppering the pons and the cerebellar peduncles as neuroimaging. We report the case of a 66-year-old woman who presented with CLIPPERS associated with swelling in the brainstem. She was hospitalized because of gait ataxia and consciousness disturbance. MRI of the brain showed FLAIR hyperintense lesions in the pons, cerebellar peduncles, cerebellum and the subcortical white matter lesion in the right occipital lobe with significant swelling in the brainstem. Diffusion-weighted MRI did not show an abnormal signal, indicating vasogenic edema. Post-contrast T1-weighted MRI showed enhanced area in the right occipital lobe and panctate gadolinium enhancement peppering brainstem. Treatment with steroids led to rapid improvement. However, she showed exacerbation of clinical and radiological findings during the tapering schedule of steroid. The biopsy from the occipital lobe revealed intense perivascular and parenchymal lymphocytic infiltrates composed of primarily T cells, B cells and macrophages. The patient was diagnosed with CLIPPERS, and treatment with increased dose of corticosteroid induced a clinical improvement. Previous reports well described a characteristic MRI finding of punctate enhancement peppering the pons. In addition, the pons and cerebellar peduncles swelling can occur in this disorder.
authors.affiliation: Department of Internal Medicine I, Osaka Medical College.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Tani
authors.forename: Hideto
authors.initials: H
authors.lastname: Nakajima
authors.forename: Kazushi
authors.initials: K
authors.lastname: Yamane
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ohnishi
authors.forename: Fumiharu
authors.initials: F
authors.lastname: Kimura
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Hanafusa
mesh_terms: D000368:Aged; D001623:Betamethasone / Q000008:administration & dosage; D001929:Brain Edema / Q000188:drug therapy / Q000209:etiology*; D001933:Brain Stem; D002493:Central Nervous System Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D011149:Pons / Q000473:pathology*; D011239:Prednisolone / Q000008:administration & dosage; D013601:T-Lymphocytes / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D001623:Betamethasone; D011239:Prednisolone